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Brain & Development 24 (2002) 345–649
www.elsevier.com/locate/braindev
KN
Human genome project and its medical implications
Y. Sakaki
Human Genome Center, The Institute of Medical Science,
The University of Tokyo, Tokyo, Japan
Human genome project (HGP) started on 1990 under an
international cooperation, and has made remarkable
progress such as the first generation genetic linkage map
of the human genome on 1994, and the draft sequence of
the entire genome on 2000. These great achievements
opened a new field of medicine and biology. Discovery of
genetic variations, particularly large amounts of single
nucleotide polymorphisms enabled us to genetically analyze
not only typical Mendelian disorders but also complex
diseases. Discovery of disease related genes opened a gate
to the medical, clinical applications of genomics such as
genetic diagnosis, gene therapy and drug discovery, and
the progress of such applied genomics will eventually establish
new types of medicine; that is, genome-based preventive
medicine and personalized medicine. The progress of
HGP, together with the development of new technologies
such as DNA chip/micro array, and yeast two hybrid system,
also allow us to explore the molecular mechanisms of
complex biological process such as development and differentiation,
immune system and neural system. I will summarize
such progress of genomics and discuss its impact on
medicine.
PLENARY LECTURE/TOPIC UPDATE
PL-1/TU-1
Developmental Neuroscience
PL-1
Clinical disorders of brain plasticity
M.V. Johnston
Kennedy Krieger Institute and Johns Hopkins University
School of Medicine, Baltimore, MD, USA
The child’s brain is far more plastic than the adult’s as
judged by its greater ability to reorganize and recover after
injury and its capacity for learning and memory. Several
cellular and molecular events have been correlated with
the enhanced plasticity of the developing brain, including
the proliferation and pruning of synapses in cerebral cortex
and the enhanced activity of excitatory neurotransmitter
receptors in the neonatal period. The N-methyl-d-aspartate
(NMDA)-type glutamate receptor is one of several cell
surface receptors involved in neuronal plasticity, and its
activity regulates neuronal survival. Over-stimulation of
the NMDA receptor by perinatal hypoxic ischemia can trigger
apoptosis or necrosis while blockade of NMDA receptors
can also trigger apoptosis. Physiologic stimulation of
the NMDA receptor as well as receptors linked to the Ras-
MAP kinase pathway can activate transcription factors such
as cAMP-response element-binding protein (CREB), stimulating
expression of genes involved in learning and memory.
An expanding group of pediatric neurologic disorders can
be linked to disorders in these signaling pathways. In Coffin-
Lowry syndrome, the degree of mental retardation can be
correlated with the ability of the protein kinase C agonist
PMA to stimulate CREB phosphorylation by the mutated
kinase RSK2 in lymphoblasts (Harum et al., Neurology
2001;56:207). Genetic disorders in similar signaling
cascades have been detected in Rubinstein-Taybi syndrome,
neurofibromatosis one, tuberous sclerosis two, and Huntington’s
disease. Encephalopathies associated with congenital
hypothyroidism, non-ketotic hyperglycinemia and lead
poisoning also appear to involve abnormalities in these
pathways. Clinical disorders of neuronal plasticity may be
amenable to treatment as their pathogenesis is understood.
TU-1A
Disease gene hunting in China
Y. Shen
Chinese national human genome research center, national
laboratory of medical molecular biology, IBMS, CAMS and
PUMC, Beijing, China
Public release of human genome sequence has ensured a
level playing field worldwide for clinicians and researchers
tracking the genes mutated in inherited diseases. Studies of
samples taken from Chinese populations are yielding
advances. The first success came in 1998, when a team
led by Jia-hui Xia of the National Laboratory of Medical
Genetics of China in Changsha, Hunan province, identified
a gene for a form of neurological deafness. Last year, my
group revealed the genetic basis of another disease, dentinogenesis
imperfecta Shields type II, in which the teeth are
discoloured and chip easily. We showed that a mutation in a
gene called dentin sialophosphoprotein (DSSP) is responsible.
Alongside our paper, researchers led by Xiangyin Kong
of the Shanghai Research Center of Biotechnology reported
that mutations in DSPP can also cause hearing loss.
Researchers led by Lin He mapped the gene for brachydactyly
type A-1, a disease in which joints of the figures are
missing, misplaced or disfigured, to a small region of chro-
0387-7604/02/$ - see front matter q 2002 Elsevier Science B.V. All rights reserved.
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346
Abstracts
mosome 2 and found that mutations in Indian hedgehog
(IHH) cause brachydactyly type A-1, beating competition
from several groups around the world.
TU-1B
The central nerve system synaptic junction: evolution,
architecture and plasticity of an adhesive device
D.R. Colman
The Montreal Neurological Institute, Canada
The central nervous system (CNS) synaptic junction is
likely to have evolved from the adherens junction of primitive
epithelia. Both junctional types are held together by
classic cadherins, and we have proposed that these bona
fide adhesion molecules are involve in establishing specific
connectivity patterns and in functioning as synaptic glue.
Data supporting this notion will be presented. In other
studies, we have been pursuing a biochemical dissection
of CNS synaptic junction, and now report the key structural
features of the presynaptic compartment may be solubilized
and then reconstituted in vitro. This is a first step in generating
a ‘synthetic’ synapse, using a minimum of functional
elements.
PL-2/TU-2
Neonatal Neurology
PL-2
Brain injury in the premature infant – recent advances
J. Volpe
Harvard Medical School and Children’s Hospital, USA
Brain injury in the premature infant is a problem of
enormous importance. Periventricular leukomalacia
(PVL) is the major neuropathological form of this brain
injury and underlies most of the neurologic morbidity
encountered in survivors of premature birth. Prevention
of PVL now seems ultimately achievable because of recent
neurobiologic insights into pathogenesis. The pathogenesis
of this lesion relates to three major interacting factors. The
first two of these, an incomplete state of development of
the vascular supply to the cerebral white matter, and a
maturation- dependent impairment in regulation of cerebral
blood flow underlie a propensity for ischemic injury to
cerebral white matter. The third major pathogenetic factor
is the maturation-dependent vulnerability of the oligodendroglial
(OL) precursor cell that represents the major cellular
target in PVL. Recent neurobiologic studies show that
these cells are exquisitely vulnerable to attack by free radicals,
known to be generated in abundance with ischemiareperfusion.
This vulnerability of OLs is maturation-dependent,
with the OL precursor cell highly vulnerable and the
mature OL resistant, and appears to relate to a developmental
window characterized by a combination of deficient
antioxidant defenses and active acquisition of iron during
OL differentiation. The result is generation of deadly reactive
oxygen species and apoptotic OL death. Important
contributory factors in pathogenesis interact with this
central theme of vulnerability to free radical attack. Thus,
the increased likelihood of PVL in the presence of intraventricular
hemorrhage could relate to increases in local
iron concentrations derived from the hemorrhage. The
important contributory role of maternal/fetal infection or
inflammation and cytokines in the pathogenesis of PVL
could be related to effects on the cerebral vasculature
and cerebral hemodynamics, to generation of reactive
oxygen species, or to direct toxic effects on vulnerable
OL precursors. A key role for elevations in extracellular
glutamate, caused by ischemia-reperfusion, is suggested by
demonstrations that glutamate causes toxicity to OL
precursors by both non-receptor- and receptor-mediated
mechanisms. The former involves an exacerbation of the
impairment in antioxidant defenses, and the latter, an
amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid
(AMPA)/kainate receptor-mediated cell death. Most
importantly, these new insights into the pathogenesis of
PVL suggest potential preventative interventions. The
latter include avoidance of cerebral ischemia by detection
of infants with impaired cerebrovascular autoregulation,
e.g. through the use of in vivo near-infrared spectroscopy,
the use of free radical scavengers to prevent toxicity by
reactive oxygen species, the administration of AMPA/
kanaite receptor antagonists to prevent glutamate-mediated
injury, or the use of maternal antibiotics or anticytokine
agents to prevent toxicity from maternal/fetal infection or
inflammation and cytokines.
TU-2A
Neuropathological studies on neonatal hypoxic-ischemic
brain damage in experimental animals
H. Yoshioka
Department of Pediatrics, Kyoto Prefectural University of
Medicine, Kyoto, Japan
Biochemical and physiological experiments on neonatal
hypoxic-ischemic brain damage may demonstrate many
biochemical or physiological derangements in the brain,
but it cannot be known without pathological verification
whether such a derangement may result in permanent brain
damage or not. Neuropathological approach has advantage
as follows. (1) It can distinguish irreversible damage from
reversible ones. Therefore, we can find which therapy
protects or reduces permanent brain damage. (2) It can
demonstrate regional difference in the response of the
brain. Here I present some results of neuropathological
studies on neonatal hypoxic-ischemic brain damage
obtained in our laboratory. Since our earlier studies
using neonatal mice suggested that hypoxia alone does
not lead to apparent brain damage, but ischemia with or

Abstracts 347
without hypoxia causes unequivocal brain lesions, we
produced a new model of global ischemia by 3-vesselocclusion
in 10-day-old rats. Using this model, we investigated
pathogenic mechanisms and effective treatments of
ischemic brain damage. (1) One-hour-ischemia reduced
cerebral blood flow (CBF) to about 10% of control level
and caused exhaustion of brain adenosine-5 0 -triphosphate
(ATP) within 20 min. (2) Although ATP was exhausted for
more than 30 min, 24 h later the brains revealed that only
10% of cortical neurons had ischemic changes. (3) Twohour-ischemia
caused diffuse brain infarct: More than 70%
of cortical neurons showed ischemic changes. (4) Most
ischemic neurons were TUNEL positive and electrophoresis
of the brain tissues showed ladder formation, but electron
microscopic study revealed only necrotic changes and
no evidence of apoptosis. (5) Post-ischemic administration
of polyethylene glycol-superoide dismutase (PEG-SOD)
reduced ischemic damage to about 10%. (6) Pre- and
post-ischemic administration of magnesium sulfate
reduced ischemic brain damage to about 20%. Intraventricular
administration of synaptotagmine antisense DNA 3
days before ischemic event prevented ischemic brain
damage.
TU-2B
Neuroimaging characteristics of neonatal brain injury
P.S. Hüppi
Department of Pediatrics, University of Geneva, Switzerland
and Department of Neurology, Harvard Medical
School, Boston, USA
Despite marked improvements in perinatal practice, perinatal
brain injury remains one of the most common complications
causing chronic handicapping conditions. Many of
the cellular and vascular mechanisms of perinatal brain
damage have been studied and show a correlation between
the nature of the injury and the maturation of the brain. In
vivo diagnostic tools are therefore needed to assess brain
development, to detect and to localize early brain injury
and monitor interventions aimed at minimizing or preventing
irreversible brain injury. Magnetic resonance (MR)
techniques have in recent years become the imaging
modality of choice for a more detailed structural as well
as metabolic and functional assessment of the developing
brain. Conventional MR imaging (MRI) has widely proven
its potential to identify normal and pathologic brain
morphology giving objective information about the structure
of the neonatal brain during development. The sensitivity
of MR imaging to delineate gray matter from white
matter, myelinated and unmyelinated, renders it suitable
for the quantitative measurement of the cerebrum, with
further application to the study of brain maturation in
vivo. Volumetric analysis of 3D-MR imaging data sets is
achieved by segmentation of the imaged volume into the
different tissue types. Quantitative 3D-MRI at term in
preterm infants with perinatal white matter injury shows
significantly decreased cortical gray matter (GM) volume
and a reduced amount of myelinated white matter (WM) as
an expression of structural alteration of brain development
after perinatal brain injury. Functional MR imaging such as
diffusion- weighted imaging (DWI) and perfusion and
blood-oxygenation-dependent BOLD imaging are new
imaging methods providing insights into brain physiology.
DWI in preterm and full term newborns have shown a
reduction of apparent diffusion coefficient (ADC) with
increasing age but also acutely reduced ADC in regions
of early ischemic injury, making this technique ideal for
the early assessment of hypoxic-ischemic injury. Magnetic
resonance spectroscopy represents the unique modality to
study brain metabolism and has become an important addition
to MRI. This review will therefore focus mainly on
recent advances in the application of magnetic resonance
techniques in pre- and postnatal neuroimaging of perinatal
brain injury.
PL-3/TU-3
Seizure Disorders
PL-3
Epilepsy and the developing brain
K. Watanabe
Department of Pediatrics, Nagoya University Postgraduate
School of Medicine, Nagoya, Japan
Age-related onset is a characteristic feature of childhood
epilepsy, and different epileptic syndromes occur at different
ages. Epileptic seizures and syndromes undergo evolutionary
changes with development, and one epileptic
syndrome may evolve into another as in age-dependent
epileptic encephalopathy. These features of childhood
epilepsy are related to the maturation of the brain as well
as the plasticity of the developing brain. Epileptic
syndromes of the newborn undergo evolutionary changes
with development. A common evolution in symptomatic
epilepsies of neonatal onset was partial epilepsy with transient
West syndrome during infancy. In symptomatic West
syndrome, focal and then multifocal discharges were
followed by the appearance of hypsarrhythmia. In symptomatic
generalized epilepsies, discrepancies between electroencephalogram
(EEG) and seizure types are common:
epileptic spasms may persist even if interictal paroxysmal
discharges tend to show localized discharges with age.
Localization-related epilepsies may demonstrate transient
generalization of paroxysmal discharges with various
degrees of clinical deterioration during a certain stage of
development. Severe myoclonic epilepsy never evolves
into Lennox-Gastaut syndrome.

348
Abstracts
TU-3A
Pediatric epilepsy: clinical update
O. Dulac
Service de Neuropediatrie Hopital Saint-Vincent de Paul,
France
One of the major challenges in paediatric epilepsy is to
prevent psychomotor and behavioural deterioration. It may
result from long lasting or frequent seizures, or from major
activation of so-called interictal paroxysmal activity. Early
identification of at risk conditions and risk factors are necessary
conditions to prevent such deterioration. Risk factors for
hypothalamic harmatoma (HH) syndrome are Sturge-Weber
disease, tuberous sclerosis and callosal agenesis that are
disclosed from birth with neuroradiology. For Dravet
syndrome, the identification of abnormal genetic background
will permit optimising new antiepileptic drug treatment and
prevent the use of worsening compounds, to prevent status
epilepticus. West and Lennox-Gastaut syndromes may be
precipitated by inappropriate medication administered for
other types of seizures. For myoclonic-astatic epilepsy, the
early diagnosis of this syndrome, presently based on the clinical/EEG
features could benefit from the ongoing progress in
molecular genetics, permitting early adequate treatment to
prevent the occurrence of myoclonic status. Symptomatic
continuous spikes and waves during slow sleep (CSWS)
may complicate pre-, peri- or early postnatal ischaemic
brain lesions. As for cryptogenic CSWS, some medications
may precipitate the generalisation and the continuous spikewave
activity.
TU-3B
Genetics of epilepsy
I.E. Scheffer
Epilepsy Research Institute, University of Melbourne, Austin
and Repatriation Medical Centre, Monash Medical Centre
and Royal Children’s Hospital, Melbourne, Australia
Revolutionary insights have been gained from the discovery
of epilepsy genes over the last 7 years. Although only ten
idiopathic epilepsy genes have been identified to date, many
encode subunits of ligand-gated and voltage-gated ion channels
which are associated with specific epilepsy syndromes.
For example, ligand-gated ion channels with mutations
include neuronal nicotinic acetylcholine receptors in Autosomal
dominant nocturnal frontal lobe epilepsy and gamma
amino butyric acid A (GABA A ) receptors in generalized
epilepsy with febrile seizures plus (GEFS 1 ) and childhood
absence epilepsy. Similarly, potassium channel genes are
mutated in benign familial neonatal convulsions and sodium
channel genes in GEFS 1 . In vitro functional studies of
sodium channel and GABA mutations show loss of function.
Two recently identified epilepsy genes suggest different
pathways. In autosomal dominant partial epilepsy with auditory
features, a gene encoding the leucine-rich, glioma-inactivated
1 gene has defects. X-linked infantile spasms are
associated with mutations in Aristaless related homeobox
gene (ARX), a homeobox gene involved in gene transcription.
All genes identified thus far are associated with the rare
group of monogenic epilepsies. These genes may not be relevant
to the common idiopathic epilepsies following complex
inheritance where several genes interact and environmental
factors play a role. The challenge is to determine the genes
involved in the common epilepsies and how they interact to
produce specific epilepsy syndromes. A range of strategies
such as sib pair analysis and association studies is being
adopted to solve the genetics of the common epilepsies but
it is unclear which will bear fruit. Unravelling the genetics of
the idiopathic epilepsies holds the promise of refining diagnosis
and treatment in the future.
PL-4/TU-4
Infectious Disease and Immunology
PL-4
Neurological manifestations of falciparum malaria in
children
C.R.J.C. Newton
Neuroscience’s Unit, Institute of Child Health, London, UK
Plasmodium falciparum remains one of the most common
causes of central nervous system infection world-wide, with
children living in sub-Saharan Africa bearing the brunt of the
disease. In comparison to other forms of human malaria, P.
falciparum appears to have a propensity for the brain. The
manifestations of severe falciparum malaria depend upon the
age of the patient, the immune status and there appear to be
differences in the clinical presentation between children
living in Africa and those in Asia. In African children,
seizures and cerebral malaria are the most common acute
manifestations. About 11% of these children with cerebral
malaria develop neurological deficits, of which half appear to
be transient. However other children develop neuro-cognitive
deficits, particularly language impairment and epilepsy.
Epilepsy is associated with complicated seizures during
admission. In Asia, cerebellar ataxia and other post-malaria
neurological syndromes occur, although the aetiology is
unclear. The impact of parasitisation and the long-term
consequences have received little attention, and may have
profound influence on children growing up in endemic areas.
TU-4A
Bacterial meningitis
V. Kalra
Pediatrics, All India Institute of Medial Sciences, New
Delhi, India
Bacterial meningitis, is a catastrophic childhood illness

Abstracts 349
despite advent of potent antibiotics. Bacterial meningitis may
be acute, recurrent orchronic andmay occur sporadically orin
epidemics. Acute bacterial meningitis is etiologically diverse
in the neonatal period, from later years. Geographic differences
in etiology, prevalence patterns, are common. Lumbar
puncture remains the single most important investigation.
Ideally blood sugar estimation – 20–30 min before a L.P.,
staining of csf cells, counting cells within 30 min, gram’s
stain of csf centrifugate and immediate plating for cultures
should be performed. Newer antigen detection methods like
CIE, LPA, Elisaand PCRare usefulduetohighspecificity and
sensitivity (in increasing order), but are not always available.
Evolution of empiric antibiotic regime from penicillin/ampicillin
and chloramphenicolto ceftrixone or cefotaxime will be
discussed. The status of I.V. corticosteroids, preceding initiationantibioticstherapyandourdataregardingoutcomewillbe
discussed. Partially treated acute bacterial meningitis
frequently poses a diagnostic dilemma making therapy decisions
difficult. The algorithm followed in this situation will be
presented. Chronic bacterial meningitis in children most
commonly is tubercular. Fungal, parasitic, risickettsial,
neoplastic or immune deficiency need to be excluded. TBM
merits early diagnosis by high index of suspicion, csf, chest
skiagram Mantoux, family screening, and cranial imaging.
The low diagnostic yield of these tests and current status of
AFB detection techniques will be reviewed. A five drug
regimeincluding streptomycin is recommended. Dexamethasone
probably reduces morbidity related to raised ICP, focal
deficits and inflammatory consequences. Long term ATT
regime is still recommended. Appearance of tuberculomas
post therapy, BCG modification of the clinical profile and
prevalence of TBM will be addressed.
TU-4B
Immune mechanisms in multiple sclerosis
H. Wekerle
Max-Planck-Institute of Neurobiology, Martinsried,
Germany
Multiple sclerosis is more than just one particular disease.
It encompasses a broad spectrum of inflammatory demyelinating
diseases, which are remarkably distinct in distribution
and cellular composition of lesions. This diversity may be
explained by an immune pathogenesis, which is by far more
complex than traditionally assumed. It now turns out that
individual MS lesions are generated in sequential steps involving
close interaction between the auto aggressive lymphocytes
and non-lymphoid target cells. In a first step,
autoimmune T lymphocytes, which are components of the
healthy immune repertoire, are activated, presumably by
microbial factors. The activated T cells then migrate through
immune organs, and undergo profound functional changes
before they break through the endothelial blood–brain
barrier. Within the brain parenchyme, the T cells act on
local glia cells to become efficient presenters of myelin autoantigens.
Glial cell activation is under the tight control of
neurons, which critically determine immune responsiveness
in the brain microenvironment. Active neurons suppress
immune reactivity, while areas with compromised neuronal
activity become preferred sites of immune reactions. Neurotrophins
are involved in neuronal suppression of brain
immune reactivity. Local antigen presentation leads to
fresh activation of the autoimmune T cells, and thus sets
the stage for the recruitment of inflammatory macrophages,
and myelin specific B lymphocytes, processes which can be
studied using transgenic knock-in mice. Myelin binding auto
antibodies cause destruction of myelin sheaths and myelin
forming oligodendrocytes. Axonal processes crossing a
demyelinating inflammatory area degenerate either following
removal of myelin, or after to direct cytotoxic T cell
attacks. Each of the pathogenic steps qualifies as a potential
target of specific immune therapies.
PL-5/TU-5
Autistic Spectrum Disorders
PL-5
Screening, diagnosis and treatment of autistic spectrum
disorders
P. Filipek
University of California Irvine, Irvine, CA, USA
The appropriate diagnosis of autism requires a two-tiered
approach: (1) routine developmental surveillance by all
primary care medical providers; and (2) a diagnostic evaluation
specific to autism. Practice parameters for the diagnosis
and evaluation of autism were formulated by the Child
Neurology Society and the American Academy of Neurology
in 2000, in conjunction with nine professional and four parent
organizations, with liaisons from the National Institutes of
Health. The National Academy of Science later developed
specific recommendations for the treatment of young children
with autism in 2001. This plenary lecture will focus on
the specific detailed recommendations that were established
for each level of diagnosis, and the appropriate intervention
for young children with autism. These consensus statements
are intended to improve the rate of early suspicion and diagnosis
of autism, and therefore early appropriate intervention.
TU-5A
Neuroimaging in autism
T. Hashimoto
Department of Education for Disabled, Naruto University of
Education Nakashima, Takashima, Naruto, Tokushima,
Japan
Autism is a behavioral disorder defined by specific qualitative
impairment of reciprocal social interaction and
communication, as well as restricted interest and stereotyped

350
Abstracts
patterns of behavior, interests, and activities. MRI studies in
autism have found a variety of alterations including a
decrease of size of various brain structures. In functional
neuroimaging studies such as functional MRI (fMRI),
magnetic resonance spectroscopy (MRS), positron emission
spectroscopy (PET) and single photon emission computed
tomography (SPECT), various abnormalities have been
reported. SPECT has found a decrease of regional cerebral
blood flow (rCBF) in the frontal and temporal regions and the
left cerebral hemisphere, and has shown the relationship
between rCBF and symptoms. PET has found metabolic
abnormalities in frontal monoaminergic neuron systems,
and a decrease of glucose metabolic activity in anterior
cingulate gyrus. MRS has found energy metabolism abnormality
in the frontal lobe using 31P-MRS, and abnormality in
medial temporal and lateral frontal regions using 1 H-MRS.
fMRI has found a lower activity in the amygdala during face
recognition test and in the right fusiform gyrus during face
discrimination test. These structural and functional imaging
findings suggest that the anatomic abnormalities are multiple
and not located, and are more likely to be the results of
abnormal development of connections of undefined
disturbed neural networks for complex information processing
involving the frontal cerebral cortex, limbic system, and
posterior fosse brain structures. In this presentation,
previously reported findings will be discussed in relations
with possible mechanism of symptom occurrence in autism.
TU-5B
ADHD: current concepts and neurobiology
M. Denckla
The Kennedy Krieger Institute, Baltimore, MD, USA
Aided by advances in cognitive neuroscience and neuroimaging,
the emerging current concept of ADHD is that it
represents a group of deficits in self-control; affected in
some combination are motor control, cognitive control, and
emotional control. So frequent is the comorbidity with
ADHD of Developmental Motor Coordination Disorder
that this combination mirrors Gillberg’s DAMP syndrome.
Neuroimaging implicates parallel underlying circuits
comprised of frontal, striatal, and cerebellar regions, with
consensus based on MRI measurements that somewhat smaller
total brain volumes (3–5% reductions) are of multifocal
(not diffuse) origin, mostly small frontal and striatal hypoplasia.
This topic update on ADHD will also review diagnosis,
behavioral genetics, prognosis (especially genderdiscrepant),
neurotransmitters (with implications for genetics
and pharmacotherapeutics) and non-pharmacological
therapies. The emphasis, however, will be on the following:
implications of motor localization clues; neurocognitive
elucidation that attention allocation (not attention per se) is
deficient; and evidence from in vivo MR neuroimaging, both
anatomic MRI and fMRI, that frontal-striatal-cerebellar
circuits deserve scrutiny in relation to ADHD.
SYMPOSIUM
SY-1
Pathogenesis and Prevention of Prenatal and Perinatal
Brain Damage
SY-01-1
Structural and functional studies on cadherins, synaptic
adhesion molecules
W. Shan, D.R. Colman
Fishberg Research Center for Neurobiology, The Mount
Sinai School of Medicine, New York, New York, USA
The ability of the mammalian CNS to perform complex
informative processes and highly cognitive functions is
based on the precise and specifically determined connectivity
of different neuronal cell types at synapses. Recently studies
implicate that cadherins, calcium-dependent cell adhesion
molecules, are involved in synaptic formation, specifying
connections among different synapses and functional modulation
during early development. The molecular mechanisms
by which the classic cadherin mediate adhesion are beginning
to be elucidated. We have identified certain key residues
at the interface of N-cadherin molecules that participate in
cell-cell adhesion formation. In addition, two different, but
conserved cadherins are highly likely to form hetero-interaction
at adherent junction sites, which may increase the
diversity of synaptic connections. Furthermore, synaptic
modulation could be activated in either physiological or
pathological conditions, indicating a role for cadherins in
synaptic plasticity. Taken together, our data provide insights
into the mechanisms of formation of adherens junctions,
synaptic targeting, and modulation of synaptic activity.
SY-01-2
Basic mechanisms and prevention of prenatal brain
damage
P. Evrard
Service de Neurologie Pediatrique et des Maladies Metaboliques,
Faculte de Medicine Xavier-Bichat, Hospital
Robert-Debre, Paris, France
Abstract not submitted
SY-01-3
Neurophysilogical analysis of periventricular
leukomalacia in preterm infants
A. Okumura, K. Watanabe, F. Hayakawa, T. Kato
Department of Pediatrics, Nagoya University Graduate
School of Medicine, Nagoya, Japan
PVL is an important cause of cerebral palsy in preterm
infants. Serial EEG recordings are not only of great prognostic
value but also useful to determine the timing of brain

Abstracts 351
injuries in preterm infants with PVL. Flash visual evoked
potentials (FVEP) are also useful for the diagnosis of PVL
and the analysis of its pathogenesis. EEG abnormalities can
be divided into two categories; acute and chronic stage
abnormalities. Acute stage abnormalities mainly consist of
changes in continuity, frequency and voltage. Acute stage
abnormalities appear immediately after acute brain insults.
Chronic stage abnormalities consist of dysmature and disorganized
patterns. Disorganized pattern refers to abnormal
deformed background activities without findings of acute
depression. They are usually recognized in infants with
PVL and indicate irreversible brain damages. As to FVEP,
we paid attention to the waveform of the negative peak with
a mean latency of 300 ms (N300). Abnormal FVEP findings
were categorized into three patterns; absent VEP, abnormal
wave form, and delayed latencies. Our recent studies
revealed that acute stage abnormalities were seen in 60%
of infants with cystic PVL, chronic stage abnormalities in
90%, and FVEP abnormalities in 80%. These abnormalities
were always recognized before cystic changes appeared on
ultrasonography. Evolutional EEG changes suggested that
the timing of injury was antenatal or perinatal. It is remarkable
that EEG abnormalities usually preceded FVEP
abnormalities. This suggested that the interval of several
days will exist from a brain insult to irreversible brain
damages in infants with PVL.
SY-01-4
Pathogenesis, plasticity and prevention of perinatal
brain damage
A. Oka, A. Hirayama, S. Takashima
Division of Child Neurology, Institute of Neurological
Sciences Tottori University School of Medicine, Japan
Infants born as prematures are highly susceptible to
ischemic white matter damage, i.e. PVL. Currently, PVL
is regarded as a main type of white matter injury, causing
cerebral palsy and intellectual deficits. In the pathogenesis
of PVL, the development of vasculature in deep white
matter as well as the vulnerability of differentiating glial
progenitor cells is an important predisposing factor, and the
cerebral hypoperfusion associated with inflammatory
factors is causally related to PVL. We have evaluated
neuropathology of PVL. While cystic lesions in periventricular
areas are characteristic, the lesions are often widelyspread,
reaching to deeper white matter in extremely lowbirth-weight
infants. Immunohistochemical studies have
revealed the early stage of axonal injury is accompanied
by accumulation of amyloid precursor protein, which is
carried by axonal transport. Axons at PVL lesions also
express adhesion molecules, such as L1 or HNK-1,
which are up-regulated by axonal growth during fetal
period, or GAP-43, a marker of axonal re-growth. Later,
focal or diffuse accumulation of neurofilament in swollen
axons persists, indicating axonal damage in PVL. These
changes indicate early axonal damage, followed by reactive
or repair mechanisms. In terms of glial cells, we found
increased immunoreactivity of nestin and myelin transcription
factor (MyT1) in PVL brains of later stage. Nestin is
expressed by multipotential stem cells, and intense labeling
is seen in early fetal white matter. Surrounding PVL
lesions of chronic stage, nestin-immunoreactive astrocytes
as well as axons are present, suggesting tissue repair.
MyT1 is a zinc-dependent DNA-binding protein, and
expressed in early progenitors of oligodendrocyte cell lineage.
In the chronic stage, there is an increase in MyT1-
immunoreactive cells, which are distributed around necrotic
foci. The increase in immature oligodendrocytes near
PVL lesions also indicates compensatory mechanism,
probably leading to plasticity.
SY-2
Neuromuscular Disorders
SY-02-1
The molecular genetics of spinal muscular atrophy:
recent progress and therapeutic implication
C.H. Wang
Department of Child Health, University of Missouri, One
Hospital Drive, Columbia, MO, USA
Spinal muscular atrophy (SMA) is a neuromuscular
disorder characterized by degeneration of the spinal cord
motor neurons. SMA is phenotypically heterogeneous and
has been classified into three subtypes. The gene for the
three types of autosomal recessive SMA was mapped to
chromosome 5q13 in 1990. Further progress in the field has
led to the identification of the survival of motor neuron
(SMN) gene in 1995. The SMN gene is presented in two
highly homologous copies: the telomeric copy SMN1, and
the centromeric copy SMN2. Over 95% of the SMA
patients harbor a homozygous deletion or mutation of the
SMN1 gene but retain at least one copy of the SMN2 gene.
The SMA severity is correlated with the copy number of
the SMN2 gene and its ability to produce intact SMN
messenger RNA (mRNA) and protein within the SMA
patients. The SMN gene plays a crucial role in the cellular
mRNA metabolism. A mouse model of SMA has been
generated that exhibits both clinical and pathological
resemblance of human SMA. The therapeutic application
of these findings includes identifying ways to augment the
production of intact SMN protein by SMN2 gene in order
to ameliorate the clinical symptoms of SMA. Several transcription
factors have been shown to induce the production
of intact SMN protein by interacting with crucial
sequences on the exon 7 of SMN2 gene. Large scale
drug screen has been started in an attempt to identify pharmacological
compounds which can stimulate in vivo
production of SMN protein. Progress in this area will be
reported.

352
Abstracts
SY-02-2
Recent advances in congenital muscular dystrophies
I. Nonaka, Y.K. Hayashi, H. Ishikawa, K. Sugie, F.
Uematsu, I. Nishino
National Center of Neurology and Psychiatry, Tokyo, Japan
Congenital muscular dystrophy (CMD) has been classified
into two major groups: Fukuyama type CMD (FCMD)
and classical CMD (non-FCMD). FCMD is prevalent in the
Japanese, comprising 1–2/100 000 population and characterized
clinically by muscle hypotonia and weakness from
early infancy and central nervous system abnormalities. The
gene for FCMD has been mapped to 9q31 and the gene
product, fukutin, consists of 461 amino acids, but its function
is unknown. Since immunoreactivity to alpha-dystroglycan
was deficient, the fukutin gene mutation is probably
related to altered glycosylation, which may lead to disruption
of the transmembranous molecular linkage. The classical
form of non-FCMD has been further subdivided into
several distinct forms, including merosin deficiency,
muscle-eye-brain (MEB) disease and congenital atonicsclerotic
muscular dystrophy (Ullrich’s disease). We have
studied nine patients with Ullrich’s disease clinically and
pathologically. From early infancy, all patients had proximal
joint contractures, including limited neck flexion and
kyphoscoliosis, and distal joint hyperlaxity. Although four
patients learned to walk, the disease is slowly progressive.
One patient had a complete collagen VI deficiency immunohistochemically
and compound heterozygous mutation in
the COL6 gene. The other eight had partial collagen VI
deficiency immunohistochemically.
SY-02-3
Mitochondrial myopathies
S. DiMauro
Columbia University College of Physicians and Surgeons,
New York, NY, USA
The ‘molecular era’ of mitochondrial myopathies started
in 1988 with the discovery of the first mutations in mitochondrial
DNA (mtDNA). The small circular molecule of
mtDNA turned out to be a Pandora’s box of pathogenic
mutations, which have been associated with a bewildering
variety of clinical syndromes. From a genetic point of
view, mtDNA mutations can be divided into two groups:
those affecting mitochondrial protein synthesis in toto; and
those affecting specific proteins of the respiratory chain. In
recent years, interest has shifted towards Mendelian genetics,
a shift justified by the fact that most proteins of the
respiratory chain are encoded by nuclear DNA (nDNA). In
addition, proper assembly and function of respiratory chain
complexes requires numerous nDNA-encoded ancillary
proteins, whose mutations cause mitochondrial encephalomyopathies
(‘murder by proxy’). Because mtDNA is the
slave of nDNA, numerous nuclear factors are needed to
insure mtDNA replication and to preserve mtDNA integrity.
Mutations in these factors impair intergenomic
communication and result in syndromes characterized by
mtDNA multiple deletions, mtDNA depletion, or both
together. Within the past year, mutations in four genes
have been associated with autosomal dominant or recessive
forms of progressive external ophthalmoplegia and multiple
mtDNA deletions, and mutations in two genes have
been associated with mtDNA depletion syndromes. Finally,
new pathogenetic concepts are being introduced. For
example, alterations of the respiratory chain may be due
to altered composition of the inner mitochondrial
membrane (IMM) lipid milieu. This is the case of Barth
syndrome, an X-linked recessive mitochondrial myopathy/
cardiopathy characterized by severe deficiency of cardiolipin,
the major phospholipid of the IMM.
SY-02-4
Lipid storage myopathies
I. Tein
Division of Pediatric Neurology, Hospital for Sick Children,
Canada
Genetic defects in fatty acid oxidation (FAO) are an
important group of infancy- or childhood-onset disorders
because they are potentially rapidly fatal and a source of
major morbidity. FAO defects encompass a spectrum of clinical
disorders including progressive lipid storage myopathy,
episodic myoglobinuria, neuropathy, pigmentary retinopathy,
progressive cardiomyopathy and recurrent hypoglycemic,
hypoketotic encephalopathy or Reye-like syndrome
with secondary seizures and potential developmental delay.
Recurrent myoglobinuria may be seen in carnitine palmitoyltransferase
II, carnitine acylcarnitine translocase, long- and
very long-chain acyl-CoA dehydrogenase, short-chain l-3-
hydroxyacyl-CoA dehydrogenase and long-chain l-3-hydroxyacyl-CoA
dehydrogenase or trifunctional protein deficiencies.
As all of the known FAO defects are inherited as
autosomal recessive diseases, there is oftentimes a family
history of sudden unexpected death or sudden infant death
syndrome (SIDS) in siblings. Early recognition and prompt
initiation of therapy, as well as institution of appropriate
preventive measures, may be lifesaving and significantly
decrease long-term morbidity, particularly with respect to
neurological sequelae. There are now at least 17 recognized
defects in FAO, most of which have been diagnosed in the
last 15 years. With significant biomedical advances and
increased index of clinical suspicion, there has been a rapid
increase in the number of diagnosed cases. In a recent survey
from the Pennsylvania newborn screening program, the incidence
of medium-chain acyl-coenzyme A dehydrogenase
deficiency was found to be as high as 1 in 8930 live births.
In order to provide the background for understanding these
disorders, an overview will be given of the pathway of FAO

Abstracts 353
and the central role of carnitine. This will be followed by a
description of the hallmark clinical, biochemical and pathological
features of specific genetic defects in FAO,
approaches to diagnosis, and current treatment methodologies.
SY-3
Molecular Basis of Neurological Diseases in Children
SY-03-1
Identification and characterization of novel mutations of
the aspartoacylase gene in non-Jewish patients with
Canavan disease
E.H. Kolodny a , B. Zeng a , Z.-H. Wang a , L.A. Ribeiro a ,P.
Leone b , S.-J. Kim a , G.M. Pastores a , S. Raghavan a , E. Ong a
a Department of Neurology, New York University School of
Medicine, New York, NY;
b Division of Neurosurgery,
Department of Surgery, University of Medicine and Dentistry
of New Jersey, Camden, NJ, USA
Canavan disease (CD) is a severe progressive autosomal
recessive neurodegenerative disorder, caused by mutations
in the aspartoacylase (ASPA) gene. While two predominant
mutations account for approximately 97% of the
mutant alleles in Ashkenazi Jewish individuals with higher
prevalence of CD, this disease has also been diagnosed in
other diverse ethnic groups. However, mutations in the
ASPA gene among non-Jewish patients are different and
more diverse. In the present study, the ASPA gene was
analyzed in 22 unrelated non-Jewish patients with CD, and
24 different mutations were found. Of these, 14 novel
mutations were identified, including five missense mutations
(E24G, D68A, D249V, C152W, H244R), two
nonsense mutations (Q184X, E214X), three deletions
(923delT, 33del13, 244delA), one insertion mutation
(698insC), two sequence variations in one allele
([10T ! G; 11insG]), an elimination of the stop codon
(941A ! G, TAG ! TGG, X314W), and one splice acceptor
site mutation (IVS1-2A ! T). The E24G mutation
resulted in substitution of an invariable amino acid residue
(Glu) in the first esterase catalytic domain consensus
sequence. The IVS1-2A ! T mutation caused the retention
of 40 nucleotides of intron 1 upstream of exon 2. The
results of transient expression of the mutant ASPA
complementary DNA (cDNA) containing these mutations
in COS-7 cells and assay for ASPA activity of patient
fibroblasts indicated that these mutations were responsible
for the enzyme deficiency. Our studies expand the spectrum
of novel mutations in non-Jewish patients with CD,
and are useful for the accurate diagnosis of CD in these
patient populations, for prenatal diagnosis of CD in informative
families, and for detection of carriers in affected
families.
SY-03-2
Facioscapulohumeral muscular dystrophy (FSHD)
N. Raksadawan
Siriraj Hospital, Mahidol University, Bangkok, Thailand
Facioscapulohumeral muscular dystrophy (FSHD), one
of the common muscular dystrophy, is transmitted as an
autosomal dominant in majority of cases while few show
a de novo mutation. It has a characteristic and distinctive
distribution of weakness. Symptoms usually begin in childhood
with weakness, which affects predominantly facescapular
stabilizer-humeral muscles and rarely in early
infantile onset associated with generalized hypotonia.
Retinal and cochlear diseases are occasionally associated
defects. Its molecular pathogenesis is still unknown, though.
FSHD locus is known on the subtelomeric region of human
chromosome 4q. Mutation is the result of a deletion of
multiple copies of 3.3-kb tandem repeats (D4Z4) which
cause a position variegation effect on more proximal
DNA. The molecular diagnosis based upon the detection
of the short fragment of the tandem repeats, using p13E11
probe after EcoRI, EcoRI/BlnI digestion, is possible in
majorities of cases. Because of unknown underlying muscle
injury mechanism, curative therapy is not yet available.
However, few clinical trials of symptomatic treatments
have been tried.
SY-03-3
Duchenne muscular dystrophy: from gene diagnosis to
molecular therapy
M. Matsuo
Division of Molecular Medicine, Kobe University Graduate
School of Medicine, Hyogo, Japan
Duchenne and Becker muscular dystrophies (DMD/BMD)
are X-linked muscular dystrophies. The isolation of the
defective gene in DMD/BMD has led to a better understanding
of the disease process and has promoted studies regarding
the application of molecular therapy. Due to frame-shifting
mutations in the DMD gene that cause dystrophin deficiency,
DMD patients suffer from lethal muscle degeneration. In
contrast, mutations in the allelic Becker muscular dystrophy
(BMD) do not disrupt the translational reading frame,
thereby resulting in a less severe phenotype. The purpose
of this report is to present the recent progress made in this
area of research, with particular reference to dystrophin
Kobe, which is caused by exon skipping during splicing
due to the presence of an intra-exon deletion. On the basis
of results from the molecular analysis of dystrophin Kobe, we
proposed a novel way of molecular therapy for DMD wherein
antisense oligonucleotides transform DMD into a milder
phenotype by inducing exon skipping. We explored a genetic
therapy aimed at restoring the reading frame in muscle cells
from a DMD patient through targeted modulation of dystro-

354
Abstracts
phin pre-mRNA splicing. In myotube cultures from a DMD
patient carrying an exon 20 deletion, the induced skipping of
exon 19 in mRNA led to the production of in-frame dystrophin
mRNA and internally deleted dystrophin in myotubes.
Our results provide evidence of restoration of dystrophin
expression from the endogenous gene in DMD patientderived
muscle cells. In addition, current proposals of other
molecular therapies for DMD are discussed.
SY-03-4
Signal transduction defects: an emerging family of
genetic encephalopathies
J. Jaeken a , K. Freson b , N. Goemans a , P. De Cock a , C. Van
Geet a,b
a Department of Pediatrics and b Center for Molecular and
Vascular Biology, University Hospital Gasthuisberg,
University of Leuven, Leuven, Belgium
Mutations in the alpha subunit of the Gs proteins
(encoded by GNAS1 located in an imprinted region on
chromosome 20q13.12–13) cause rare endocrine disorders.
The GNAS1 gene also encodes XL-GNAS1, which is
paternally expressed and maternally methylated. Using a
modified platelet aggregation test (Freson et al., Thromb
Haemost 2001;86:733–738) to evaluate Gs function we
identified six patients (from four families) with an internal
repeat extension of the XL-GNAS1 exon 1 on the paternal
allele. These patients had mainly a neurological syndrome
(psychomotor retardation, hypotonia, behaviour disturbances,
movement disorders) as well as variable dysmorphy
(mainly of face and hands) and, in only one, a
clinically important bleeding tendency. This defect was
associated with over expression of GNAS1 and increased
cAMP levels in platelets and fibroblasts upon stimulation.
In four patients this defect was combined with a polymorphism
in G-b 3 . (Jaeken et al., J Inherit Metab Dis
2000;23(Suppl. 1):205). In two other patients (sisters)
with severe psychomotor retardation, axial hypotonia,
hyporeflexia, overgrowth, thrombocytosis and hypercholesterolemia
a maternally inherited G-b 3 null-allele associated
with the same G-b 3 polymorphism on the paternal allele,
was identified. One of them died at the age of 4 years.
Conclusion: G protein defects can be associated with
neurological syndromes. Platelet function analysis is a
valuable tool in the identification of these (and other)
signal transduction defects.
SY-03-5
Gene therapy/cell therapy for genetic neurological
disorders
Y. Eto, T. Ohashi
Department of Pediatrics/Department of Gene Therapy,
Institute of DNA Medicine, Tokyo Jikei University School
of Medicine, Tokyo, Japan
Among various genetic diseases, we focused to lysosomal
storage diseases (LSD) which are caused by a genetic
deficiency of lysosomal enzyme and accumulates various
compounds in lysosomes. Recently, LSD patients can be
treated by: (1) enzyme replacement therapy for Gaucher
disease, Fabry disease, Hunter syndrome, Pompe disease
and Hurler syndrome. The enzyme replacement therapy is
limited to treat in non-neurological form of LSD. (2) Bone
marrow transplantation (BMT) is also potentially important
therapy to treat LSD since BMT may treat neurological
involvement of LSD, if treated in early period. (3) Cell
therapy/gene therapy are most promising treatment in
future. We are trying to treat LSD using mucopolysaccharidoses
(MPS) VII model mice and twitcher mice (model
for human Krabbe disease). Using these model mice, we
tried to treat these mice with adenovirus vector by intravenous
administration or intraventricular administration.
Visceral involvement in MPS VII mice was successfully
treated by adenovirus administration at least for 1 month.
In twitcher mice, intraventricular administration of adenovirus
showed reduced number of globoid cells in CNS,
which indicated that the CNS involvement could be treated
with gene therapy. Many LSD patients show neurological
disorders, since these cells can be converted into neuronal
cells in a certain condition. These results suggest that
mesenchymal cells can be used fro the treatment of CNS
involvement in LSD.
SY-4
Epilepsies and Related Disorders in Infancy, Childhood
and Adolescence
SY-04-1
Epileptogenic malformations of the cerebral cortex:
classification and genetic mechanisms
R. Guerinni
Department of Child Neuropsychiatry, University of Pisa,
Italy
Abstract not submitted
SY-04-2
Seizure induced brain injury in immature rat: short
term and long term
Y.-W. Jiang, G.-J. Zhang, H.-Y. Cao, X.-R. Wu
Department of Pediatrics, Peking University First Hospital,
Beijing, China
Objective: To study seizure induced brain injury and its
mechanism in immature rats. Method: We established two
seizure models: flurothyl inhalation induced seizure (Postnatal
day 15) (in vivo model) and magnesium-free culture
induced epileptiform discharge in cultured rats embryo
cortical neurons (in vitro model). The apoptosis and cellu-

Abstracts 355
lar MTT uptake were investigated as the indices of shortterm
brain injury, while the mossy fiber sprouting, behavior
and learning ability, and susceptibility to seizure-induced
brain injury in adulthood were observed as the indices of
long-term brain injury. We also investigated the neuron
NMDA receptor (NR) expression and intracellular calcium
after recurrent seizures, in order to find some clue of the
mechanism of the seizure-induced brain injury. Results:
From short time observation after seizure, we found that
the expression of NR subunits-NR1, NR2A protein upregulated
in hippocampus and cerebral cortex in vivo;
meanwhile, the cellular MTT uptake decrease, intracellular
calcium increase and were observed in cultured cortical
neurons. However, we had not found the obvious neuron
apoptosis increase. In long-term study, we found that the
susceptibility to seizure-induced brain injury, such as apoptosis,
mossy fiber sprouting, and learning and memory
defect after the second seizure in adulthood, were increased
in rats that had seizure experience during development,
either febrile convulsion or the kainite-induced convulsion.
Conclusions: The seizure induced brain seizure in developmental
brain were relatively less serious and lethal, but it
will influence the brain for a long time, which will made
the brain more susceptible to new pathogenic stimulus in
the adulthood.
SY-04-3
Idiopathic focal epilepsies in infancy
F. Vigevano
Ospedale Pediatrico Bambino Gesù, IRCCS, Roma, Italy
The 1989 classification of epilepsies and epileptic
syndromes (Commission, 1989) includes among the idiopathic
localization-related epilepsies the occipital form and
that with centrotemporal spikes, both with onset during
childhood, and among generalized epilepsies familial and
non-familial neonatal convulsions and benign myoclonic
epilepsy of infancy. In recent years, numerous publications
have reported localization-related epilepsy with onset
during early infancy, idiopathic aetiology and favorable
outcome. In 1963, Fukuyama reported cases occurring in
the first 2 years of life that were characterized by partial
seizures, absence of a etiologic factors and benign
outcome. Later, other reports of this clinical entity studied
the localization and semiology of seizures (Watanabe et al.,
1987, 1990, 1993), the prognosis (Sugiura et al., 1983), and
the presence or absence of familial occurrence (Vigevano
et al., 1990; Vigevano et al., 1992; Vigevano et al., 1994).
In particular, Watanabe and coworkers on several occasions
described cases with partial seizures of a different
type, proposing the term benign partial epilepsy of infancy
with complex partial seizures (BPE with CPS) and benign
partial epilepsy of infancy with secondarily generalized
seizures (BPE with SGS). Most of these cases were not
familial. Vigevano and coworkers directed attention to
the presence of cases with a family history of convulsions
with benign outcome during infancy, with autosomal dominant
inheritance, suggesting the term benign infantile
familial convulsions (BIFC). Benign infantile convulsions
are divided now into familial and non-familial forms, even
though the two forms can overlap. Genetic studies in familial
forms led to the identification of a marker on chromosome
19 (Guipponi et al., 1997). This was not confirmed by
later studies, therefore genetic heterogeneity was hypothesized
(Gennaro et al., 1999). Recently Malacarne (Malacarne
et al., 2001) studying eight Italian families with
BIFC mapped a novel locus on chromosome 2. In 1997,
Szepetowski (1997) described the association between
BIFC and variably expressed paroxysmal choreoathetosis.
Following the identification of a specific marker on chromosome
16, this entity constitutes a variant of the familial
forms, called infantile convulsions and choreoathetosis.
However, the chromosome 16 marker may account for
more than this particular phenotype (Caraballo et al.,
2001). Bureau and Maton (1998), Bureau et al. (1998)
and Capovilla and Beccaria (2000) described another
form of benign epilepsy with partial seizures, onset in
infancy or early childhood and benign outcome. In these
children, seizures did not present in clusters; rather they
were quite sporadic with prevalently complex partial type
semiology, and without automatisms. The EEG findings
showed peculiar interictal anomalies characterized by
central and vertex spikes or spikes and waves only evident
during sleep.
SY-04-4
Benign focal epilepsies in childhood and adolescence
N. Fejerman
Hospital de Pediatria ‘J.P. Garrahan’, Department of
Neurology, Combate de los Pozos 1881, 1245 Buenos
Aires, Argentina
Abstract not submitted
SY-04-5
Lafora’s progressive myoclonus epilepsy: clinical and
genetic advances
A.V. Delgado-Escueta b , S. Ganesh a , T. Suzuki a ,S.
Francheschetti c , C. Riggio c , G. Avanzini c , A. Rabinowicz d ,
S. Bohlega e , J. Bailey b , M.E. Alonso f , A. Rasmussen f , A.E.
Thomson d , A. Ochoa f , A.J. Prado f , M.T. Medina g ,K.
Yamakawa a
a Laboratory for Neurogenetics, RIKEN Brain Science Institute,
Wako-shi, Japan;
b Epilepsy Genetics/Genomics
Laboratories, Comprehensive Epilepsy Program, UCLA
School of Medicine and VA GLAHS West Los Angeles Medical
Center, Los Angeles, USA; c Instituto Nazionale Neurologico,
Besta, Milano, Italy;
d FLENI Medical Center,
Buenos Aires, Argentina; e King Faisal Medical Center,

356
Abstracts
Riyahd, Saudi Arabia; f National Institute of Neurology and
Neurosurgery, Mexico City, Mexico; g Direccion de Investigation
Cientifica, Universidad National Autonoma de
Honguras, Tegulcigalpa, Honduras
Mutations in the EPM2A gene encoding a dual-specificity
phosphatase (laforin) cause an autosomal recessive
fatal disorder called Lafora’s disease (LD) classically
described as an adolescent onset stimulus sensitive
myoclonus, epilepsy and neurologic deterioration. We
present correlation between mutations in EPM2A and
phenotypes of 22 patients (14 families). In addition to
classical LD associated mainly with mutations in exon 4
(P ¼ 0:0007), we identified atypical LD with childhood
onset dyslexia and learning disorder followed by epilepsy
and neurologic deterioration associated mainly with mutations
in exon 1 (P ¼ 0:0015). To understand the two
subsyndromes better, we investigated the effect of five
missense mutations in the carbohydrate binding domain
(CBD-4; coded by exon 1) and three missense mutations
in the dual phosphatase domain (DSPD; coded by exons 3
and 4) on laforin’s intracellular localization in HeLa cells.
Expression of three mutant proteins (T194I, G279S and
Y294N) in DSPD formed ubiquitin positive cytoplasmic
aggregates suggesting they were folding mutants set for
degradation. In contrast, none of the three CBD-4 mutants
showed cytoplasmic clumping. However, CBD-4 mutants
W32G and R108C targeted both cytoplasm and nucleus
suggesting that laforin had diminished its usual affinity
for polysomes. Our data, thus, represents the first report
of a novel childhood syndrome for LD. Our results also
provide clues for distinct roles for the CBD-4 and DSP
domains of laforin in the etiology of two subsyndromes
of LD. To study the ontogenesis of LD pathology and
laforin functions, we disrupted the Epm2a gene in mice.
Supporting the concept of childhood onset or atypical
human LD, homozygous null mutants developed widespread
degeneration of neurons, most of which occurred
in the absence of Lafora bodies, as early as 1–2 months
of age. Dying neurons characteristically exhibited swelling
in the endoplasmic reticulum, Golgi networks and mitochondria
in the absence of apoptotic bodies or fragmentation
of DNA. As Lafora bodies became more prominent at
4–12 months, organelles and nuclei were disrupted. The
Lafora bodies, present both in neuronal and non-neural
tissues, were positive for ubiquitin and advanced glycation
end products only in neurons, suggesting different pathological
consequence for Lafora inclusions in neuronal
tissues. Neuronal degeneration and Lafora inclusion bodies
predate the onset of impaired behavioral responses, ataxia,
spontaneous myoclonic seizures and EEG epileptiform
activity. Our results suggest that LD is a primary neurodegenerative
disorder that may utilize a non-apoptotic
mechanism of cell death.
SY-5
Neuroimaging
SY-05-1
Introduction: focused on functional neuroimaging
K. Iinuma
Tohoku University School of Medicine, Sendai, Japan
During the last two decades of the 20th century, various
neuroimaging techniques have strikingly developed.
Among various neuroimaging methods, functional imagings
give us valuable information about the pathophysiological
mechanisms and condition of neurological disorders. In this
symposium, the several neuroimaging techniques and their
applications will be explained. PET as relatively early
developed imaging method is recently shifting to understand
the receptor binding or brain metabolism. The functional
MRI will give us more detailed mapping of the brain
for fine functions by the development of various methods of
tasks. Magnetic resonance spectroscopy can give us the
information about characteristics of the tissues and/or cells
of the brain, moreover is capable to give the information on,
for example, GABA transmission, etc. Optical topography
recently has been developed by means of near-infrared spectroscopy
(NIRS). This method is multichannel NIRS that
makes spacial expression, and has an advantage of excellent
time resolution. This may have a possibility to analyze the
various phenomena concerning of the brain function, such
as epileptic seizures, motor and mental activity, etc.
SY-05-2
Applications of PET scanning in pediatric neurology
H.T. Chugani
Division of Pediatric Neurology, Positron Emission Tomography
(PET) Center, Children’s Hospital of Michigan,
Wayne State University, Detroit, MI, USA
The clinical role of PET scanning is mainly in localization
of epileptic foci for surgical treatment in refractory epilepsy.
PET tracers used in epilepsy include 2-deoxy-2 ( 18 F) fluorod-glucose
(FDG) and various ligands for evaluating neurotransmitter
function. In temporal lobe epilepsy, interictal
FDG-PET identifies areas of decreased glucose utilization
that correspond to epileptogenic areas (sensitivity: 80–
90%). In nonlesional extratemporal lobe epilepsy, FDG-
PET provides useful lateralization and localization data to
guide the placement of intracranial electrodes. Neurophysiological
correlations based on coregistration of MRI, PET and
subdural electrodes indicate that the seizure onset zone typically
lies in the periphery or boundary of the cortical hypometabolism
rather than within the hypometabolic zone. Our
data also show that the size of the hypometabolic zone
increases along the major propagation pathways as a function
of duration of intractable epilepsy, thus supporting the

Abstracts 357
concept of network growth and secondary epileptogenesis
proposed by Morrell. In a number of childhood epilepsy
syndromes characterized by generalized seizures, such as
infantile spasms and Lennox-Gastaut syndrome, resection
of focal PET abnormalities corresponding to focal ictal and
interictal EEG abnormalities is associated with improved
seizure and cognitive outcome. In PET studies on patients
with infantile spasms, there is evidence of complex corticosubcortical
interactions believed to be important in an agedependent
secondary generalization of focal cortical
discharges to result in the typical spasms. Only about 20%
of patients with cryptogenic infantile spasms’ show a single
PET focus amenable to resection. The other 80% of patients
show more than one focus, and most of these will not be
optimal surgical candidates. Some of these infants will benefit
from the use of newer PET tracers (see below), which may
reveal the primary seizure focus. The finding of bilateral
symmetric hypometabolism suggests a nonlesional etiology
for the spasms and is a useful indication to pursue further
neurogenetic/neurometabolic evaluation rather than a surgical
approach. In children with unilateral Sturge-Weber
syndrome, we have shown that early and rapid loss of functional
activity in the affected hemisphere is associated with a
better cognitive outcome than slow progression, presumably
because early demise of the affected hemisphere allows the
intact hemisphere to undergo reorganizational changes early
in the course of the disease. Recent studies using other PET
tracers have attempted to provide a more specific and accurate
assessment of seizure foci. 11 C-flumazenil labels central
benzodiazepine receptors and is particularly useful in showing:
decreased receptor binding in medial temporal sclerosis,
dual pathology, perilesional epileptogenic zones, seizure
onset zones, and potential secondary epileptic foci.
11 C-
alpha-methyl-l-tryptophan (AMT), an analogue of tryptophan,
traces serotonin synthesis and kynurenine pathways
in the brain. AMT-PET in patients with epilepsy demonstrate
focally increased uptake in cortical regions of epileptogenesis
interictally. In children with tuberous sclerosis and
intractable epilepsy, focal increase in AMT uptake is seen
in the region of epileptogenic tubers, but not in non-epileptogenic
tubers. Other ligands, which label histamine, opioid
and serotonin receptors have also been applied in patients
with epilepsy. Ontogeny of brain glucose metabolism,
GABA A receptors and serotonin synthesis has been studied
in children using PET. These studies have shown characteristic
developmental changes, which are disturbed in various
disorders. For example, autistic children do not undergo the
normal transient developmental increase of serotonin synthesis
seen in normal children. Focal patterns of abnormal
tryptophan uptake are also seen in autistic children. Glucose
metabolism PET studies in newborns show the very early
functional maturation of limbic structures believed to be
involved in infant attachment. Children adopted from Romanian
orphanages show disturbances of glucose metabolism in
some of these same regions. The potential of PET scanning in
the study of pediatric neurological disorders is enormous, but
this technique remains under utilized in children for a variety
of reasons.
SY-05-3
Functional MRI (fMRI) in children
W.J. Logan
Division of Neurology, Department of Pediatrics, The
University of Toronto, The Hospital for Sick Children,
Toronto, Canada
It has been recognized for over a decade that function, in
addition to structure, can be examined with MRI. The detection
and localization of function with MRI depends on the
hemodynamic changes that are known to accompany
changes in neural activity. Most modern MR scanners
have the capability for fMRI studies. The use of fMRI in
children is appealing because it is non-invasive, has no
radiation effects, is potentially widely available, can be
repeated many times and provides high spatial resolution
localization of brain function. Drawbacks such as need for
patient restraint and confinement, susceptibility to movement
induced artifact, need for a cooperative patient, extensive
data analysis time required and poorer temporal
resolution than electromagnetic techniques place some
limits on the application of fMRI. With improvements in
technique and greater experience, these limitations and
other constraints are becoming less of a concern. fMRI activation
does occur in children; in older children, this resembles
that seen in adults. Children are very amenable to fMRI
study. The major clinical application of fMRI in children is
brain mapping of sensory, motor, language and possibly
memory functions for pre-surgical planning. Other clinical
applications include evaluation of the integrity of neurological
pathways and cerebrovascular reactivity. Potential
clinical applications include diagnosis of specific impairments
such as dyslexia, predicting recovery of function
after brain injury and localizing the epileptic focus. These
are the subjects of current research studies. Other areas of
research are determining the mechanisms of plasticity, the
effect of rehabilitation and development on patterns of activation
and development of new paradigms especially for
younger children. There will be advances in the study of
sedated patients, in combining electromagnetic and fMRI
studies and in improving the techniques of investigation
such as real time data analysis and improved motion control
and correction. With advances in the above areas one can
predict an increasing use of fMRI in children.
SY-05-4
Proton magnetic resonance spectroscopy in children
with acute brain injury
S. Ashwal
Department of Pediatrics, Loma Linda University School of
Medicine Loma Linda, CA, USA

358
Abstracts
Proton MRS is an emerging technology that allows for the
quantitative non-invasive assessment of regional brain metabolic
activity. MRS has been used to study a wide variety of
pediatric neurometabolic disorders, tumors and demyelinating
diseases. It has also been used to assess prognosis after
acute brain injury (perinatal asphyxia, near drowning, etc.)
and it is useful in localizing focal epilepsies. Brain metabolites
assessed with MRS and often expressed as ratios
include: (1) N-acetyl aspartate (NAA) found in neurons; (2)
creatine and phosphocreatine (Cre) that reflect energy metabolism;
(3) choline-containing compounds (Cho) released
during membrane disruption; (4) presence of lactate indicating
a disturbance in cerebral energy metabolism; and (5)
glutamate/glutamine that is a reflection of asphyxial injury.
Markedly reduced levels of NAA and increased lactate have
been seen in patients who ultimately suffer severe long-term
neurologic disability. Usually this can be seen 3–5 days after
injury and is a diffuse phenomenon present in cerebral gray
and white matter. Patients with malignant tumors are likely to
have moderate to marked increases in choline and reduced
NAA whereas patients with lower grade tumors may show
milder abnormalities. MRS is also useful in evaluating children
with traumatic brain injury (TBI) as is a new MR technique
known as susceptibility weighted imaging which is
more sensitive in detecting hemorrhagic lesions associated
with diffuse axonal injury that also show very abnormal MRS
patterns that correlate with outcome. Multivoxel MRS allows
non-invasive sampling of many brain regions. It is likely that
as MRS technology continues to improve, its use for the
diagnosis of many nervous system disorders of children
will increase.
SY-05-5
Diffusion tensor imaging
A. Ohnuma
Division of Pediatric neurology, Miyagi Prefectural Takutoh
Rehabilitation Center for Children, Miyagi, Japan
The recent development of diffusion tensor imaging
(DTI) has made it possible to visualize anatomic details of
the human brain and to provide quantitative measures of
fiber tract integrity and orientation. The evidence of brain
plasticity was confirmed via DTI and FMRI in the intellectually
normal five subjects (aged 8–26 years) with unilateral
extensive brain lesions, which developed in the prenatal
(cortical dysplasia one and encephalo-clastic lesion two),
perinatal (encephalo-clastic lesion 1) and neonatal (intracranial
bleeding) stage. Fractional anisotropy (FA) images
included whole brain axial images and coronal pyramidal
tract images at the brainstem. FMRI during the motor task
was performed by the affected hand grasping. DTI depicted
markedly reduced FA of the pyramidal tract of the affected
hemisphere in two cases, moderately reduced in two and
bilateral normal in one. FMRI showed ipsilateral activations
in two cases, bilateral in one and contralateral in one. The
mode of the reorganization of the pyramidal tracts seemed
to depend on the stage of the development of the hemispherical
brain damages and the state of the residual brain tissues
of the affected hemisphere. DTI was also examined in the
cases with various kinds of white matter disorders, such as
Pelizaeus-Merzbacher disease, X-linked adrenoleukodystrophy,
metachromatic leukodystrophy, Cockayne
syndrome and Fukuyama type congenital muscular dystrophy,
in comparing with normal controls. Quantitative
measures of diffusivity and FA were useful for evaluating
the white matter status of these disorders.
SY-6
Channelopathies
SY-06-1
Mutations of sodium channels in GEFS 1 and SMEI
K. Yamakawa
Laboratory for Neurogenetics, RIKEN Brain Science Institute,
Japan
GEFS 1 , a clinical subset of febrile seizures (FS), is characterized
by frequent episodes beyond 6 years of age (FS 1 )
and various types of subsequent epilepsy. Recent evidences
have indicated that the neuronal voltage-gated sodium channels
(SCN1A, SCN2A, SCN1B) are responsible for GEFS 1 .
Some of mutant channels showed slowed inactivation that
may lead to the augment of sodium ion influx, cause hyperexcitability
of neurons, and finally result in epileptic
seizures. SCN1A has been also reported to be mutated in
severe myoclonic epilepsy in infancy (SMEI). SMEI is an
extremely intractable epilepsy; normal development before
onset; seizures beginning during 1st year of life in the form
of generalized or unilateral febrile clonic seizures; secondary
appearance of myoclonic seizures, and associates with
ataxia and mental decline. SCN1A mutations in SMEI were
heterozygous and de novo, and most of them are frameshift
or nonsense. Mutations in GEFS 1 are all missense mutations,
and these suggest the distinct pathologies of GEFS 1
and SMEI even if SCN1A is responsible for both diseases.
In my talk, I will summarize the SCN1A mutations of
GEFS 1 and SMEI and discuss the genotype-phenotype
correlation as well as the predicted molecular pathology
of these diseases.
SY-06-2
Benign familial neonatal convulsions
S. Hirose, A. Mitsudome
Department of Pediatrics, School of Medicine, Fukuoka
University, Fukuoka, Japan
Benign familial neonatal convulsions (BFNC) is monogenic
epilepsy inherited via an autosomal dominant trait and
characterized by clusters of generalized and partial seizures

Abstracts 359
afflicting exclusively, if any, neonates, and remits spontaneously.
However, the incidence of subsequent epilepsy
later in life is also higher in individuals who suffer from
BFNC. Mutations of two KQT-like K 1 -channel genes, the
KCNQ2 and KCNQ3 genes, were identified as the underlying
abnormalities of BFNC. To date, some ten mutations
of KCNQ2 have been discovered while only two mutations
including one found in a Japanese pedigree with BFNC have
been identified in KCNQ3. All mutations were heterozygous.
KCNQ2 and KCNQ3 synergistically contribute to
the formation of the M-current, which controls the subthreshold
electroexcitability of neurons. Dysfunction of
either KCNQ2 or KCNQ3 can hence result in indistinguishable
BFNC phenotype. The exact pathomechanisms of agedependency
and propensity for future epilepsies in BFNC
remain to be determined. We have recently shown that
KCNQ K 1 -channels serve as a predominant inhibitory
system in CNS during neonates, since GABAergic-transmission
governs the inhibitory system afterwards. Thus,
deficient KCNQ K 1 -channels cause convulsions during
the neonatal period. We have recently suggested that
abnormalities of KCNQ2 and KCNQ3 are not necessarily
the only causes of BFNC phenotypes, but rather other
genes are probably involved in the pathogenesis of the
BFNC phenotype, i.e. further genetic heterogeneity underlies
this familial epilepsy. Deficiency of other ion channels
and their modulation may result in the BFNC phenotype as
well.
SY-06-3
Single gene mutation, dual phenotype, and differential
mechanisms in stargazer mutant
X.-X. Qiao
Department of Cellular Biology and Anatomy, Louisiana
State University Health Sciences Center, Shreveport, LA,
USA
Voltage-dependent calcium channels (VDCC) play a
key role in regulating a broad spectrum of cellular functions
in both developing and mature central nervous
system. While more neurological disorders have been
linked to mutations of these genes in humans, several
spontaneous mutant mouse strains have recently been
found to harbor different VDCC subunit mutations. Interestingly,
all of these mutants share a similar phenotype of
characteristic spike-wave epilepsy and ataxia. Investigation
of these mutants has furthered our understanding of
the role of VDCC genes in neuronal function and provided
more insights of the mechanisms underlying inherited
neurological diseases. Studies from one such mutant, stargazer,
which carries a defected VDCC g2 subunit, have
revealed differential cellular processes involved in different
brain regions. In the frontal brain, an autonomous
increase in cortical network excitability in vitro has been
correlated with the prolonged seizure activity in vivo.
Aberrant mossy fiber sprouting and increased neuropeptide-Y
expression in the hippocampus are subsequent to
the epileptic discharges. In the cerebellum, the only area
that is not invaded by the seizure activity, severe impairment
of motor coordination and sensory-motor learning is
correlated with regionally restricted molecular and cellular
defects. The abnormalities include altered cerebellar granule
cell migration, maturation, and synaptic transmission.
The underlying molecules are linked to the selective failure
of BDNF mRNA expression and AMPA receptor
synaptic targeting. These results revealed significant regional
specificity to the defects in stargazer brain. It provides
clear evidence that calcium channelopathies initiate
complex, region-specific, molecular defects in synaptic
signaling during brain development.
SY-06-4
GABA gene mutations in human epilepsies symposium
of channelopathies
I.E. Scheffer
Epilepsy Research Institute, University of Melbourne,
Austin and Repatriation Medical Centre, Monash Medical
Centre and Royal Children’s Hospital, Melbourne, Australia
GABA is the major inhibitory neurotransmitter in the
central nervous system. GABA receptors are heteropentameric
ligand-gated chloride channels. GABA receptors in
human brain most commonly comprise alpha subunits, beta
subunits and a gamma subunit. The alpha and beta subunits
can form a functional receptor alone. The gamma subunit
contributes to the benzodiazepine binding site and is essential
for benzodiazepine potentiation of action potentials.
Three mutations of the gamma 2 subunit gene of the
GABA A receptor (GABRG2) were identified in families
with GEFS 1 , one also had childhood absence epilepsy
(CAE). In vitro functional studies using two electrode
voltage clamp recordings of the mutations expressed in
functional GABA receptors showed marked attenuation
of the GABA-mediated current with two mutations. In
the third mutation, GABA current was not altered, however
benzodiazepine potentiation was abolished. Recently, a
family with Autosomal Dominant Juvenile Myoclonic
Epilepsy with a mutation of the alpha 1 subunit gene of
the GABA A receptor (GABRA1) was reported. In vitro
studies also showed reduction of the GABA activated
current. The range of mutations of GABA receptors in
the idiopathic epilepsies still remains to be explored. It is
likely that other subunit genes will be involved in the
idiopathic generalized epilepsies, including GEFS 1 . Genotype-phenotype
correlation will be essential to understanding
the neurobiology of these disorders. Eventually the
clinician may treat patients based on the rational understanding
of the interaction of a number of ion channel
mutations.

360
Abstracts
SY-06-5
Epileptic channelopathies in man and mouse
J. Noebels
Department of Neurology, Baylor College of Medicine,
Houston, TX, USA
Inherited disorders of ion channels are now the single
most common known cause of idiopathic generalized
epilepsy. Despite this rapid progress in gene discovery,
we are only beginning to understand their intriguing pathophysiology.
Channelopathy phenotypes arise in both the
developing and mature central nervous system, and show
remarkable clinical diversity, including tonic/clonic,
absence, and myoclonic epilepsy, as well as other episodic
neurological disorders. The mutations target both voltageand
ligand-gated channels, and produce disease by altering
the biophysical properties of the pore subunit or by modifying
auxiliary subunits that regulate membrane insertion,
modulation, and localization of the channel complex. In
almost all cases, the direct result is to prolong intrinsic
membrane depolarisation or impair synaptic transmission.
Recent studies in orthologous mouse models suggest that
the mechanisms for age-dependent epileptogenesis in these
inherited errors are more complex than initially realized.
Despite widespread expression, only certain neural
networks are vulnerable at various developmental stages,
presumably accounting for phenotypic diversity. The selectivity
is explained by the differential contributions of individual
channels to the excitability and firing behavior, and
by the presence of compensatory subunits. As one example,
generalized spike-wave absence epilepsy is seen in a
cluster of four mutant mouse models, each representing a
subunit of the brain voltage-gated calcium channel. In two
of these models (a1a and b4), the compensatory subunits
have been identified, and their absence in thalamic neurons
contributes to thalamocortical synchrony. We have recently
identified further downstream cellular plasticity in thalamic
low threshold calcium currents that may also directly
contribute to the seizure phenotype.
SY-7
What Happens to a Child at Risk for Developmental
Delay/Disability
SY-07-1
Introduction
S. Harel
Division of Pediatrics, The Institute For Child Development
and Pediatrics Neurology, Tel Aviv, Israel
Abstract not submitted
SY-07-2
School age outcomes of pre-school children diagnosed
with either global developmental delay or specific
language impairment
M. Shevell, A. Majnemer, R. Platt
Montreal Children’s Hospital-McGill University, Canada
The objective of this study is to describe prospectively the
school age (6–7 years) functional and developmental
outcomes in a cohort of children diagnosed previously
with either a global developmental delay or a specific
language impairment. Two cohorts have been assembled:
(a) global developmental delay (n ¼ 99); and (b) specific
language impairment (n ¼ 72). These cohorts were initially
assessed and diagnosed at a mean age of 37.6 and 43.3
months, respectively. Functional, developmental and
language outcomes were assessed in each cohort between
the ages of 6–7 years using a variety of measures. These
included the Battelle Developmental Inventory, Vineland
Adaptive Behavior Scale, WeeFIM, Child Health Questionnaire,
Parenting Stress Index, Child Behavior Checklist/4–
18, Expressive One-Word Picture Vocabulary Test and the
Peabody Picture Vocabulary Test-Revised. Outcomes on
each of these measures for each cohort in this ongoing
study will be described and co-related with factors identified
at initial intake to establish the possibility and strength of
prognostic variables.
SY-07-3
Cerebral visual disorders in children with brain lesions:
outcome and correlation with neuroimaging
G. Cioni, A. Guzzetta, F. Tinelli
Division of Child Neurology and Psychiatry, Stella Maris
Scientific Institute and University of Pisa, I Calambrone,
Pisa, Italy
Children with brain lesions of antenatal or perinatal
onset are at high risk for early disturbances of their visual
development, detectable since the first months of life by
means of new behavioural and electrophysiological techniques.
Several studies have reported that various aspects of
visual function, such as visual acuity, visual fields, optokinetic
nystagmus, fixation shift, and perception of movement
are often impaired in these children. A strong
correlation between the results of visual assessment, and
cognitive and motor development also has been also
reported, suggesting a crucial role for visual function in
the early development. Long-term follow-up of these children
have revealed subtle neuropsychological disorders.
Neuroimaging techniques, and brain MRI in particular,
are useful in predicting the occurrence and extent of visual
impairment. However, the correlation between imaging
findings and function is not always consistent. This is probably
due to the involvement of the complex extra-striatal

Abstracts 361
visual pathways and also to the plasticity of the developing
brain. Children often show less severe visual impairments
than adults with apparently similar brain lesions. Newer
MR techniques, particularly fMRI, can provide other
important contributions to this issue. These results are
important for understanding the advantages but also the
limitations, of plasticity and reorganization of the immature
visual brain.
SY-07-4
The study investigates long-term effects of slight
biological and slight socioeconomic risks on complex
achievement variables using two different data sets
G. Spie, C. Spiel, G. Sange, P. Wagner
Department of Child Neurology/Psychiatry, and Special
Education for Children and Adolescents-Hospital Klagenfurt,
Klagenfurt, Australia
On the one hand, observations collected in the Vienna
developmental study (VDS), on the other hand, the same
kind of data from the Viennese study ‘children at risk’
(VCRS) were used. The sample of the VDS study consisted
of 94 randomly selected children. The sample of the VCRS
study consisted of 129 children who were selected for slight
biological risks at birth. In addition a comparison sample
without known biological risks was drawn from the general
population. Needless to say that only compliant members of
the study were followed in the long run. The following risk
conditions were analyzed in both samples: biological risks,
socio-economic status, hospitalizations, severe strains and
life-events, and early occurring developmental variables. As
outcome variables cognitive competence (fluid and crystallized
intelligence) was collected at the age of 12 years, and
school achievement from the first to the fourth grade(ages
6–10) modeling the overall academic development during
primary school. Multiple regression analyses were used in
both studies to investigate the effects of risk conditions and
earlier developmental parameters on outcome variables
mentioned before.
SY-07-5
Neuropsychological outcome of children with
intrauterine growth retardation (IUGR)
Y. Leitner a , A. Fattal-Valevski a , R. Geva a , H. Bassan a ,H.
Goez a , A.J. Jaffa b , S. Harel a
a The Institute for Child Development and Pediatric Neurology
Unit, Division of Pediatrics, b Department of Obstetrics
and Gynecology, Lis Maternity Hospital, Tel Aviv Sourasky
Medical Center, Sackler Faculty of Medicine, Tel Aviv
University, Israel
Intrauterine growth retardation (IUGR) occurs in 3–10%
of all pregnancies, and is more prevalent in children with
neurocognitive disabilities. Our study was conducted to
characterize the neurodevelopmental and cognitive difficulties
specific to IUGR children and identification of early
risks and clinical predictors of these difficulties. Since
1990, a group of 320 children with IUGR has been
followed-up annually from pregnancy to school age, by
neurodevelopmental and psychological evaluation, and
data collected by socioeconomic, obstetric and neonatal
risk questionnaires. The risk questionnaires and neurodevelopmental
evaluations were all scored according to Prechtl’s
‘optimality concept’. Psychological evaluation was
performed by standard IQ tests. The data presented in this
study demonstrates the changes observed in the IUGR
group, versus the controls matched for gestational age and
socioeconomic status at three points of the follow-up, i.e.
age 3 (n ¼ 130), 6–7 years (n ¼ 110), and 9–10 years
(n ¼ 56). Significant differences in growth parameters
were found in all age groups. At all ages (3, 6–7 and 9–10
years), the score of the IUGR children was significantly
poorer (P , 0:005, P , 0:05, and P , 0:005, respectively)
on the neurodevelopmental score, than the matched
controls. At ages 6–7 and 9–10 years, the IUGR children
had lower IQ scores (P , 0:05; P , 0:005). No differences
were found in IQ scores at age 3. Statistically significant
items on the 3-year neurodevelopmental test that differed
IUGR children from controls were in the area of motor
coordination. At age 6–7, a specific profile of difficulties
in coordination, special maturation tasks and graphomotor
skills is typical of the IUGR children. At 9–10 years of age,
attention span, activity, coordination, timed coordination
performance, visuomotor dysfunction, language verbal
fluency and school achievements were typical for IUGR
children. The clinical parameters that best predict neurodevelopmental
outcome at 3 years were the cephalization
index [CI]: (head circumference [HC]/birth weight based
on the ‘brain sparing’ process expressing the severity of
the IUGR process) (P , 0:005) and the HC (P , 0:005).
At 6–7 years of age the best predictors were the neonatal
risk score (P , 0:005) and weight (P , 0:005). At 9–10
years, the best predictors were CI (P , 0:001) and weight
(P , 0; 005). The best predictors for IQ at age 3 years were
HC (P , 0:05) and maternal education (P , 0:005); at 6–7
years neonatal risk score (P , 0:001); HC (P , 0:005), and
maternal education (P , 0:001); and at 9–10 years the CI
(P , 0:005); HC (P , 0:005) and maternal education
(P , 0:005). At age 6–7 years neurodevelopmental
outcome and IQ score were worse in IUGR children with
neonatal complications than those without (P , 0:05;
P , 0:005, respectively). Children with IUGR diagnosed
prenatally had the same neuropsychological outcome as
those diagnosed at birth, probably due to early delivery
and careful perinatal and obstetric care. Preliminary conclusions
of this long-term follow-up: children with IUGR lag
behind in their somatic and neurocognitive development.
The neuropsychological items that most clearly differentiated
IUGR children from the controls were those requiring
motor coordination, visuomotor skills, attention span,

362
Abstracts
language and school achievements, hinting at later learning
disabilities. Clinical predictors of neurocognitive development:
CI and the neonatal risk predict neurodevelopmental
outcome. CI, neonatal risk score, HC, maternal education
and paternal occupation predict cognitive ability. Neurodevelopmental
outcome was worse in IUGR children with
neonatal risk. IUGR children diagnosed prenatally, despite
being at high risk, had the same outcome as the low-risk
children diagnosed only at birth, probably reflecting careful
obstetric and neonatal care. At a younger age, the biological
parameters have a greater impact on neurodevelopment,
while later environmental influences, such as maternal
education, appear to gain importance in cognitive performance.
SY-07-6
The outcome of early intervention in
neurodevelopmental disabilities
T. Velickovic
Ljubljana, Slovenia
Abstract not submitted
SY-8
Neurometabolic Disorders Update
SY-08-1
Screening for neurometabolic disorders
B. Wilcken
The Children’s Hospital, Westmead, New South Wales,
Australia
Screening implies pre-symptomatic diagnosis. One classical
criterion for screening in newborns is that there should be
benefit to the baby from early diagnosis. Newborn screening
began in the 1960s with a test for phenylketonuria, which had
been found to account for 1% of institutionalized mentally
retarded persons in western countries. Later, screening for
primary congenital hypothyroidism detected another cause
of mental retardation, and indicated iodine-deficiency areas
where endemic cretinism occurred. The new newborn
screening by tandem mass-spectrometry enormously
increases the potential for early detection of genetic metabolic
disorders, detecting amino acid, organic acid, and fatty
acid disorders in a single test. Screening newborns for untreatable
disorders is not favored, but as treatment becomes more
available the classes of disorder, which are tested for, will
expand. The more important neurometabolic disorders have
been hard to screen for, as many do not have suitable
biochemical markers in blood or urine, or common DNA
mutations. There is promise of a newborn test for lysosomal
storage disorders, and treatment by enzyme replacement
therapy is currently available or developing for many of
these. Some rare neurometabolic disorders that could likely
be detected by screening using modifications to current technology
include disorders of creatine deficiency, serine deficiency,
purines and pyrimidines, cholesterol synthesis,
carbohydrate deficient glycoprotein syndromes, and peroxisomal
disorders. It is important that each potential screening
initiative is carefully evaluated for evidence of benefit,
before widespread adoption.
SY-08-2
Postnatal and prenatal diagnosis of lysosomal storage
diseases
H.-P. Shi a , W.-M. Zhang a , Y.-F. Guo a , S.-M. Zhao b , N.-H.
Sun b
a Institute of basic Medical Sciences, CAMS, School of Basic
Medicine, PUMC, Beijing, China; b PUMC Hospital, Beijing,
China
Objective: LSD constitute a group of inherited metabolic
diseases, in which lysosomal acid hydrolases are deficient,
resulting in accumulation of the substrate of the affected
hydrolase within lysosomes. Since there is no definitive
treatment for LSD, except Gaucher disease, accurate prenatal
diagnosis is the only way to prevent the birth of affected
fetuses. Methods: Microanalysis of enzyme activity is
adapted from the Department of clinical genetics, Erasmus
University, Rotterdam, Netherlands. Fifteen kinds of methods
for enzyme assays were set up. Results: Since 1991, 202
index patients were diagnosed based on the characteristic
clinical manifestation and specific enzyme assay. Prenatal
diagnosis has been carried out in 43 pregnancies at risk of
LSD (13 mucopolysaccharidoses, seven G M1 gangliosidosis,
four G M2 gangliosidosis, seven metachromatic leukodystrophy,
five Gaucher disease, four Niemann-Pick disease and
three mucolipidosis) in early pregnancy, 11 affected fetuses
were detected. Gaucher disease (GD) is the most prevalent
LSD. We have performed gene analysis from 10 Chinese
patients with GD. Mutation L444P is the most frequent and
accounts for 40% of the alleles, which is associated with all
types of GD. The genotype of one case is L444P/L444P. His
mother had another pregnancy, the prenatal diagnosis was
carried out by both enzyme assays and polymerase chain
reaction (PCR)/restriction fragment length polymorphisms
(RFLP). The result is that the fetus is a heterozygote of GD.
Conclusion: Microanalysis of enzyme activity is a reliable
method for postnatal and prenatal diagnosis of LSD.
SY-08-3
Biology and therapy of Niemann-Pick disease, type C
M.C. Patterson
Columbia University, New York, USA
Niemann-Pick disease, type C (NPC), is an autosomal
recessive, progressive neurodegenerative disorder resulting
from mutations in NPC 1 (.95% of cases) or NPC2. The
NPC1 gene product is an integral membrane protein with

Abstracts 363
homology to the sterol-sensing domain of SREBP cleavage
cleavage – activating protein (SCAP) and hemimegalencephaly
(HMG) CoA reductase and to the Drosophila morphogen,
patched, and is expressed in a late endosomal
compartment. Cultured fibroblasts from NPC patients show
impaired intracellular vesicular trafficking, of multiple
macromolecular cargoes, leading to a net accumulation of
free cholesterol and glycosphingolipids in membrane bound
vesicles. The function of the NPC2 gene product (epididymal
secretory protein E1 (hE1)) in the brain is under investigation.
Clinical expression ranges from fetal/neonatal onset
with predominant hepatic and pulmonary disease, through
childhood presentations with multisystem neurologic
disease, to psychiatric and cognitive adult disease. G M2 ganglioside
accumulation in NPC correlates with meganeurite
formation and ectopic dendritogenesis. Pharmacologic inhibition
of glycosphingolipid synthesis by OGT 918 (that
competitively inhibits glucosyltransferase synthase) leads
to increased survival in the Balb/c murine model of NPC.
A genetic model of glycosphingolipid synthesis in NPC,
created by a cross between the Balb/c mutant and a GalNAc
transferase knockout, shows no survival advantage over the
untreated Balb/c mutant. These data suggest that lactosylceramide,
glucosylceramide and ganglioside accumulation
must all be reduced to produce increased survival in murine
NPC. A phase I/II clinical trial of OGT 918 in human NPC is
in progress.
SY-08-4
Neuropathic Gaucher disease: diagnosis and
management
G.M. Pastores
Neurogenetics Unit, Department of Neurology, New York
University School of Medicine, New York, NY, USA
GD is a defect glycosphingolipid metabolism due to a
deficiency of lysosomal glucocerebrosidase. Most patients
do not have primary CNS involvement (type 1 GD); some
develop acute or chronic neurodegeneration. Presence of the
N370S mutation precludes CNS disease, while homozygosity
for L444P is often seen with severe disease and a relatively
high (but not invariable) risk of neurologic
involvement. Brainstem auditory evoked response (BAER)
may show abnormal peak forms and inter-peak latencies, and
short-latency somatosensory evoked potential (SSEP) testing
may reveal giant cortical potentials, which correlate with
development of cognitive impairment. Although brain
imaging and neurophysiological studies provide prognostic
information, absence of abnormal findings does not exclude
the possibility of CNS involvement. Enzyme therapy safely
reverses the extra-neuronal manifestations. Treatment does
not appear to ultimately influence the neurologic disease
course in acute Neuronopathic Gaucher disease (NGD).
Treatment of chronic NGD patients has been reported to
stabilize supranuclear palsy and cognitive function, although
there are patients who have worsened and developed progressive
myoclonic encephalopathy accompanied by cranial
MRI and EEG. Assessment of therapeutic response for
chronic NGD is complicated by wide heterogeneity in clinical
expression. Novel therapeutic strategies, including direct
delivery to local sites of pathology, may be necessary to
achieve optimal responses. Substrate synthesis inhibitors
(SSI) have recently been shown to effect a clinical response
in patients with type 1 GD. The safety and effectiveness of
SSI for NGD remains to be established.
SY-08-5
Chemical chaperone therapy for lysosomal diseases with
central nerve system pathology
Y. Suzuki a , E. Nanba b , K. Ohno b , J. Matsuda c , S. Ogawa d
a International University of Health and Welfare, Otawara,
Japan; b Tottori University, Yonago, Japan; c National Institute
of Infectious Diseases, Tokyo, Japan; d Keio University,
Yokohama, Japan
Some mutant enzyme proteins are labile and rapidly
degraded in somatic cells from patients with Fabry disease
(Fan, Ishii, Asano, Suzuki: Nature Med, 1999). If an
exogenous substrate analog compound of low molecular
weight is added to the cell, association of these two molecules
stabilizes the mutant enzyme protein, which is then
safely transported to the lysosome and expresses the catalytic
activity after spontaneous dissociation with the
compound (chemical chaperone) in the lysosome. We
further confirmed this paradoxical phenomenon for b-galactosidase
deficiency (G M1 -gangliosidosis and Morquio B
disease) and b-glucosidase deficiency (Gaucher disease).
After screening of newly synthesized compounds, we
found two potent competitive inhibitors: GalX for b-galactosidase
and GlcX for b-glucosidase (tentative nomenclature).
They are enzyme inhibitors in vitro, but induced
expression of the mutant enzyme activity at low concentrations
in cultured fibroblasts from some patients, respectively,
with these two diseases. Mutant enzyme activities
were remarkably elevated after several days of culture
with one of these compounds. The mutations responsive
to this procedure mainly represented late onset or atypical
clinical phenotypes. Furthermore we produced a few mouse
lines expressing phenotype-specific mutant human b-galactosidase,
by introducing cDNA as transgene into the
enzyme-deficient knockout mouse. This new disease
model, knockout-transgenic mouse, serves for animal
experiments of our therapeutic approach to human b-galactosidase
deficiency disorders. One of the model mouse lines
expressing the R201C mutation was fed with GalX solution
for a week. All tissues including the brain showed 5–15-fold
elevation of the b-galactosidase activity. This result indicates
that the compound GalX passed through the blood–
brain barrier to restore enzyme activity in the brain, which is
sufficient for intracellular degradation of stored substrates.

364
Abstracts
This is the first achievement for developing a new therapeutic
approach by oral medication to the brain pathology in
lysosomal storage diseases.
SY-9
Child and Adolescent Epilepsy in the Framework of
the ILAE/IBE/WHO Global Campaign against Epilepsy
SY-09-1
The ILAE/IBE/WHO global campaign against epilepsy:
A progress report
S. Jan
India
Abstract not submitted
SY-09-2
Epilepsy in infancy and childhood: Incidence,
prevalence and clinical spectrum
S. Ohtahara
Department of Child Neurology, Okayama University Medical
School, Okayama, Japan
Onset of epilepsies is mostly in childhood; about 70% of
epileptic patients have their initial attack within the first 3
years of life. Response to the treatment is usually favorable
in childhood epilepsy in comparison with that of adult
epilepsy. This may suggest the possibility of remarkable
reduction of epileptic patients by the proper treatment of
childhood epilepsy. The real condition of childhood
epilepsy will be outlined according to the epidemiological
findings, particularly two extensive neuroepidemiological
investigations of childhood epilepsy in Okayama Prefecture.
Relating the intractable cases, cardinal epileptic
syndromes are overviewed. Characteristics of childhood
epilepsy are mentioned, referring to the conceptualization
of the age-dependent epileptic encephalopathy and its
developmental aspects.
SY-09-3
Development and disruption: long-term effects epileptic
interference with early cognitive development
S.J. Wallace
University Hospital of Wales, Cardiff, UK
Of the stages of development of the brain, neuronal
migration and organisation; and, the formation of synapses,
with their reinforcement or apoptosis, seem to be the most
relevant. The peak period of migration is passed well before
fetal viability, but organisation and synaptic formation
continue into the early years. When epilepsy develops
very early, poor later cognitive development may merely
be a reflection of severe abnormalities in prenatal brain
development: these would be impossible to correct. Epilepsies
with onset after the neonatal period will be the main
subject for discussion, since there could be some scope for
amelioration of subsequent cognitive difficulties. Four main
aspects will be considered. Biochemical/metabolic reasons
for neuronal damage following epileptic seizures include
the actions of excito-toxic amino acids; and during status
epilepticus, additionally, hypoxia and metabolic acidosis.
Disruption of learning can be secondary to the presence of
the epilepsy of particular syndromes, such as those of West,
Dravet and Lennox-Gastaut, and in epilepsies with specific
cognitive symptomatology. Possible effects of treatment of
epilepsy, such as drugs, which might interfere with, or
enhance, cognition and educational progress, need consideration.
Secondary effects on psychosocial functioning, e.g.
alterations in parental management and expectations, and,
reduced educational and social opportunities can be important.
The less florid, but equally important, educational
problems of children with benign focal epilepsies and
absences will be addressed. Epilepsy is a further additional
handicap in children with cerebral palsy, when it is associated
with enhanced cognitive problems.
SY-09-4
The impact of epilepsy. The influence of epilepsy on the
lives of the child, the parents and the siblings
M. Endziniene
Kaunas University of Medicine, Lithuania
One of the most difficult aspects of having epilepsy is not
the epilepsy itself, but the problem of adjusting to it. In early
years, physical impact of epilepsy on the child is most
prominent (injuries caused by seizures, medication side
effects, unpleasant medical procedures). In later years,
emotional and behavioral consequences as well as difficulties
in psychological and social adjustment come to front.
The unpredictability of seizures make the patients feel not as
good as other people. Poor self-esteem, depression, anxiety;
fear of being isolated by peers and of being unable to meet
parental expectations; learning difficulties, multiple restrictions,
uncertainty in the future and continuous striving to
avoid overprotection are the key problems that that the teenagers
have to deal daily. On the other hand, the burden of
having a chronically ill child has great influence on the
family relationships and social roles. It sometimes takes
months to accept the diagnosis of epilepsy, to settle realistic
expectations and to learn how to help the child in coping
with its problems. The feelings of guilt, anxiety, helplessness
and doubts lead to overprotection and excessive restrictions
towards the disabled child. Loss of parental attention
and care may cause emotional problems in healthy siblings.
Parental inability to cope with personal emotions and to plan
family life often lead to complete disruption of family relations
or professional career, and to social isolation. Providing
adequate knowledge and timely counseling for the

Abstracts 365
family members and for the child, teaching them to take
active part in dealing with epilepsy-related problems can
prevent stigmatization and improve social adjustment.
Education of healthcare professionals as well as of the
public also plays a major role.
SY-09-5
Living with epilepsy – a patient with epilepsy from India
R. Sehgal
India
Abstract not submitted
SY-10
Tourette Syndrome
SY-10-1
Phenomenology and natural history of tic disorders
J.F. Leckman
Child Study Center, South Frontage Road, Yale University
School of Medicine, New Haven, CT, USA
Tics are isolated, disinhibited fragments of normal motor
or vocal behaviors. Like habits, tics often arise from a
heightened and selective sensitivity to cues from within
the body or from the outside world. Many patients report
being besieged by premonitory somatosensory urges (bodily
cues) that are often localized to discrete anatomical areas.
These urges and the internal struggle to control them can be
as debilitating as the tics themselves. Motor tics usually
begin between the ages of 3 and 8 years. Typically vocal
tics follow the onset of motor tics by several yrs. In uncomplicated
cases, motor and vocal tic severity peaks early in
the second decade with many patients showing a marked
reduction in tic severity by the age of 20 years. However, the
most severe cases occur in adulthood. Motor and phonic tics
occur in bouts over the course of a day and wax and wane in
severity over the course of weeks to months. Less well
known is the ‘self-similarity’ of these temporal patterns
across different time scales. Knowledge of the temporal
patterning of tics is fundamental for the practitioner as it
informs decisions about when to initiate or change anti-tic
medications, and when to be patient and simply provide
close monitoring and support to the family. A deeper understanding
of the multiplicative processes that govern these
temporal patterns may clarify both microscopic neural
events occurring in millisecond time scales as well as
macroscopic features of the natural history of tic disorders
that occur over decades. In addition to tics, many patients
suffer with symptoms of attention deficit hyperactivity
disorder and/or obsessive-compulsive disorder. When
present, these coexisting conditions can add greatly to the
morbidity associated with tic disorders and detract from the
patient’s overall quality of life.
SY-10-2
Neurobiology of Tourette syndrome
H.S. Singer
Department of Pediatrics Division, The Johns Hopkins
University, USA
Neuroanatomy: Significant data, derived from volumetric
MRI, area measurements of the corpus callosum, imaging of
glucose metabolism and blood flow, coulometer paradigms,
transcranial magnetic stimulation, and functional MRI,
supports the proposal that Tourette syndrome (TS) is associated
with changes in frontal-subcortical circuits. Volumetric
MRI studies in subjects with TS have shown:
significant differences in the symmetry of the putamen
and lenticular region in boys and a reduction in the size of
these structures in adults; larger volumes of a region that
approximated dorsal prefrontal and smaller volumes of
regions that approximated premotor and orbitofrontal cortex
in boys; and a larger percentage of white matter in the right
frontal lobe. fMRI studies of tic activity and suppression
have implicated various cortical regions, basal ganglia,
and thalamus. Neurochemistry: The distribution of classical
neurotransmitters (e.g. dopamine, serotonin, GABA, glutamate,
acetylcholine, norepinephrine, and opioids) within the
basal ganglia and frontal-subcortical circuits raises the
possibility that a variety of transmitters could be involved
in the pathobiology of TS. In general, current hypotheses are
based on extrapolations from clinical trials evaluating the
response to specific medications; from studies of cerebrospinal
fluid (CSF), blood, and urine; from neurochemical
assays on a limited number of postmortem brain tissues;
and from SPECT and PET investigations. Microarray analysis
in TS and control postmortem putamen has identified
several significant differences in gene expression. PET
studies have primarily focused on the dopamine and serotonin
systems. A recent report has shown that TS patients, as
compared to controls, have an increased release of dopamine
in the putamen following a pharmacologic challenge
with amphetamine. Neuroimmunology: Although TS is a
genetic disorder, environmental factors, especially infections,
have been hypothesized to evoke or exacerbate tics.
The existence of pediatric autoimmune neuropsychiatric
disorders associated with streptococcal infection
(PANDAS), however, remains controversial. Confirmatory
studies, which seek to identify an immune-mediated
mechanism involving molecular mimicry, to date, have
only been partially successful. A small number of patients
with PANDAS have responded to immunomodulatory therapy
with either intravenous immunoglobulin (IVIG) or plasmapheresis.
Antineuronal antibodies measured against three
distinct fractions (supernatant, pellet, and synaptosomes)
from adult postmortem caudate, putamen, and globus pallidus,
however, showed no difference between PANDAS
patients and controls. Additionally, the validity of studies
suggesting that the microinfusion of TS or PANDAS sera

366
Abstracts
into rodent striatum caused significant increases in oral
stereotypic behaviors and episodic utterances have been
questioned.
SY-10-3
Recent advances in genetics on Toulette syndrome
J. Walkup
Department of Pediatrics Division, The Johns Hopkins
University, USA
Abstract not submitted
SY-10-4
Tourette syndrome in Taiwan
H.-S. Wang
Division of Pediatric Neurology, Chang Gung Children’s
Hospital, College of Medicine, Chang Gung University,
Taoyuan, Chinese Taipei
The prevalence of TS in a primary elementary school here
is around 0.6%. It is no more a rare or degenerative disorder;
it is a model of neuropsychiatric disorder in children.
Although the pathogenesis of TS is still uncertain, the high
incidence offamilial cases up to 30% suggests a possibility of
genetic origin. Pharmacological, electrophysiological, and
neuroimaging evidences all implicate the hyper-responsiveness
of dopamine influencing the cortical-striatal-thalamocortical
circuits of patients with TS. Facing patients with tics,
first of all we must exclude the possibilities of Tourettism
with secondary etiologies. Ninety percent of patients with
primary tics occur transiently and spontaneously subside
within 1 year. Those patients with tics persisting longer
than a year will be chronic tic disorder or TS depending on
how many types of motor and/or vocal tics they have ever
had. Tics are mild in 73% of patients with TS. For them,
understanding and acceptance from family, teachers, and
friends are the most important things. When tics are so severe
that medication is necessary, haloperidol is no longer the first
or only choice. Clonidine or atypical neuroleptics such as
risperidone or olanzapine should be used first for their
minor side effects. Many other medicines such as topiramate
are still in trial. Some children visiting our Tourette Clinics
were found to have high serum copper concentrations that are
under our further evaluation.
SY-10-5
Toulette syndrome in Asian countries
Y. Nomura
Segawa Neurological Clinic for Children, Chiyoda-ku,
Tokyo, Japan
Abstract not submitted
SY-11
Neurocutaneous Syndromes
SY-11-1
Advances in understanding neurological and
behavioural phenotypes in tuberous sclerosis
P. Curatolo
Pediatric Neurology, Tor Vergata University of Rome,
Rome, Italy
Tuberous sclerosis (TSC) is a disorder of cells migration,
proliferation, and differentiation. Cell lineage and cell
migration disorders in the developing cortex of TSC children
produce very different neurological phenotypes including
epilepsy, cognitive impairment and autism. Cortical
tubers constitute the hallmark of the disease. At the moment
about 400 different mutations in both TSC genes are known.
Patients with TSC1 mutation have on average milder
disease in comparison with patients with TSC2 mutations.
They have a lower frequency of seizures and moderatesevere
mental retardation, fewer cortical tubers, less severe
kidney involvement and facial angiofibromas, and no retinal
hamartoma. Epilepsy is the most common neurological
feature. Seizures often begin in the first months of life and
are frequently intractable. Selected drug resistant patients
could be considered for surgical treatment. The finding of
the multiple areas of cerebral involvement should not automatically
preclude epilepsy surgery in a child with intractable
seizures and well-defined seizures origin. Autism
appears to be more common in infants with frontal and
parieto-temporal tubers and may be due to an early dysfunction
in the associative areas. Autism could be also considered
a secondary effect of seizures and/or mental
retardation. An alternative and more intriguing explanation
is that this abnormal behavior may reflect a more direct
effect of the abnormal genetic program. A couple of genome
scans in autism suggested that a potential susceptibility gene
may be located on chromosome 16p13. The genetic analysis
of the short arm of the chromosome 16 will help to localise
such candidate gene and clarify its position with respect to
the TSC2 locus.
SY-11-2
Recent progress about mutational analysis of TSC1 and
TSC2 genes and functions of the hamartin protein
E. Nanba
Gene Research Center, Tottori University, Yonago, Japan
TSC is a neurocutaneous syndrome characterized by
development of unusual tumor-like growths. Involvement
of the brain is associated with the most problematic clinical
manifestations of TSC, including intellectual retardation,
epilepsy and abnormal behaviors. Until now, over 300
mutations of TSC1 and TSC2 were reported. We have

Abstracts 367
surveyed the mutations of TSC1 and TSC2 from 76 Japanese
patients. We analyzed the all the exons of both genes
by single strand conformation polymorphism method
(SSCP) followed by sequencing. Nine TSC1 mutations
and 20 TSC2 mutations were found. The mutations were
not clustered on a particular exons in either of the genes.
All mutations were could not be found in the family
members. The nonsense mutations in TSC1 and missense
mutations in TSC2 were relatively popular and no splicing
mutation was found. The patients with TSC2 mutations tend
to exhibit relatively severe mental retardation in comparison
to those with TSC1 mutations. Our mutation detection rate
was only 40% and there are several possibilities including
other undiscovered gene for Japanese TSC patients for the
rate. To analysis of the function of hamartin that is TSC1
gene product, we have tried to screen the proteins binding to
TSC1 gene product by yeast two-hybrid method. We found
several genes and have continued further study.
SY-11-3
Typical and atypical non-neoplastic brain abnormalities
on MRI in children and adolescents with
neurofibromatosis type 1
O. Eeg-Olofsson, R. Raininko, L. Thelin
University Hospital, Uppsala, Sweden
The occurrence, localization and longitudinal course of
non-neoplastic MRI abnormalities in children and adolescents
with neurofibromatosis type 1 (NF1) were studied.
Thirty-five patients who satisfied the criteria for NF1 underwent
114 MRI examinations. They were 9 months to 18
years old at the time of their first examination, and 23
were examined more than once (2–11 times). The followup
time varied from 3 months to 10 years (mean: 4 years).
High signal intensity lesions on T2-weighted images were
seen in the cerebellum, brain stem, and deep cerebral gray
matter and, less frequently, in the cerebral white matter in
89% of the subjects. Proton density-weighted and T1-
weighted images showed changes in 80 and 50%, respectively.
During follow-up, some new lesions developed,
some disappeared and some changed in size and appearance
without correlation to the patient’s age. In four patients, all
boys, one or two of the lesions with a high T2 intensity were
expansive. They were located in the upper medulla oblongata,
in the medulla oblongata expanding into the spinal
cord, in the left cerebellar hemisphere, and in the wall of
the left lateral ventricle. The last-mentioned lesion showed
contrast enhancement that disappeared in 2 years. All those
lesions had atypical features, commonly regarded as signs
of a neoplasm, but they receded without specific treatment.
There were no related clinical symptoms in any patients.
Obviously, the border between neoplastic and non-neoplastic
lesions is indistinct. High signal lesions on T2-weighted
MR images should be included as another criterion for the
diagnosis of NF1.
SY-11-4
Vascular malformations and neurocutaneous diseases
I. Pascual-Castroviejo
Pediatric Neurology Service, University Hospital La Paz,
Madrid, Spain
Neurocutaneous diseases (an old concept is phakomatoses)
are frequently associated with vascular intra or extracranial
malformations. Vascular anomalies mostly occurs in
classical phakomatoses such as SWS and also in other disorders
described more recently. However, a great part of the
neurocutaneous diseases can develop some type of vascular
anomalies. Arteriography and especially magnetic resonance
arteriography (MRA) show a great variety of vascular
malformations. Neurifibromatosis type1 (NF1): it can be
occasionally associated with internal carotid asymmetry or
with moyamoya disease. SWS: The presence of leptomeningeal
angiomatosis ipsolateral to the facial nevus flammeus
distributed over a partial or total zone innervated by the first
sensory branch of the trigeminal nerve is a necessary criterion
in the diagnosis of SWS. Furthermore, angiomas in the
ocular choroids and absence of various images in the cortical
region of the affected hemisphere can be seen. Cutaneous
hemangioma-vascular complex syndrome: This is
the most frequent neurocutaneous disease, surpassing the
NF1. This disease was described in 1978. It is associated
with persistence of the trigeminal artery and absence of
carotid and/or vertebral arteries, mostly of the same side
of the cutaneous hemangioma, in a great part of the patients.
Angiomatous or mega-arterial intracranial anomalies can be
seen as well. Increase and decrease in parallel with the
cutaneous and the intracranial arteries are usually seen.
Conclusion: During the last years new neurocutaneous
diseases appeared and new vascular findings were discovered;
MRA is the most recommended study.
SY-11-5
Vascular malformations and neurocutaneous diseases
I. Pascual-Castroviejo
Pediatric Neurology Service, University Hospiutal La Paz,
Madrid, Spain
Neurocutaneous diseases (an old concept is phakomatoses)
are frequently associated with vascular intra- or extracranial
malformations. Vascular anomalies mostly occur in
classical phakomatoses such as Sturge-Weber syndrome
(SWS) and also in other disorders described more recently
(1, 2). However, a great part of the neurocutaneos diseases
can develop some types of vascular anomalies. Arteriography
and especially magnetic resonance arteriography (MRA)
show great variety of vascular malformations. NF1: It can be
occasionally associated with internal carotid asymmetry or
with moyamoya disease. SWS: The presence of leptomeningeal
angiomatosis ipsilateral to the facial nevus flammeus

368
Abstracts
distributed over a partial or total zone innervated by the first
sensory branch of the trigeminal nerve is a necessary criterion
in the diagnosis of SWS. Furthermore, angioma in the
ocular choroid and absence of venous images in the cortical
region of the affected hemisphere can be seen. Cutaneos
hemangioma-vascular complex syndrome: This is the most
frequent neurocutaneous disease, surpassing the NF1. This
disease was described in 1978 (1). It is associated with persistence
of the trigeminal artery and absence of carotid and/or
vertebral arteries, mostly of the same side of the cutaneous
hemangioma, in a great part of the patients. Angiomatous or
mega-arterial intracranial anomalies can be seen as well.
Increase and decrease in parallel of the cutaneous and the
intracranial arteries are usually seen. Conclusion: During
the last years new neurocutaneos diseases appeared and
new vascular findings were discovered. MRA is the most
recommended study.
SY-12
Education of Child Neurologists
SY-12-1
Overview of peripheral neuropathy in childhood 2002
R. Ouvrier
The Children’s Hospital at Westmead, Australia
This presentation describes the findings in a biopsy
series of 260 cases of polyneuropathy in children up to
16 years of age. Conditions in which we have had a particular
interest will be emphasised and illustrated with
videotapes. Approximately 83% of cases were genetic in
origin, and about 17% were acquired. Of the acquired
neuropathies, acute and chronic inflammatory polyneuropathies
are the most frequent (8% of the series) and the
most important, since effective treatment is available.
Chronic polyneuropathies are of two main pathological
types-axonal degenerative and de- (and re-) myelinating.
The axonal forms constituted some 137 cases (53%). Their
molecular basis is, in most cases, poorly understood.
Hereditary motor and sensory neuropathy (HMSN) of
neuronal type commencing in early childhood and the
newly described severe infantile axonal neuropathy with
respiratory failure (SIANR or SMARD) will be discussed
in detail. There were 103 cases of demyelinating neuropathies,
most of which were due to inherited mutations of
specific myelin proteins. In this paediatric series, there
were five documented point mutations of the methyl
prednisolone (MPZ) and three of the PMP22 genes, all of
which presented with features of the Dejerine-Sottas
syndrome. X-linked Charcot-Marie-Tooth disease is
usually caused by mutations of connexin 32. There were
five cases in the series. Most cases of chronic demyelinating
neuropathy presenting in childhood can be precisely
diagnosed to enable accurate genetic counselling. Unfortunately,
specific treatment is not yet available for such cases
but the expansion of the understanding of these conditions
gives real hope for an eventual cure.
SY-12-2
Education of child neurologists in China
X.-R. Wu
Department of Pediatrics, First Hospital, Peking University,
Beijing, China
In early 1960s, several child neurology groups were
established spontaneously in Beijing, Shanghai, and
Guonzhou cities, they were the pioneers and founders of
child neurology in China. In 1985, Chinese Pediatric
Neurology Society was formally established as one of the
subsocieties of Chinese Pediatric Society. Since late 1970s,
the OPEN policy greatly promoted the training and international
professional exchange in child neurology of
China. (1) The national pediatric resident postgraduate
education program started in 1986 organized by Ministry
of Health of China, it included total 5 years training,
divided into first 3 years and later 2 years. During the 5
years, there were national doctor’s license exam and final
resident postgraduate education training qualified exam.
For child neurologists, in addition to pass the 5 years training,
should be further trained in a domestic child neurology
eligible center (1 year), or passed the master or Ph.D.
training programs of child neurology, or in adult neurology
(6 month–1 year), or in child neurology of foreign countries.
The national specialist examination systems of China
are still during working. (2) Child neurology training class:
Since 1980–2001, total 95 classes in 25 cities of China
were held, 4290 pediatricians have been trained. Four to
8 weeks for each class. In addition to general child neurology
class, also special topic classes such as seizure disorders,
cerebral palsy, anticonvulsants pharmacology,
neonatal screening, EEG, learning and behavior problems,
etc. (3) International professional exchange in child neurology:
Send pediatricians to be trained in child neurology
training program in foreign countries as USA, Japan,
Australia, etc., also in Hong Kong area. We invited child
neurology experts or neuroscientists from foreign countries
to visit and give lectures, to establish collaborated research
projects, etc. We encourage young doctors to attend international
child neurology meetings. (4) So far, more domestic
child neurology textbooks or reference books have been
published, also there are more international child neurology
textbooks and journals can be seen in most big cities
library in China. The young generation of Chinese child
neurologists already have been grown up today in China.
We express our heartfelt thanks to all of the friends in the
world they have been so kindly supported us during the
passed 20 years.

Abstracts 369
SY-12-3
Electroencephalographic photosensitivity among
Zimbabwean youths
J.B. Familusi, B. Adamolekun
Departments of Pediatrics and Medicine University of
Zimbabwe, Harare, Zimbabwe
To clarify the factors associated with EEG photosensitivity,
the records of patients who had EEG examinations in the
city of Harare, Zimbabwe between 1968 and 1996 were
studied. EEG photosensitivity was confirmed in 107 of a
total of 9082 youths (age 0–25 years), giving an overall
photosensitivity prevalence of 1.17% in the study population.
Photosensitivity occurred more frequently in females
than in males, and the peak age period for its occurrence was
during adolescence. A significantly higher prevalence of
photosensitivity was recorded among whites and Asians
than among black, while the coloured population had an
intermediate prevalence. The monthly and seasonal incidence
of photosensitivity in the present study showed no
correlation with the prevailing mean monthly or seasonal
temperatures, sunshine duration and sunlight intensity in
Harare during the period covered by the study. These findings
indicate that sunshine-related factors do not play a
dominant role in the occurrence of photosensitivity, thereby
negating previous opinions which attributed the relative
rarity of photosensitivity in black Africans to high levels
of exposure to sunshine in tropical Africa. Our findings
therefore corroborate the view that photosensitivity depends
primarily on genetic rather than environmental factors.
SY-12-4
Education approach to metabolic abnormalities in
childhood
K. Swaiman
Pediatric Neurology, St. Paul, MN, USA
The education of trainees directed at metabolic disease
must convey a sense of systematic evaluation. This evaluation
begins with the likelihood of a metabolic disease based
on history and examination. In all cases, the patterns of
genetic transmission must be understood. Among the
amino acidopathies, the prototypic disease is phenylketonuria.
As has been the case for many amino acidopathies,
a number of forms of phenylketouria have been associated
with various intricacles of the involved metabolic pathways.
Other common amino acidopathies are maple syrup urine
disease which is the result usually of abnormalities in
branched-chain ketoacid decarboxylase, homocystinuria
(of which there are several types), and a number of other
less common conditions. Many of these conditions, involving
essential amino acids, are treated with special dietary
preparations. In some cases, patients will respond to cofactor
administration because the abnormal protein enzyme
requires greater than normal concentrations of the cofactor.
Hyperammonemia is extremely toxic to nervous tissue.
Although hyperammonemia may be a secondary finding in
a number of conditions, significant metabolic central
nervous system disruption may result. When the enzyme
deficiency in Krebs-Hensleit urea cycle pathway is proximal
in the urea cycle pathway, as a rule, the more severe the
clinical condition. Lysosomal disease such as the sphingolipidoses,
mucopolysaccharidoses, mucolipidoses, glycogen
storage disease, glycoproteinoses and some other storage
disease are the result of abnormal accumulation of normal
substrates and their catabolic products within lysosome.
Research has resulted in a much better understanding of
mechanisms common to these diseases, as well as mechanisms
specific to each disease. The peroxisomal disorders, a
group of disease entities that share structural and/or functional
abnormalities of the peroxisomes, are inherited and
may have profound neurologic and systemic effects. Some
of the disorders lack peroxisomes in cells, while others have
single or multiple peroxisomal enzymatic deficiencies in
spite of the presence of normal peroxisomes. Abnormalities
of energy metabolism are frequently accompanied by lactic
acidosis. Hypoglycemia and hyperammonemia may be
evident. Abnormalities may be present in pyruvate metabolism
which are usually due to abnormalities in pyruvate
carboxlase deficiency or variations thereof, pyruvate dehydrogenase
complex deficiency, citric acid cycle abnormalities,
and respiratory chain abnormalities. Brain and muscle
are chiefly involved. For most cell type, when the oxygen
supply is adequate, energy requirements are met in mitochondria
by oxidation of pyruvate, fatty acids, or ketone
bodies. These substances are converted into acetyl-CoA
and enter the citric acid cycle and the respiratory chain.
Impairment of oxidative metabolism may occur due to
disorders of substrate transport, of substrate utilization, of
citric acid cycle function, or of the respiratory chain.
SY-12-5
Intractable pediatric epilepsy syndromes in early
infancy
S. Ohtahara
Department of Child Neurology, Okayama University Medical
School, Okayama, Japan
The new classification of epilepsy adopted the epiltptic
encephalopathy, among which Ohtahara syndrome, early
myoclonic encephalopathy, peculiar early-onset type of
symptomatic partial epilepsy, and severe myclonic epilepsy
in infancy (Dravet syndrome) have their onset in early
infancy. The concept of the epileptic encephalopathy and
these epileptic syndromes will be mentioned with their
etiology, clinicoelectrical characteristics, diagnostic
criteria, differential diagnosis, treatment, prognosis and
mutual relationship. Ohtahara syndrome is delineated, particularly
from the developmental viewpoint.

370
SY-13
Pervasive Developmental Disorders
SY-13-1
Earliest signs of autism in infants
Abstracts
in sarcastic sentences. The number of landmine was also
significantly high in HFPDD. Conclusion: ADHD children
were balanced in this test, however HFPDD children
showed specific difficulty in sarcastic sentences. This
might cause their social troubles.
P. Filipek
University of California Irvine, Irvine, CA, USA
Infant siblings of autistic children are routinely followed
by the author to document the earliest signs suspicious for
autism or other developmental disorders. Longitudinal
videotapes will be shown for several case studies, to demonstrate
the wide variability of the early warning signs, and the
effects of early intervention on these babies.
SY-13-2
Genetics
J.T. McCracken
Division of Child and Adolescent Psychiatry, UCLA Neurophychiatric
Institute, Los Angeles, USA
Abstract not submitted
SY-13-3
Pragmatic difficulties of figurative and sarcastic
sentences in pervasive developmental disorders
T. Koeda
Department of Humanity Education, Faculty of Education
and Regional Sciences, Tottori University, Tottori, Japan
It is well known that the children with high function
pervasive developmental disorders (HFPDD) fail the theory
of mind tasks. These children often have the episode of
social troubles. The causes of the troubles are not only the
mind blindness but also the language impairments, i.e.
comprehensive difficulties of figurative and/or sarcastic
situations. We made a novel test using figurative and sarcastic
sentences for HFPDD to know their pragmatic skills. The
test is constructed from five of figurative and five of sarcastic
sentences. The normative distribution of this test was
studied. Normative distribution: Two hundred normative
children, second grade to sixth of primary school (age 7–
12 year-old), 96 boys, were carried out the test. The results
are following; the number of correct answers in figurative
sentences is increasing as grade. In sarcastic sentences, there
is no significant difference except for between the second
and sixth grade. Most serious incorrect answer, we called
‘landmine’, is not so much in normative children. HFPDD
and attention deficit and hyperactivity disorder (ADHD) 15
of HFPDD and nine of ADHD children were nominated ion
this study. There is no significant difference between
HFPDD and ADHD in figurative sentences, however
HFPDD children answered more incorrectly than ADHD
SY-13-4
The neurrophysiological aspect of pervasive
developmental disorders
M. Miyao
Department of Developmental Neuropsychology, National
children’s medical center National center for child health
and development, Japan
Childhood autism is now widely viewed as being of
developmental neurobiological origin that is defined behaviorally
by severe deficiencies in reciprocal social interaction,
verbal and nonverbal communication, and restricted
interests. Yet, localized structural and functional brain
correlates of autism have to be established. Structural
brain-imaging studies performed in autistic patients have
reported abnormalities such as increased total brain volume
and cerebellar abnormalities. However, none of these
abnormalities fully account for the full range of autistic
symptoms. A large number of investigation techniques are
used to relationships between the clinical and neurophysiological
data were necessary to analysis in the field of pervasive
developmental disorder. The prevalence of epilepsy in
the general population is 0.5%; the published figures on
epilepsy in autism range 4–7%. One of the most difficult
things to understand in the field of autism is the many papers
describing brainstem auditory evoked potentials, cortical
evoked potentials and event related potentials. Midlatency
auditory evoked responses (P1), measuring the ascending
reticular activation system and their thalamic target cells,
have been reported as abnormal in children with autism.
Event related potential P3 waves are smaller in children
with autism compared to controls. Resting EEGs from the
bilateral frontal, central, and occipital regions were examined
though power spectrum. The frequency of the dominant
peak in the occipital EEGs reached maximum at 12–20
years. And development of sleep spindles was analyzed.
Two spindle types, 11–13 Hz frontal lobe spindles and
12–14 Hz centro-parietal lobe spindles were recorded. The
frontal lobe spindles were frequently recorded at 8 years, the
centro-parietal spindles were frequently recorded at 18
years. Topographical maps of the EEG coherence demonstrated
that a synchronous component at the anterior-occipital
areas and right and left were higher in subjects with
autism compared to controls. We think that neurophysiological
research into childhood autism has a particular interest
in the broader field of developmental neuropsychology
because it could give some explanation of casual mechanisms
that underlie autistic syndrome and other developmental
syndromes.

Abstracts 371
SY-14
Neurosurgery
SY-14-1
Technological advances in pediatric neurosurgery
S. Constantini
Department of Pediatric Neurosurgery, Dana Children’s
Hospital, Israel
Several technological advances that occurred in the last
10 years completely altered the setup in the pediatric
neurosurgical operating room. These innovations have
significantly changed our approach towards children with
complex brain and spinal cord problems. Technological
advances are not all positive and common sense has to
be preserved. Economical issues, an enormous logistic
burden, and the potential for errors are all reasons to
review our new reality with respect to prioritization, decision-making,
and optimal usage. The lecture will try and
provide an overview on our modern operating room and
cover areas such as neuro-navigation-intraoperative MRIneuroendoscopy,
and real-time, intraoperative evoked
potential monitoring. The new ‘gadgets’ and their ability
will be presented together with day-to-day dilemmas that
come along.
SY-14-2
Surgical concepts of fetal hydrocephalus and
dysraphism with its specific plasticity
S. Oi
Department of Neurosurgery, The Jikei University, School
of Medicine, Tokyo, Japan
The specific plasticity of the immature brain and spinal
cord is one of the most significant factors affecting the
therapeutic outcomes in hydrocephalus and some forms
of dysraphic state. In fetal hydrocephalus, the time of
onset of the hydrocephalic state is various initially involving
different stage of the neuronal maturation process. In
order to analyze and anticipate the postnatal outcome, we
proposed a new classification of congenital hydrocephalus,
namely, the perspective classification of congenital hydrocephalus
(PCCH) Stage I–V (J. Neurosurg. 88:685–694,
1998). Our data clearly indicated that the time of the
onset of hydrocephalic state is the key in different stages
of the neuronal maturation process in the affected brain of
the fetus. It may be indicated to perform the intrauterine
decompressive procedure as the fetal surgery. Although we
have performed the experimental fetal surgery on the
animal models, the best operative technique is still debatable.
On the other hand, regarding the fetal surgery for
fetal dysraphism, especially to fetal spina bifida, there have
been over 200 fetuses with myeloschisis operated during
the fetal period in utero in the United States of America
since April, 1997. The results suggested that the early
repair of myeloschisis, such as in PCCH Stage II, may
decrease the occurrence of Chiari malformation and hydrocephalus
after birth. In order to clarify both embryological
and surgicoanatomical concept of the dysraphic state, we
have proposed a new classification, namely, embryo-pathogenetic
surgico-anatomical classification of dysraphism
(Nervous System in Children 27:213–222, 2002). In our
prospective analysis of spina bifida in the last 10 years, it
has been identified to be important to classify the morphological
findings of the fetus MRI for the indication and goal
of the treatment in fetal surgery in spina bifida/cranium
bifidum. The current state of these surgical of the fetal
CNS anomalies will be further discussed from the specific
plasticity of the fetus brain in the process of neuronal
maturation.
SY-14-3
Current status of craniofacial surgery
A. Hockley
Neurosurgeon, The Birmingham Children’s Hospital,
Birmingham Great Britain
Abstract not submitted
SY-14-4
Neurosurgical treatment of phacomatoses
C. Di Rocco
Institute of Neurosurgery, Catholic University medical
School, Rome, Italy
Phacomatoses constitute a heterogeneous group of
conditions of a particular interest to the pediatric neurosurgeon.
Children with neurofibromatosis 1 are at a higher risk
of malignancy, mainly optic pathways tumors, than the
general population. Though the criteria for diagnosis are
generally met when the patients are 8 year old, optic pathways
gliomas become often symptomatic by 3 years of age.
The occurrence of these tumors, the evolution of which is
particularly unpredictable, arises significant problems in
terms of management. In aggressive lesions the combination
of ‘conservative’ surgical treatment and chemotherapy
seems at the moment to represent the best option. Patients
with tuberous sclerosis need surgical operation generally
before puberty in cases in whom a progressive subependymal
giant cell astrocytoma in the region of the foramen of
Monro exerts a mass effect on the adjacent neural structures
and/or causes an obstructive hydrocephalus. The
direct surgical excision of the tumor may be curative and
also assures the best change to avoid the insertion of a CSF
shunt device. More rarely, the surgical operation is
required in the very young subject to remove a cortical
tuber responsible for an epilepsy refractory to the medical
treatment in order to reduce the number and the severity of

372
Abstracts
seizures and favoring psychomotor development. Drugresistant
seizures are indeed the main reason for operative
treatment in infants and children with Sturge-Weber
disease and linear nevus syndrome with hemimegalencephaly.
In nearly all the case, the operation is required in the
very young age. While only a subgroup of patients with
Sturge-Weber disease actually needs of hemispheric ablative
or deafferentative surgical procedures, the large majority
of subjects with hemimegalencephaly and epilepsy
benefit of an early operation. Several types of deafferentation
of the malformed cerebral hemisphere are available.
On the grounds of his experience, the author will discuss
the pros and the cons of the various techniques.
SY-14-5
Syringomyelia in children
T. Abe, A. Isoshima, S. Tani, S. Oi
Department of Neurosurgery, Jikei University School of
Medicine, Japan
We performed a retrospective study of 23 patients with
syringomyelia under the age of 15 in whom the results of
MRI and clinical course had been used to indicate the characteristic
feature of syringomyelia in children. On the basis
of MRI and intraoperative findings, cases of syringomyelia
were classified into three types according to the associated
lesions: Chiari malformation types 1 and 2, and based
arachnoiditis. Pediatric patients accounted for 12% of all
cases of syringomyelia in our series. Syringomyelia with
Chiari type 1 malformation was most common type of syringomyelia
in children, accounting for 91% of cases, and
highly associated with pituitary dwarfism that accounted
for 22%. The most common initial symptom was scoliosis
in children and dissociated sensory disturbance in adults.
We found that foramen magnum decompression (FMD)
was the most appropriate treatment for syringomyelia with
Chiari type 1 malformation. During FMD, dural plasty using
Goatex should be done to prevent the post operative
arachnoid adhesion around the foramen magnum, but C2
laminectomy should not be done to prevent the postoperative
swan neck deformity in children. FMD with 4th ventricle-subarachnoid
shunt was done for the patients with Chiari
Type 1 malformation, basilar impression, small posterior
fossa and flat posterior fossa base. Our surgical treatment,
which had been selected on the basis of the results of MRI,
successfully collapsed the syrinx without complications in
most patients. However, four patients needed to have
reoperation because of syrinx reexpansion. The causes of
syrinx reexpansion were scar formation around the foramen
magnum and new bone formation at the site of posterior
fossa craniectomy.
SY-14-6
Microsurgical treatment for hypothalamic hamartoma
in children with precocious puberty
S.-Q. Luo, C.-D. Li, Z.-Y. Ma, Y.-Q. Zhang, G. Jia
Department of Neurosurgery, Tiantan Hospital, Beijing,
China
Objective: To study the surgical treatment of hypothalamic
hamartoma causing precocious puberty. Methods:
Twelve children (six girls and six boys) with precocious
puberty secondary to hypothalamic hamartoma were
recruited for our study. The mean age of the patients was
45 months old (ranged from 13 months to 9 years), and the
mean age of the onset of puberty was 9 months. All patients
were treated by microsurgery. Results: All patients had a
high percentile of stature, body weight, bone growth and
serum levels of sexual hormones. MR scan revealed an
isointense mass below the tuber cinereum extending into
supersellar and interpeduncular cistern, ranging from 3 to
25 mm in diameter, consistent with pedunculate hamartoma.
Ten hamartoma that was less than 16 mm in diameter were
removed completely and the other two hamartoma that were
large than 21 mm in diameter were gross partially removed
via a right pterional approach. The symptoms and signs of
precocious puberty resolved completely and sexual
hormone levels decreased to the pre-pubertal range without
any postoperative complications in all ten patients whose
hamartoma were totally removed, the other two patients
whose hamartoma were gross partially removed also
improved the symptom. Conclusion: Microsurgery is a
good choice of treatment for pedunculate hypothalamic
hamartoma if the hamartoma was totally removed.
SY-15
Oriental Medicine
SY-15-1
Tongue acupuncture in brain disorders in children
V. Wong a , J.G. Sun b
a Department of Paediatrics, The University of Hong Kong;
b The Jockey Club MRI Engineering Centre, The University
of Hong Kong, Hong Kong, China
Introduction: Tongue acupuncture (TAC) is an innovative
acupuncture technique invented by Dr Sun. We
launched a collaborative project of integrating traditional
Chinese medicine (TCM) into our NeuroHabilitation
Program for children with cerebral palsy, developmental
delay/mental retardation, autistic spectrum disorder
(ASD), ataxia, cortical visual impairment (CVI), stroke,
and various neurological functional disabilities such as
drooling. Objective: To study the efficacy and tolerability
of TAC in children with neurological disorders. Patients
and methods: Inclusion criteria: Children with neurological

Abstracts 373
disabilities undergoing conventional interdisciplinary
Neuro-Habilitation program. More than 500 children were
enrolled into the study during 1999 Feb–2002 May with
parental consent. TAC was given to specific tongue
acupoints daily (5 days per week) for 4–8 weeks
(Total ¼ 20–40 sessions). The acupoints chosen were determined
by the functional disability of the neurological disorder.
Objective outcome measures were used to document
efficacy (Pre-TAC and Post-TAC), depending on the
problems addressed. Randomized placebo control trials,
some double blind, were conducted for autistic spectrum
disorder, cerebral palsy and stroke. Results: Functional
improvement of various degrees occurred depending on
the age and severity of the disabilities. Some improvement
was noticeable within a few TAC sessions, especially for
drooling, gait pattern (scissoring or tiptoeing) and hyperactivity.
The intermediate effect was sustained with repeated
courses. Occasional pain and minor bleeding occurred in
some. Conclusion: (1) There is a need for integration of
TCM and Western medicine in NeuroHabilitation program.
There are limitations in both disciplines as the basic philosophy
and theories are different. Both are complementary to
each other (TCM with .2000 years of clinical human
experience and Western medicine with 100 years of scientific
research basis). (2) Currently we are investigating the
underlying mechanism of TAC in neurological plasticity
with functional neuroimaging modalities (fMRI, PET scan
of the brain). We hope to document brain regeneration with
TAC and create a Tongue Acu-Map for linking 14 meridians
and ‘Zung-Fu’ or organ systems.
SY-15-2
Neurotransmitters and acupuncture
E.-Y. Shen
Department of Pediatrics, Mackay Memorial Hospital,
Chinese Taipei
Neurotransmitters are released from their storage vesicles
by exocytosis. Previous study showed the release of endorphin
from periaqueductal grey matters after electric stimulation
on some specific acupoint. It is obvious that the
endogenous analgesic system may play an important role
in analgesia and pain treatment by acupuncture therapy.
Chinese scientist (Han et al.) reported that intraventricular
or intrathecal injection in rats with scrotonin precursor
resulted in an increase of analytic effect by acupuncture.
Zhu et al. reported that excitatory neurotransmitter, glutamic
acid and inhibitory neurotransmitters, GABA, also play
important role on acupuncture analgesia. Clinically,
acupuncture has been proved to be effective in treating
many neurological disorders in Chinese as well as other
parts of the world. From the TCM point of view, the circulation
of Qi in the meridian is important in diagnosis and
treatment of diseases. However, it will be worthwhile for the
neurologist to understand the release of neurotransmitters in
the brain during acupuncture stimulation.
SY-15-3
Studies of mechanism of ‘Kangxian’ capsule on
convulsion in experimental epilepsy rats induced with
metrazol
P.-P. Zuo a , F.-L. Yao b , X.-K. Li a ,R.Ma b
a Institute of Basic Medical Science, Chinese Academy of
Medical Science, Beijing, China; b Attached Hospital, Tianjin
University of Traditional Chinese Medicine, Tianjin,
China
‘Kangxian’ capsule is composed of 14 traditional Chinese
medicine, such as Acorus gramineus soland, Ginseng, and
Gastrodia alata Blume, etc. ‘Kangxian’ capsule had been
used to treat difficult and complicated cases for many
years in clinic and they have been restored to health. To
elucidate its action mechanism, in this study we adopted
the epilepsy rats induced with metrazol. The behavioral
experiments showed that it effectively improved the
epilepsy symptoms, increased the convulsion threshold
and extended the latent period in epilepsy rats. ‘Kangxian’
capsule could inhibit the decrease of GABA and regulate the
balance between Glu and GABA. We particularly observed
the critical enzyme, glutamate decarboxylase (GAD), which
transferred excited transmitter Glu to inhibited transmitter
GABA and played an important role. ‘Kangxian’ capsule
might affect the brain function through regulating this metabolizing
process. Compared to control group, it also significantly
down regulated the expression of c-fos in cerebral
cortex and hippocampus (P , 0:01). ‘Kangxian’ capsule
also improved the cognitive function, which was related
with NMDA receptor and Ach system in epilepsy rats.
These results suggested that ‘Kangxian’ capsule was
multi-targeting, and its action was through different
mechanisms that need further research.
SY-15-4
Antiepileptic action and its immunological mechanisms
of traditional Chinese medicine
L. Wang, H. Zhang
Pediatric Neurology, Peking University First Hospital,
Beijing, China
Objective: Traditional Chinese medicine is a great
thesaurus against human disease upon the theory ‘fu zheng
gu ben’. To investigate the effects of antiepileptic action,
learning-memory and its immunological mechanisms of
CaoGuo ZhiMu Tang (CGZMT), we have done a comprehensive
study from gross, cellular to molecular levels, both
in clinic and animal experiments. Methods: (1) Clinic effects
of CGZMT were observed in 22 children with epilepsy by

374
Abstracts
open label. (2) Three animal seizure models (maximal electric
shock-MES, pentylentetrazol seizure threshold-PST,
audiogenic seizure-AGS) were randomly divided into six
groups (n ¼ 10), i.p. NS10 ml/kg, CGZMT 10 g/kg,
CGZMT 20 g/kg, phenobarbital 50 mg/kg, clonazepam
(CZP) 0.1 mg/kg, CZP 1 mg/kg, respectively. Antiepileptic
effects were measured at CGZMT peak time and 7–14 days
afterwards. Three progresses (acquisition, consolidation and
retrieval) of learning-memory were measured by step down
test. The immunological functions effects of CGZMT were
studied by using the colorimetry, spectrophotometry (QHS),
quantitative hemolysis 3 H-TdR incorporation, and optical
microscope. Results: (1) Seizures-controlled rate was
64.6% in children with epilepsy. There were no relationships
with patient’s age, gender, duration of disease,
mono/polytherapy. (2) Seizures-free rate of CGZMT was
75% in MES, 80% in AGS and 100% (CGZMT 5 g/kg
was added CZP 0.1 mg/kg). CGZMT could prolong the
latency of PST. There was no statistical significance
compared with phenobarbital (PB). CGZMT had no adverse
effects and could accelerate learning speed and learningmemory
score. CGZMT could enhance the activity of
macrophage (MV), biological activity of interleukin-2
(IL-2), proliferation of T lymphocyte stimulated by concanavalin-A
(ConA), RBC-C 3 bRR and RBC-ICR, lower
proliferation of B lymphocyte stimulated by lipopolysaccharide
(LPS), level of antibody forming cell and hemolysin
antibody. Conclusion: CGZMT had antiepileptic efficacy
with accelerating cognitive function by increasing the activity
of MV, cell immune function and RBC adhesion function.
The improving of body immune state, i.e. ‘fu zheng gu
ben’.
SY-16-1
HIV-1/AIDS in children
SY-16
Infectious Diseases
A.L. Belman
School of Medicine, State University of New York, Stony
Brook, NY, USA
Since the initial descriptions of AIDS in children two
decades ago, much has been learned about the biology of
human immunodeficiency virus 1 (HIV-1) and the cells it
infects. Much has also been learned about maternal-infant
viral transmission and the natural history of HIV-1 infection.
Key studies led to strategies to interrupt maternal-infant
transmission resulting in a significant decline in neonatal
HIV-1 infection. More proficient diagnostic techniques
made possible early diagnosis of HIV-1 infected neonates
and infants during asymptomatic or mildly symptomatic
disease stages. Major treatment advances led to better control
of viral replication and thereby altered the course of disease
progression. HIV-1/AIDS associated neurological disorders
declined in parallel. A dramatic decline in the numbers of
infants born HIV-1 infected has been observed in countries
where currently recommended therapies are readily available.
Children with HIV-1/AIDS are living longer, many
already surviving into adolescence and young adulthood.
Unfortunately, worldwide, this is not the case; and it is worldwide
where the epidemic predominates and where the
complexity of medical care required and expensive drug
therapies are limited. Many issues remain. Just how HIV-1
affects the developing central nervous system (CNS) is still
not clearly understood and is the focus of continuing
research. The long-term effects of prenatal exposure to antiretroviral
agents are not yet known and continue to be
studied. Just exactly how HAART therapy may affect early
signs of pediatric HIV-1/AIDS associated CNS disease,
should it develop, is unclear. As new antiretroviral agents
are developed, and new combination drug regimens instituted,
the potential for neurological complications, toxicities,
and adverse drug interactions (for example with AEDS)
exists and will need to be identified and monitored.
SY-16-2
Viral encephalitis
Hj.M.I. Hussain
Penang, Malaysia
Traditionally discussions on viral encephalitis have
focused on Herpes viruses and Japanese Encephalitis.
However in recent years new viruses have been recognized
to cause outbreaks of viral encephalitis with a high case
fatality rate. Dengue hemorrhagic fever is often associated
with neurological manifestations especially headache,
altered sensorium and convulsions. These were thought
initially to arise from an encephalopathy secondary to circulatory
changes, cerebral hemorrhage or hepatic derangement.
Now the virus has been isolated from the cerebral
spinal fluid of patients with encephalopathy proving dengue
to be a neurotropic virus. In recent years outbreaks of Enterovirus
71 in Bulgaria, Malaysia and Taiwan have resulted in
many deaths among young children. Most of the children
died from fulminant pulmonary edema secondary to brainstem
encephalitis, similar to deaths from poliovirus in the
late fifties. Other affected children developed acute flaccid
paralysis. This raises concerns that Enterovirus 71 may
replace polio as a cause of acquired paralysis in children.
Most recently, a newly identified virus, the Nipah virus
caused an outbreak of encephalitis involving 265 adults in
Malaysia. There were 105 deaths with 12 relapses among
the survivors. As in Japanese encephalitis, swine are thought
to act as amplifying host for the Nipah virus. Spread to
humans is via direct contact with secretions and blood
products. Climatic and environmental changes are believed
to have precipitated the outbreak of Nipah encephalitis.
Continued global warming and deforestation may result in
similar outbreaks in future.

Abstracts 375
SY-16-3
Tuberculosis of the central nervous system in children
P. Visudhiphan, A. Visudtibhan, S. Chiemchanya
Department of Pediatrics, Ramathibodi Hospital, Bangkok,
Thailand
Tuberculosis in developing countries remains a significant
public health problems. The number of cases is increasing,
partly due to wide spreading of HIV infection in these
regions. Tuberculous meningitis (TBM) is one of the most
common extrapulmonary manifestations of tuberculosis in
children. Despite effective antituberculosis drugs, morbidity
and mortality rate remain high. Early death and poor clinical
response are usually caused by failure to recognize the
disease and begin appropriate therapy in the early stage.
The duration and combination of drugs therapy of TBM in
children is still a controversial issue. Early study showed
that treatment with isoniazid (INH) and rifampin (RIF) for
12-months was generally effective in children with drugsusceptable
TBM. Due to increasing incidence of drug resistant
tuberculosis, the American Academy of Pediatrics
recommends a 12-month regimen of antituberculosis
drugs in children with TBM. But some experts have
shown that regimen between 6 and 9 months are equally
effective. We conducted, non-randomized, prospective
open clinical investigation in non-compromized host of all
cases of TBM tuberculomas, and spinal tuberculosis who
were admitted at the Department of Pediatrics, Ramathibodi
Hospital from January 1991 to December 2000. The combination
of antituberculosis drug treatment including daily
INH, RIF, pyrazinamide (PZA) and the fourth drug was
streptomycin (SM) or ethambutol (EMB) for 2 months
then followed with daily INH and RIF for 7 months.
There were 31 patients enrolled in this prospective study,
which include cases of TBM 23, TBM with tuberculomas
four, and spinal tuberculosis with spinal cord compression
in four patients. Of 23 TBM and four tuberculomas patients,
four, 11 and 12 patients were admitted in the first, second
and third stages of the disease respectively. Twenty-four of
these patients and four of spinal tuberculosis received the
initial treatment with INH, RIF, PZA and SM and the other
three patients received EMB instead of SM as the initial
treatment. Four patients required ventriculoperitoneal
shunting in the early stage to relieve their severe hydrocephalus.
Two patients who had tuberculomas needed craniotomy
for partial removal of the mass and confirmation of the
diagnosis. All cases of spinal tuberculosis with spinal cord
compression had surgical decompression of the spinal cord.
The outcome of these patients revealed no death. There was
no significant neurological deficit of the patients who were
admitted in stages I and II and also in cases of spinal tuberculosis.
But eight out of 12 patients who were admitted in
the third stage had mild to severe neurological sequel. The
mycobacterium were identified from the CSF in six patients,
and all susceptible to antituberculosis drugs given. No
recurrent of tuberculous infection observed at the last
followed up examination from 1 to 9 years.
SY-16-4
Treatment and prophylaxis of falciparum malaria
S. Looareesuwan
Hospital for Tropical Diseases, Faculty of Tropical Medicine,
Mahidol University, Bangkok, Thailand
The treatment for uncomplicated malaria is aimed at
producing a radical cure using the combination of: artesunate
(4 mg/kg per day) plus mefloquine (8 mg/kg per day)
for 3 days; a fixed dose of artemether and lumefantrine (20/
120 mg tablet) named Coartem w (four tablets twice a day
for 3 days for adults weighing more than 35 kg); quinine 10
mg/kg 8-h plus tetracycline 250 mg 6-h for 7 days (or
doxycycline 200 mg once a day for 7 days as an alternative
to tetracycline) in patients aged 8 years and over; a combination
of atovaquone and proguanil called Malarone w (in
adult, four tablets given daily £ 3 days). In treating severe
malaria, early diagnosis and early treatment with a potent
antimalarial drug is recommended to save the patient’s life.
The antimalarial drugs of choice are: intravenous quinine
or a parenteral form of an artemisinin derivative (artesunate
i.v./i.m. 2.4 mg/kg followed by 1.2 mg/kg injection at
12 and 24 h and then daily for 5 days; artemether i.m. 3.2
mg/kg injection followed by 1.6 mg/kg at 12 and 24 h and
then daily for 5 days; arteether i.m. (Artemotil w ) with the
same dose of artemether; artesunate suppository (5 mg/kg)
given rectally 12 h for 3 days). Oral artemisinin derivatives
(artesunate, artemether, dihydroartemisinin with the dose 4
mg/kg per day should replace parenteral forms when
patients can tolerate oral medication. Oral mefloquine (25
mg/kg divided into two doses 8 h apart) should be given at
the end of the artemisinin treatment course to reduce recrudescence.
Early recognition of malaria’s complications and
their adequate treatment also play an important role as
these complications (hypoglycemia, pulmonary oedema,
acute renal failure, metabolic acidosis, convulsions) often
increase the mortality rate. Other symptomatic and supportive
treatments include the careful monitoring of fluid input
and urine output, the provision of good nursing care and
the avoidance of harmful adjuvant treatment. In spite of
these efforts, mortality from severe malaria is still high.
There is no absolutely guaranteed chemoprophylaxis of
malaria. Therefore, personal protection (sleeping beneath
treated mosquito nets, avoidance of exposure to mosquito
bites) is recommended. In low-transmission areas, the use
of repellents and an awareness of any fever in the 6 weeks
after exposure in areas endemic for malaria should be
encouraged. Most visitors to low transmission areas probably
require no chemoprophylaxis. However, in high transmission
areas or in high-risk groups chemoprophylaxis
(malarone w , doxycycline, primaquine with food) is recommended.

376
Abstracts
SY-16-5
New concepts in the immunopathogenesis of central
nervous system (CNS) infections
P.K. Peterson, S. Hu, G. Gekker, W.S. Sheng, M.C.-J.
Cheeran, J.R. Lokensgard
Neuroimmunology Laboratory, Minneapolis Medical
Research Foundation, and the University of Minnesota
Medical School, Minneapolis, MN, USA
Despite the emergence of new etiologies and the serious
nature of encephalitis, little is known about the cellular and
molecular mechanisms involved in defense and neuropathogenesis
of infections within the brain. To address
this void in knowledge, research in our laboratory has
focused on interactions between neurotropic agents and
homogeneous populations of microglia, the resident macrophages
of the brain, astrocytes, the predominant brain cell
type, and highly enriched neurons. We have studied a variety
of intracellular pathogens including HIV-1, the etiologic
agent of AIDS dementia, cytomegalovirus, an
important cause of congenital brain disease and of encephalitis
in immunocompromised patients, and herpes
simplex virus (HSV)-1 which causes a devastating encephalitis
in immunocompetent individuals. Striking differences
have been found in the growth characteristics and in
the induction of cytokines and chemokines by these viruses
in microglia, astrocytes, and neurons. While several proinflammatory
cytokines can inhibit viral replication in glial
cells and neurons, the inability of brain cells to produce
interferon represents a major defect in host defense of the
brain. To solve this deficiency, it appears that chemokines
are used to attract CD4 1 and CD8 1 lymphocytes into the
infected brain. While this strategy may lead to resolution of
infection, in HSV-1 encephalitis it appears that ingress of
activated T-cells contributes to immune-mediated brain
damage. These concepts of the brain as an ‘immunologically
underprivileged site’ and of microglia and T-cells as
‘double-edged swords’ may guide development of new
therapeutic approaches to CNS infections.
MORNING SEMINAR
MS-1
Nutrition and Developing Brain
MS-01
Nutrition and the developing brain: the role of
polyunsaturated fatty acids
B. Koletzko
University of Munich, Germany
Intrauterine and early postnatal growth is characterised
by a very rapid deposition of lipids. The brain, retina and
other membrane rich tissues rapidly incorporate long-chain
polyunsaturated fatty acids (LCPUFA), especially docosahexaenoic
acid (DHA, C22:6n-3). In utero, the foetus is
supplied with preformed LCPUFA by placental transfer.
After birth, breast fed infants receive appreciable amounts
of preformed LCPUFA that meet intrauterine accretion
rates in membrane rich tissues. Stable isotope studies
show that human milk LCPUFA contents are not primarily
regulated by maternal tissues and not maternal diet. In
contrast, infant formulas based on vegetable oils did not
contain LCPUFA. Term infants fed formulas without
LCPUFA show a LCPUFA depletion in blood and tissues,
including the brain. Several studies have evaluated the
supplementation of infant formulas with different sources
of LCPUFA, which may normalise LCPUFA status relative
to reference groups fed human milk. Double blind
randomised trials demonstrated that LCPUFA induce
significant improvements of visual acuity and a variety
of measures of cognitive development (Bayley, Fagan,
Brunet-Lezine, and others). Our recent studies in children
with phenylketonuria indicate that DHA modulates brain
function also during school age. From the available data
we conclude that LCPUFA are conditionally essential
substrates in infants and children that modulate visual
and cognitive function.
MS-2
Peripheral Neuropathy
MS-02-1
Progressive understanding on the pathogenesis of
Guillain-Barré syndrome
F.-C. Cai
Department of Neurology, Children’s Hospital, Chongqing
University of Medical Sciences, Chongqing, China
Guillain-Barré syndrome (GBS) is an acute autoimmune
polyneuropathy and currently is recognized as a heterogeneous
disorder with several different subtypes including
acute inflammatory demyelinating polyneuropathy
(AIDP), acute motor axonal neuropathy (AMAN),acute
motor-sensory axonal neuropathy (AMSAN), and Miller-
Fisher syndrome, etc. Patients usually have antecedent
events (an illness of respiratory or gastrointestinal tract in
most), however recently there are strong evidences
supporting that at least 30–40% of GBS patients are
infected with Campylobacter jejuni prior to the onset of
the disorder in 2 weeks. Infection by C. jejuni or other
organisms may trigger an antibody response in patients
with GBS but not in those uncomplicated cases. Most
current studies of pathogenesis are centered on the hypothesis
of molecular mimicry between LPS of C. jejuni and
epitopes of host’s nerve gangliosides. Antibodies to ganglioside
GM 1 are present in 14–50% of GBS patients, but
more common in cases with axonal degeneration associated
with any subtype. Antibodies to ganglioside GQ1b
are very closely associated with Fisher syndrome. However

Abstracts 377
attempts to match the subtypes of GBS to the fine specificity
of antiganglioside antibodies have not yet full
explained the pathogenesis. It has been indicated that
special properties of the infecting organism could be act
an important role, since some strains such as Penner 0.19
and 0.41 are particularly associated with GBS but not with
enteritis. It is also to be important for the immunogenetic
background of the patient because the risk of developing
GBS after infection with type 0.19 is estimated only to be 1
in 158. Exotoxin of C. jejuni could enhance the immunogenic
pathogenicity of antibodies to GM 1 such as destroying
blood-nerve barrier, but no similarly immunogenic
consequence to nerves as ganglioside GM 1 in rats. T
cells are also involved in the pathogenesis of most or
even all forms of GBS. Response of T cells to any myelin
proteins such PO, P2, or PMP22 could induce experimental
autoimmune neuritis but the specificity of activated T cells
in pathogenesis of GBS still not quite clear. On the basis of
increased immunological understanding of GBS over the
past 15 years, both of plasmapheresis and IVIG infusion
have been shown as effective therapies to the disease.
MS-02-2
Advances in childhood neuropathy 2002
R. Ouvrier
The Children’s Hospital at Westmead, Australia
In a biopsy series of 260 cases of polyneuropathy in
children up to 16 years of age, approximately 83% of
cases were genetic in origin, and about 17% were acquired.
Of the acquired neuropathies, acute and chronic inflammatory
polyneuropathies were the most frequent (8% of the
series) and the most important, since effective treatment is
available. Chronic polyneuropathies are of two main pathological
types – axonal degenerative and de- (and re-)
myelinating. The axonal forms constituted some 137
cases (53%). Their molecular basis is, in most cases, poorly
understood. HMSN of neuronal type commencing in early
childhood and the newly described severe infantile axonal
neuropathy with respiratory failure (SMARD) will be
discussed in detail. The latter condition has been shown
to be due to a mutation of the IGHMBP2 gene and prenatal
diagnosis is now possible. There were 103 cases of demyelinating
neuropathies, most of which were due to inherited
mutations of specific myelin proteins. In this paediatric
series, there were five documented point mutations of the
MPZ and three of the PMP22 genes, all of which presented
with features of the Dejerine-Sottas syndrome (DSS).
Other causes of DSS will be reviewed. Most cases of
chronic demyelinating neuropathy presenting in childhood
can be precisely diagnosed to enable accurate genetic
counselling. Unfortunately, specific treatment is not yet
available for such cases but the expansion of the understanding
of these conditions gives real hope for an eventual
cure.
MS-3
Case Presentations
MS-03
Cerebral vascular malformations: intravascular
interventional therapy
Xiang Cai
Department of Pediatrics, Peking University First Hospital,
Beijing, China
Abstract not submitted
MS-4
Ethical Decisions in Neurology of Early Life
MS-04-1
The minimally conscious state in children
S. Ashwal
Department of Pediatrics, Loma Linda University School of
Medicine, Loma Linda, CA, USA
The minimally conscious state is a condition of severely
altered consciousness in which minimal but definite behavioral
evidence of self or environmental awareness is
demonstrated. To make the diagnosis of the minimally
conscious state, evidence of limited but clearly discernible
self or environmental awareness must be demonstrated on a
reproducible or sustained basis by one or more of the four
types of behaviors: (1) simple command-following; (2)
gestural or verbal ‘yes/no’ responses (regardless of accuracy);
(3) intelligible verbalization; and (4) purposeful
behavior including movements or affective behaviors that
occur in contingent relation to relevant environmental
stimuli and are not due to reflexive activity. Minimally
Conscious State (MCS) differs from the vegetative state
(VS) in several ways. Patients in a vegetative state have
no awareness of self or the environment whereas MCS
patients have definite although limited awareness of self
or the environment. VS patients have no evidence of
language comprehension or expression whereas MCS
patients may show a limited but reproducible form of
simple communication. MCS patients are also more likely
to experience pain and suffering in contrast to VS patients.
MCS must be distinguished from the VS because the
evaluation, care, medical decision-making and prognosis
differ in these two conditions. MCS can occur in children
with acute brain injuries, progressive neurometabolic disorders
or nervous system developmental malformations.
Additional research concerning prognosis, long-term
outcome and treatment effectiveness will be needed to
help clarify many of the medical, ethical and legal issues
surrounding the care of these patients.

378
Abstracts
MS-04-2
Ethical decisions in neurology of early life
P. Evrard
Service de Neurologie Pediatrique et des Maladies Metaboliques,
Faculte de Medicine Xavier-Bichat, Hospital
Robert-Debre, Paris, France
Abstract not submitted
MS-05
Medical Informatics
MS-5
Medical Informatics
D. Stumpf
Department of Neurology, Northwestern University Medical
School, Evanston, Illinois, USA
Abstract not submitted
MS-6
Environmental Medicine
MS-06-1
Preventing lead neurotoxicity in Shanghai, China
X.-M. Shen
Child Development and Rehabilitation Center, Shanghai
Children’s Medical Center, Shanghai, China
Abstract not submitted
MS-06-2
Neurotoxic effects of lead in children
M. Markowitz
Henry and Lucy Moses Division, Montefiore Medical
Center The University Hospital for the Albert Einstein
College of Medicine, Bronx, NY, USA
The recognition that lead induces central and peripheral
nervous system disease dates back more than 2000 years.
Over the last two millennia these effects were recognized
only at the clinical level; the neurologic aspects of the
disease were characterized by symptoms of encephalopathy
and peripheral neuropathy. In the latter half of the 20th
century an appreciation of the subclinical effects grew as
cross-sectional and then longitudinal studies of children
with low level lead exposure demonstrated detrimental
effects on cognition and behavior. Laboratory studies elucidated
some of the biochemical mechanisms responsible for
these lead effects. Most recent studies raise the question of
whether there is any threshold below which lead is safe in
humans.
MS-07
Headache
MS-7
Headache
D. Rothner
Department of Neurology, Cleveland Clinic S-71, Cleveland,
OH, USA
Abstract not submitted
MD-08-1
Short introduction
MS-8
Rett Syndrome
Y. Nomura
Segawa Neurological Clinic for Children, Chiyoda-ku,
Tokyo, Japan
Abstract not submitted
MS-08-2
The autonomic problem in Rett disorder
A.M. Kerr
Academic Centre, Department of Psychological Medicine,
Gartnavel Royal Hospital, Glasgow Scotland, UK
The British Isles Survey data comes from clinical examination
and postal health questionnaires, providing a longitudinal
account of the natural history of the condition. 1094
individuals aged 2–66 years are under continuing review
(1982–2002). Indices of health and severity first developed
in 1996 are now matched to MECP2 test results (300 cases),
contributing to national and international projects linking
MECP2 mutation type and site to clinical presentation.
Non-invasive out-patient monitoring of autonomic function,
using the NeuroScope e (MediFit Diagnostics Ltd, London,
UK) developed by Drs Peter Julu and Stig Hansen, has
helped to explain the non-epileptic vacant spells which are
common in Rett and which seem to contribute to sudden
deaths accounting for at least 20% of all (74) UK deaths.
Detailed analysis of clinical events, electroencephalogram,
breathing rhythm, vagal tone and baroreflex sensitivity in 47
cases has revealed inadequate brainstem regulation of
central autonomic cardio-respiratory function. Episodes
have been documented of brainstem shut down and of inappropriate
brainstem activation. Without accurate autonomic
monitoring these potentially life-threatening events may
appear to the clinician as shallow, apneustic, deep or interrupted
breathing associated with brief periods of altered
consciousness and pallor or cyanosis, readily confused
with epilepsy. That the autonomic abnormality can be

Abstracts 379
measured non-invasively and objectively will permit the
development and evaluation of effective intervention.
MS-08-3
MECP2 and beyond: phenotype-genotype correlations
in Rett Syndrome and related disorders
J. Christodoulou
Western Sydney Genetics Program, Children’s Hospital at
Westmead, Department of Paediatrics and Child Health,
University of Sydney, Sydney, NSW, Australia
Rett syndrome [RTT; OMIM 312750] is a severe neurodevelopmental
disorder primarily affecting females. The
diagnosis is based on the presence of a constellation of
clinical features associated with a specific developmental
profile. However, there is marked clinical variability, even
amongst classical RTT patients. The recent recognition that
methyl CpG-binding protein 2 (MECP2) gene mutations
cause RTT, has permitted definitive diagnosis in many
cases, including atypical variants. Large cohort studies
suggest that clinical severity can in part be predicted by
the type of mutation (missense versus truncation), the
affected region of the methyl-CpG binding protein-2
(MeCP2) protein (main binding domains versus non-critical
critical domains), and whether there is skewing of X-inactivation.
More recently it has been recognised that MECP2
mutations may be associated with other clinical phenotypes,
including an Angelman syndrome-like phenotype, nonspecific
mental retardation, with or without spasticity, and
with or without an X-linked inheritance pattern, or neonatal
encephalopathy in males. Moreover, the marked clinical
variability even in patients with identical mutations,
suggests that other currently unknown genetic and epigenetic
factors also modulate the clinical phenotype. The
development of an international database, with pooling of
data from multiple sources, will hopefully increase numbers
sufficiently to permit a more thorough examination of these
interactions. Finally the development of in vitro assays of
MeCP2 function and mouse models for RTT will help clarify
the effects of loss of protein function on embryonic
development and later neurodevelopmental progress, and
pave the way for a better understanding of the reasons for
the phenotypic variability seen in humans.
MS-08-4
Concluding remarks
S. Naidu
The Kennedy Krieger Research Institute, Baltimore, MD,
USA
Abstract not submitted
MS-9
Neuro-Oncology
MS-09-1
Childhood brain tumors: biologic breakthroughs,
therapeutic advances, and 0ngoing challenges
R.J. Packer
Children’s National Medical Center, Department of Neurology,
Washington DC, USA
Childhood brain tumors are the leading cause of morbidity
and mortality in children with cancer. Progress is being made
in some forms of childhood brain tumors, especially medulloblastomas
(MB). Although surgery and radiation therapy
remain the mainstays of treatment, chemotherapy is playing
an increasing role in the management of most forms of malignant
childhood brain tumors and some benign tumors. Molecular
genetic studies have recently suggested that children
with MB can be more discretely stratified into risk groups
using molecular genetic findings. In some subgroups of children
with MB, after treatment with aggressive surgery,
immediate postoperative radiotherapy coupled with
chemotherapy during and after radiation therapy, survival
rates as high as 85–90%, 5 years after diagnosis, can be
expected. In other subsets of patients, similar treatment will
result in disease control in less than one-third of patients. For
other malignant childhood brain tumors, such as brain stem
gliomas and high-grade cortical gliomas, alterations in therapy
have not resulted in improved survival. A relatively
newly-recognized subtype of primitive tumor, the atypical
teratoid tumor, has recently been characterized and carries
an horrendous prognosis. Low-grade glial tumors comprise
nearly 50% of all childhood brain tumors and surgery remains
the primary option for the majority of patients with such
tumors. However, for very young children who harbor tumors
in eloquent areas of brain, chemotherapy has been shown to be
effective in delaying, if not obviating, the need for extensive
surgery or radiotherapy. With the therapeutic advances seen,
thereisneedtopaygreaterattentiontothelong-termqualityof
lifeofsurvivorsofchildhoodbraintumors.Thereisincreasing
evidence that survivors have significant neurologic, neurocognitive
and psychosocial sequelae. The approaches being
utilized must attempt to reach a better balance between survival
and quality of life of survivors.
MS-09-2
Pediatric neuooncology-brainstem and spinal cord
tumors
S. Constantini
Division of Pediatric Neurosurgery, Dana Children’s
Hospital, Tel-Aviv-Sourasky Medical Center, Tel-Aviv,
Israel
Abstract not submitted

380
Abstracts
MS-10
Therapeutic Drug Monitoring
MS-10-1
Therapeutic drug monitoring (TDM) of anti-epileptic
drugs in children
P. Walson
Professor of Pediatrics, University of Cincinnati Director,
Division of Clinical Pharmacology and Clinical Trials
Office Children’s Hospital Medical Center, USA
Accurate measurement and proper interpretation of drug
concentrations (TDM) can greatly improve medical diagnosis
and management. However, TDM requires much more
than just measurement of drug concentrations. TDM is a
process by which measurement of drug concentrations is
combined with knowledge of the drug’s pharmacokinetics
and pharmacodynamics, as well as with patient specific
analytical and clinical data. TDM is useful for all drugs in
some specific clinical situations. TDM is especially useful
for drugs with: (a) a well-defined relationship between
concentration and effects (either therapeutic or toxic); (b)
wide inter- or intra-individual differences in drug clearance;
and (c) where this is no readily available method to clinically
assess therapeutic or toxic effects. Antiepileptic drugs
(AEDs) are examples of drugs for which properly done
TDM has been shown to improve efficacy while decreasing
toxicity. A number of published studies done over the past
20 years in our laboratory with older AEDs will be briefly
reviewed. Some important general and specific principles of
pediatric TDM will be discussed including some practical
and theoretical differences between children and adults.
Some examples will be discussed to illustrate how proper
pediatric TDM differs from ‘numbers only’ reporting of
concentrations. These studies illustrate how much can be
learned about pediatric AED use from a review of properly
collected, analyzed and interpreted AED TDM database.
These data also demonstrate the wide intra-individual differences
in AED clearance and illustrate why TDM is required
to effectively individualize AED dosing to treat pediatric
seizure patients. Finally, data on the usefulness of TDM
of newer AEDs will be discussed.
MS-10-2
Developmental and therapeutic pharmacology of
antiepileptic drugs
H. Miura
Department of Pediatrics, Kitasato University School of
Medicine, Sagamihara, Kanagawa, Japan
We investigated the clinical effects and plasma levels of
zonisamide (ZNS) in children with cryptogenic localizationrelated
epilepsies. ZNS is absorbed slowly and its biological
half-life is long as compared with that of other antiepilepic
drugs. The peak-to-trough plasma level ratios during a day
were as small as 1.28 ^ 0.15 in children taking a daily dose of
8 mg/kg of ZNS once a day as a single drug. The plasma level
(mg/ml) to dose (mg/kg per day) ratios estimated by the
trough and peak plasma levels both increased with advancing
age, but the peak-to-trough plasma level ratios were maintained
almost uniformly throughout the pediatric age period.
A wide range of the plasma levels was associated with
complete freedom from seizures. However, the clinical
effects of ZNS were in agreement with the range of generally
accepted therapeutic plasma levels of 15–40 mg/ml. Any
patient who receives polytherapy is at risk to develop drug
interactions. Concurrent administration of carbamazepine
(CBZ) decreases plasma concentrations of ZNS. However,
ZNS does not alter plasma concentrations of CBZ or its
primary metabolite, carbamazepine-10,11-epoxide (CBZ-
E). Drug-protein binding interactions are one source of side
effects. A simultaneous administration of valproic acid
increases the total plasma CBZ-E levels relative to the
CBZ dose associated with the raised free fractions of CBZ
and CBZ-E. The free plasma concentrations of CBZ-E above
1.5 mg/ml may be responsible for the side effects.
WORKSHOP
WS-1
Cerebral Palsy
WS-1-1
Etiology and risk factors for cerebral palsy
V. Kalra
Department of Pediatrics, All India Institute of Medial
Sciences, Ansari Nagar, New Delhi, India
Cerebral palsy (CP) implies a group of non-progressive
neuromotor impairment syndromes due to an acute insult to
the developing brain. Prenatal and perinatal period provides
greatest vulnerability. The casual factors are complex, often
multiple and the attributable risk from an individual factor is
often difficult to define in a patient. Preterm, extreme low
birth weight, multiple pregnancies, perinatal hypoxia, hypoglycemia,
hypothermia, sepsis and jaundice remain
commonly identifiable perinatal factors in our data. In
developing countries with domiciliary deliveries, perinatal
hypoxia still remains an important contributory factor.
Prevention of perinatal hypoxia neonatal sepsis, hypothermia
hypoglycemia are important in developing countries.
Congenital intrauterine infections are important identifiable
factors due to therapy issues. Malformations of the brain
remain an important cause to identify. Maternal infections
are assuming greater importance. Maternal socioeconomic
status, anemia, systemic diseases, hypertension, iodine deficiencies
unattended during pregnancy are not uncommon in
developing countries. Neuroinfections and acquired neurological
insults in early childhood are also more frequent than
in developing countries. Risk factors for CP in developing

Abstracts 381
countries may be multiple. Reduction implies a multipronged
strategy by improved obstetric and perinatal care.
WS-1-2
Evaluation of the risk factors associated with cerebral
palsy in children
Y.-Y. Zhong
Cheng Du Children Hospital, Cheng Du, China
Objective: To evaluate the risk factors associated with the
pathogenesis of childhood cerebral palsy (CP). Method: A
cross-sectionalsurvey on the prevalence of CP was conducted
in 1–6 years old children in Leshan area, Sichuan Province.
Cluster sampling and 1: 2 case control study methods were
used to investigate the risk factors of CP pathogenesis. Result:
A total of 148 723 children were surveyed among which 308
(2.07‰) were diagnosed as CP. Low birth weight, twins and
premature birth were associated with significantly increased
CP prevalence. The single factor analysis showed that the
pathogenesis of CP were multifactorial. The multifactorial
analysis revealed the significant risk factors which included
delivery at home, low 5 min Apgar score, illness within the 1st
monthoflife,maternal‘cold’withfeverinearlygestation,low
protein intake (meat and egg) during pregnancy and poor
education of the mother. Conclusion: The prevalence and
clinical features of CP in Leshan Area is comparable to the
advanced countries. The relevant risk factors can be seen
primarily in the gestational and perinatal periods which may
involve in the mother and the child, the environment and
heredity, etc. Better recognition of and improvement in gestational
and perinatal health care should be a critical way to
reduce the occurrence of CP in children.
WS-1-3
Severe forms of cerebral palsy
K. Kodama
National Rehabilitation Center for Disabled Children in
Japan, Japan
In this presentation I will explain the prevalence and medical
problems of very severely handicapped children and
special care systems for them in Japan. They have severe
motor problems combined with profound cognitive damages,
often accompanied by various kinds of medical problems.
We estimate there are about 35 000 such cases in Japan
now. Though their diagnoses are not same, over 70% of
them are seemed to be cerebral palsy. About two third of
them need drug therapy against epilepsy or extreme hypertonus.
In most severe group among them, nearly 50% have
upper respiratory problems, swallowing difficulties and
gastro-easophageal refluxes. Also hip joint dislocation,
high degree of scoliosis or multiple deformities they have.
Since mid of 1960s we have been developing care systems for
them. The number of special institutions with the functions of
medical hospital and life care home has been increasing up to
100 and about 16 000 cases are now living in such institutions.
Also we combined day care community centers, home
visiting nursing services and respite day or night care systems
and made special community networks. Furthermore, we are
paying our efforts to prevent future disabilities from early
stages of their lives. This time I will report how they changed
their posture and motor conditions and other disabilities
through over 20 years life, how we predict their future course
from young period and how we prevent their wrong course.
WS-1-4
Risk factors for pathological long bone fractures in nonambulatory
cerebral palsy children: a case control study
C.H. Ko, V.S.K. Ho, E.C.C. Wong, E.C.P. So, M.K.Y. Koo,
P.W.T. Tse
Developmental Disabilities Unit (DDU), Department of
Paediatrics, Caritas Medical Centre, Hong Kong, China
Introduction: Pathological long bone fractures is a
common problem in non-ambulatory spastic or dyskinetic
CP children. This is a retrospective study to identify the
risk factors for this problem. Methods: From June 1992 to
June 2001, all the CP children residing in the DDU who had a
history of pathological fracture were reviewed. The data
collected included the demographic data, nutritional status,
mode of feeding, contracture status, orthopedic surgery and
postoperative immobilization within 12 months prior to fracture,
anti-epileptic therapy, and general health status. The
comparison group composed of concomitant CP residents
who did not have a history of fracture. Multivariate analysis
was utilized to identify the independent risk factors for pathological
fracture. Results: The patients composed of 19 children
with 25 fractures, including 21 in the femur, two in the
tibia or fibula, one in the radius and one in the humerus. The
comparison group composed of 108 children. The body mass
index (BMI) (P ¼ 0:0127, odds ratio ¼ 0.74) and presence of
contracture in any extremity (P ¼ 0:0457, odds ratio ¼ 2.72)
were found to be significant independent risk factors for
pathological fracture. Discussion: In non-ambulatory spastic
or dyskinetic CP children, a low BMI and the presence of
severe extremity contracture were found to be significant risk
factors leading to pathological fracture. The habilitation
goals should include intensive physiotherapy and anti-spasticity
therapies to prevent contractures, as well as nutritional
measures to augment the BMI.
WS-1-5
Cerebral palsy – therapeutic possibilities
G. Cole
The Robert Jones and Agnes Hunt Orthopaedic Hospital,
Oswestry, Shropshire, UK
In recent years an increasing choice of treatment modal-

382
Abstracts
ities have become available for treating the mobility
problems associated with cerebral palsy. Although the benefits
conferred by certain treatments are clear, other management
options are more controversial. In addition to
conventional orthopaedic surgery and physiotherapy,
multi-level surgery, intramuscular botulinum injections,
selective dorsal rhizotomy, intrathecal baclofen, targeted
training and sophisticated orthoses all have their advocates.
Less orthodox strategies too, such as hyperbaric oxygen,
cranial osteopathy and lycra suits have been in vogue. Cerebral
palsy is a heterogeneous condition and it might be legitimately
argued that its management should be highly
individualised. For professionals in the field however there
is now a bewildering choice of treatment options available in
many countries, with only scant guidelines about the criteria
of choice. Our lack of full understanding of some of the basic
concepts of neuromuscular function such as tone and spasticity
has contributed to some of this confusion and this has
been made worse by the lack of universally acceptable
‘outcome measures’ for comparing different treatment techniques.
Gait analysis has been shown to be useful in the child
with hemiplegia, diplegia and mild quadriplegia (less so with
ataxic and dyskinetic cerebral palsy), not only for determining
intervention, but also in its use of measuring outcomes in
a range of therapeutic options. It is however a very expensive
academic outcome tool and other less technical procedures
have been developed in recent years for determining
outcome. Tools such as gross motor function measure
(GMFM), paediatric evaluation of disability inventory
(PEDI), etc., have the major advantage of being performed
at home by trained therapists but all such tools lack some
versatility. Individual treatment options will be discussed,
including the criteria currently available, which help us to
make individual decisions about individual children.
WS-1-6
Treatment of 140 cerebral palsied children based on
combination of traditional Chinese medicine and
Western medicine
X.-J. Zhou, N.-X. Zhuang, S.-Q. Zheng, T. Chen, Y.-F. Luo
The Children’s Hospital, Zhejiang University School of
Medicine, Hang Zhou, China
Objective: To investigate the effective approaches and
practicable plan for the rehabilitation on childhood cerebral
palsy. Methods: Cerebral palsied children received multiple
therapy based on age, type and status of cerebral palsy,
including preparation of traditional Chinese medicine,
scalp acupuncture, body acupuncture, point-injection therapy,
auricular-plaster therapy, massage and pressing points,
manipulation, physiotherapy, occupational therapy, etc.
And they not only were handled in hospital, but also at
home. Results: The majority of cerebral palsied children
got progress on motor and social adaptation capacity.
There were highly significant differences on motor and
adaptive capacity between before and after treatment by
signed rank test (P , 0:01). Conclusion: It is a pattern of
rehabilitation suitable for the Chinese children with cerebral
palsy that is based on combination of traditional Chinese
medicine and Western medicine, rehabilitation in hospital
and at home. If the pattern of treatment can be better implement
and carried out, the most of cerebral palsied children
will be rehabilitated effectively.
WS-2
Epilepsy
WS-2-1
The clinically efficacy of midazolam on childhood status
epilepticus
Q.-K. Huang, M. Zhao, J.-C. Gu
Department of Pediatrics, Shanxian Central Hospital,
Shandong, China
The purpose of this study was to explore the safety and
efficacy of midazolam in the treatment of childhood status
epilepticus. Forty-three patients with status epilepticus (41
with convulsive status epilepticus and two with epilepsia
partialis continua) were treated from June 1997 to June
2001. The underly disease was idiopathic status epilepticus
in 35 patients and symptomatic status epilepticus in eight
patients. They were divided randomly into the treatment
group and control group. During the study, the pulse, respiration,
blood pressure were monitored. The treatment group
included 22 cases (12 boys and ten girls) aged 8 months–12
years. The midazolam was given in a dose of 0.05–0.2 mg/kg
bolus for the patients with continuous seizures, and 3–15 mg/
kg per min infusion for those who had frequent seizures. The
seizure ceased 10 min to 4 h after the administration of midazolam,
and no comp1ication occurred. The control group
included 21 cases (11 boys and ten girls) aged 10 months–
12 years diazepam, phenobarbital, lidocain and thiopentone
was given sequentially. The seizure ceased 15 min–42 h after
the first anticonvulsant administration. In control group, the
case with seizure continuous for 42 h that died of underlying
disease, three patients need intubating and assisted ventilation
because of respiration suppresion. The time interval
from midazolam administration to seizure ceased was significantly
shorter than that of control group (Rank test
P , 0:05). It is concluded that midazolam is safe and effective
in treating the childhood status epilepticus.
WS-2-2
Five children with frontal lobe epilepsy
J. Li, H.-B. Zhang
Jinan Children Hospital, Jinan, China
We report five children (four male and one female; age is
22 days, 5, 6, 8 and 8 years, respectively) with frontal lobe

Abstracts 383
epilepsy. Three children were misdiagnosed as dyssomnia,
hysteria and tetany. Two children were diagnosed as undetermined
epilepsy. Twenty-three seizures (duration range
10–45 s) of the five children were evaluated with longterm
(12–15 h) video-EEG monitoring; two children had
seizures during sleep, two in the wake of or during sleep,
and the other when awake. The same child suffered from
similar symptoms at every onset. All five children presented
with atypical tic seizures; one child suffered from panic and
crying in sleep, one adversive seizures, two tonic postural
seizures, the other paresthesia with both lower extremities
tics. All of the five children became flushed with panic.
Common EEGs of five children are negative. Typical spikes
coming from frontal lobe can be found on video-EEG in
both seizure term and interval and noted in sleep. Our
cases show that the clinical manifestation of frontal lobe
epilepsy varied, being characterized by high seizure
frequencies, short duration, and nocturnal preponderance,
with vegetative and psychiatric symptoms. Video-EEG is
of value in diagnosis because of a high positive rate, and
the relation between clinical manifestation and cerebral
electrical activity can be synchronized on video-EEG.
There is one child who had seizures in the neonatal period,
which has never been reported before.
WS-2-3
Reappraisal of a peculiar form of acute encephalitis/
encephalopathy presenting with catastrophic status
Y. Awaya a,b , K. Hayashi b , Y. Fukuyama b,c , M. Osawa b
a Department of Pediatrics, Seibo International Catholic
Hospital, Tokyo, Japan; b Department of Pediatrics, Tokyo
Women’s Medical University, Tokyo, Japan; c Child Neurology
Institute, Tokyo, Japan
Background: In 1986 and 1989, the authors described ‘a
peculiar type of post-encephalitic epilepsy’ as a probable
new clinical entity, the characteristics of which consisted
of acute onset of encephalitis/encephalopathy with catastrophic
status followed by an evolution into intractable
chronic partial epilepsy. Since, similar cases have been
reported sporadically in Japan, but no description exists in
non-Japanese literature yet, except for Baxter’s abstract in
1999. Methods: Medical records of five patients prospectively
observed by the authors were reviewed. In addition,
a review of Japanese literatures from 1988 to 2001 identified
29 compatible cases in 18 papers. Based upon 34 cases thus
gathered, clinical features of the syndrome were reassessed.
Results: (1) Age of onset: 7 years old in average (range 1–15
years). (2) Preceding fever in all, except one. (3) Cardinal
symptoms in acute stage; seizures, consciousness disturbance
and fever. (4) Catastrophic status epilepticus, necessitating
general anesthesia and intubation for one to a few
weeks. (5) Stabilizes one to several months later and evolves
to intractable CPS and mental impairment. (6) Mild CSF
pleocytosis in some, virological and other exams negative.
Discussion and conclusion: Differential diagnosis includes
acute/subacute encephalitides, intoxication, Reye syndrome,
brain anomalies, metabolic and circulatory disturbances. No
clues suggestive for etiology are available. This is a unique
devastating status which requires urgent seizure control as
well as life-supportive measures.
WS-2-4
Study of genotypes and phenotypes of severe myoclonic
epilepsy in infancy
T. Fujiwara a , T. Sugawara b , E. Mazaki-Miyazaki b ,K.
Fukushima a , Y. Takahashi a , M. Watanabe a , K. Hara a ,T.
Morikawa a , K. Yagi a , K. Yamakawa b , Y. Inoue a
a National Epilepsy Center, Shizuoka Medical Institute of
Neurological Disorders, Shizuoka, Japan; b Laboratory for
Neurogenetics, RIKEN, Brain Science Institute, Saitama,
Japan
Purpose: Recent studies have demonstrated that frameshift
mutation and nonsense mutation of the sodium ion
channel a1 subunit (SCN1A) gene are responsible for
SME in infancy (Class et al., 2001). We analyzed the
SCN1A genotypes and phenotypes in a relatively large
number of Japanese SME patients. Subjects and methods:
Mutations of the SCN1A gene were studied in one pair of
monozygotic twins (details of phenotypes have been
reported, Fujiwara et al., 1990) and 29 single patients
(total 31 cases) with SME. The methods of analysis were
according to the previous report (Sugawara et al., 2001).
This study was approved by the Ethical Committee of
RIKEN, Brain Science Institute and of Shizuoka Medical
Institute of Neurological Disorders. Informed consent was
obtained from all the patients or parents. Results: Mutation
of the SCN1A gene was found in 25 of 31 cases (81%),
consisting of six cases of frameshift mutation, ten cases of
non-sense mutation (including the monozygotic twins), and
nine cases of missense mutation. No SCN1A gene mutation
was detected in the remaining six cases. Genetic analyses of
the parents of seven patients gave negative results. These
mutations were therefore considered to be de novo mutations.
Conclusion: Mutations of the SCN1A gene were
detected at a high rate (81%) in Japanese SME patients.
The mutations were diverse, ranging from frame shift,
nonsense to missense mutations. The relationship between
genotypes and phenotypes is under investigation.
WS-2-5
The effects of epilepsy-surgery on the quality-of-life of
children
M. Boonzaaijer, R. van Empelen, A. Jennekens-Schinkel, C.
Volman, C.W.M. van Veelen, P.C. van Rijen, O. van
Nieuwenhuizen
UMCU/WKZ, Department of Paediatric Physical Therapy,
Utrecht, Netherlands

384
Abstracts
Quality of life (QoL) is more and more considered an
important indicator of the impact of medical treatment.
With respect to epilepsy surgery in childhood, success of
the operation is measured not only at the level of impairments
(ICIDH 2000), but the child and his parents are also
asked to evaluate postoperative changes in the child’s functioning
in respect of participation. Quality of life, therefore,
is a multidimensional construct that can hardly be understood
solely by quantitative research (van Zuuren, 1999;
Wallander, 2001). Objective: To identify the effects of
epilepsy-surgery on QoL of children suffering from intractable
epilepsy. Design: explorative, pre-post surgery,
prospective, partly qualitative and partly quantitative
research. Methods: ‘HAY’ (how are you) (Bruil, 1999), a
Dutch health-related QoL-questionnaire with a generic and
an epilepsy-specific part, for children between the ages of 7
and 13 years. CBSK (Veerman, 1997), a Dutch adaptation
of the Self-Perception Profile for children between the ages
of 7 and 13 years (Harter, 1985). An open interview was
held with nine children and their parent(s) to obtain an
unrestrained idea of QoL before and after epilepsy surgery.
The qualitative data have been analysed quantitatively.
Results: Both parental and children’s perceptions changed
on all subscales of the HAY. Children report positive
changes in physical and cognitive activities and in feelings
about these activities. Parents report that the children have
become more active in social, physical and cognitive fields
and more proficient in physical activities. Both parents and
children report that, postoperatively, the frequencies of
seizures and of treatment contacts have significantly
decreased. On the CBSK the amelioration on the sub-scale
‘physical appearance’ is significant (P , 0:05) and the
amelioration on the sub-scales Social Acceptance,
Romance, Global Self-worth and Friendship tend to significance
(P , 0:1). The interview data amplify the questionnaire
findings: post-operatively, the child initiates contacts
with peers, the seizure-related necessity of intense one-toone
accompaniment has disappeared, freedom to undertake
social activities has increased accordingly, and – but not
least – the child’s energy has increased. Conclusion: Both
parents and children report a significant improvement in
frequency of social activities, the self-perceptions of the
children improve, some to a statistically significant degree,
and the parents report a significant energy gain.
WS-2-6
Memory impairment in specific childhood epilepsy
syndromes
A.M. Nolan, A. Redoblado, S. Lah, M. Sabaz, J.A. Lawson,
A.M. Cunningham, A. Bleasel, A.M.E. Bye
Department of Neurology, Sydney Children’s Hospital and
The Children’s Hospital; The Universities of New South
Wales and Sydney, Sydney, Australia
Aim: Memory impairment in children with epilepsy can
be a severe disability. Its incidence according to specific
childhood epilepsy syndromes is not well studied. We evaluated
verbal and visual memory in generalised idiopathic
epilepsy (GIE), frontal lobe epilepsy (FLE), temporal lobe
epilepsy (TLE), central epilepsy (CE) and non-localised
partial epilepsy (PE). Methods: From a population of 130
children with epilepsy aged 6–18 years monitored with
video-EEG telemetry, 107 were identified with GIE
(n ¼ 16), FLE (n ¼ 25), TLE (n ¼ 34), CE (n ¼ 12) or
PE (n ¼ 20). Syndrome identification was determined by
International League Against Epilepsy criteria using clinical
data, seizure semiology, interictal and ictal recordings. Each
child had detailed memory evaluation using age-normed
instruments. Memory impairment was defined as scores
more than two standard deviations below the normed
mean. Results: Memory impairment was found in 5% of
GIE and CE, 8% of FLE, 18% of TLE and 20% of PE
children. Four verbal and one visual subtest showed statistically
significant differences in performance between
syndrome groups. GIE had significantly better performance
than TLE in two verbal and the visual subtest (P ¼ 0:019,
P ¼ 0:024, P ¼ 0:055). In one verbal and the visual subtest
GIE had significantly better performance than PE
(P ¼ 0:011, P ¼ 0:019). A trend for better performance in
FLE than TLE approached significance on one verbal subset
(P ¼ 0:052). Discussion: In childhood epilepsy, memory
impairment is a quantifiable problem. Children with GIE
perform best, and significantly worse memory function
can be demonstrated in TLE and PE compared to GIE.
This information is invaluable in planning educational and
therapeutic interventions.
WS-2-7
Genetic abnormalities underlying severe myoclonic
epilepsy in infancy in Japanese
G. Fukuma, S. Hirose, T. Inoue, S. Yasumoto, M. Ohfu, A.
Watanachai, A. Mitsudome, Study Group on Epilepsy
Genetics in Japan
School of Medicine, Fukuoka University, Fukuoka, Japan
Objective: To identify genetic abnormalities underlying
SMEI in Japanese. Background: Recently, mutations of the
neuronal voltage-gated Na 1 channel 1 subunit gene
(SCN1A) have been identified as a cause of SMEI.
SCN1A and genes encoding other components of Na 1 channels
in the brain such as 2, one and two subunits (SCN2A,
SCN1B and SCN2B, respectively) were candidate genes for
SMEI. Methods: Our study recruited 54 unrelated individuals
whose clinical manifestations were consistent with
SMEI and 96 healthy volunteers. Each participant or a
responsible person signed an informed consent form
approved by the Ethics Review Committee of Fukuoka
University or similar committees of the participating institutions.
Genetic abnormalities of SCN1A, SCN2A, SCN1B
and SCN2B were sought in genomic DNA using a direct

Abstracts 385
sequencing method with an ABI 3700 sequencer. Results:In
SCN1A, eight heterozygous nonsense, 23 missense and
three frame-shift mutations resulting in a premature stop
were found in 44 individuals with SMEI. The mutations
identified in the patients were not found in 96 healthy volunteers
and hence considered to be disease-causing mutations.
No mutation was found within the examined region of
SCN2A, SCN1B and SCN2B. Conclusions: In the first
report made by a Belgian group, mutations of SCN1A
were found in all patients they studied while only 44 of
54 patients bore causative mutations in SCN1A in our
subjects. The relations between phenotype and genotype
of SMEI should be further delineated.
WS-2-8
Severe myoclonic epilepsy of infancy and SCN1A:
phenotypical-genotypical correlation
B. Ceulemans a,b , L. Claes c , L. Lagae a,d , D. Audenaert c ,J.
Del-Favero c , M. Boel a,d , C. Van Broeckhoven c ,P.De
Jonghe c , P. Evrard e , S. Raskin f , S. Ala-Mello g , L. Basel-
Vanagaite h , N.I. Wolf i , B. Plecko j
a Epilepsy Center for Children and Youth, Pulderbos,
Belgium; b Neurology, University Hospital Antwerp; c Molecular
Genetics, University Antwerp;
d Child Neurology,
Gasthuisberg, Leuven, Belgium; e Inserm, Paris, France;
f Genetika, Parana, Brazil; g Medical Genetics, Helsinki,
Finland; h Medical Genetics, Petah Tikva, Israel; i Paediatric
Neurology, Heidelberg, Germany; j Neuropediatrics,
Graz, Austria
SMEI is a rare epileptic syndrome included in the International
League Against Epilepsy (ILAE) classification as
an epileptic encephalopathy. Although it has been
mentioned that the family-history for febrile seizures and/
or epilepsy is frequently positive in children with SMEI, we
described, last year, herterozygotic de novo mutations in the
alfa subunit of a neuronal voltage-gated sodium channel
SCN1A in seven Belgian patients. We hypothesised that
loss of function or at least severe damage of this protein
causes the clinical picture of SMEI. A new group of ten,
all well characterised, cases of SMEI and mutations in the
same gene will be presented. Four are Belgians, five are
other Europeans and one is Brazilian. In a few families
with GEFS 1 SMEI occur as the most severe clinical phenotype.
On behalf of our own results and the published cases
with known mutations a phenotypical-genotypical correlation
will be presented.
WS-2-9
Rotatory seizures in non-lesional frontal lobe epilepsy –
ictal recordings with video-EEG, SPECT and MEG
O. Kanazawa, J. Tohyama
Department of Pediatrics, Epilepsy Center, National Nishi-
Niigata Central Hospital, Niigata, Japan
Rotatory seizures, during which patients turn around their
axis one or more times are also known as circling seizures or
gyratory seizures. Pure stereotypical rotatory seizures seem
to be a rare epileptic manifestation, the mechanism of body
turning still being obscure. We report on two girls, who had
had daily rotatory seizures with normal brain MRI findings.
Patient 1, who was 5 years 8 months of age, began to have
brief episodes of body turning to the right. Maximal seizure
frequency was over 20 times per day. Her interictal EEG
showed left frontopolar spikes. Ictal SPECT showed left
frontal hyperperfusion. Two months later, complete seizure
control was obtained by medication with carbamazepine
200 mg. Patient 2, who is 10 years 6 months of age at
present, began to have brief episodes of body turning to
the left at age 7 years 10 months. Maximal seizure
frequency was around 80 times per day. She had two 1-
year remission periods, but subsequently seizures relapsed
at age 10 years 1 month. Her interictal EEG showed right
frontal spikes. Ictal SPECT showed right frontal hyperperfusion.
Ictal magnetoencephalography revealed right frontal
dipole sources. Four months later, her seizures were
controlled by medication with phenytion 300 mg and primidone
250 mg. Because circling constitutes the only ictal
behaviour observed, patients may be misdiagnosed as
having a psychogenic reaction. Ictal SPECT findings in
our patients may support that basal ganglia involvement is
a necessary part of the mechanism of rotational seizures, as
Vercueil et al. have suggested.
WS-3
Specific Learning Disabilities
WS-3-1
Dyslexia in the Chinese language
C.K. Leong
Professor Emeritus, University of Saskatchewan and
Adjunct Professor, The Chinese University of Hong Kong,
Hong Kong, China
This paper discusses the nature of reading disabilities in
Chinese. Recent data from children in Beijing and Hong
Kong provide suggestive evidence to show the universality
and specific constraints on phonological analysis in learning
to read Chinese and to prevent reading difficulties.
WS-3-2
FMRI and reading
L.-H. Tan
Joint Laboratories for Language and Cognitive
Neuroscience 3A, The University of Hong Kong, Hong
Kong, China
Functional MRI findings of neuroanatomical mechanisms
underlying Chinese and English reading will be reviewed.

386
Abstracts
Our studies using several cognitive tasks have indicated that
peak activation in the processing of Chinese characters was
located in the left middle frontal cortex (BA 9 and 46),
regions that previous investigations with English and other
alphabetic languages have not commonly identified. Because
the left middle frontal cortex is known to subserve visualspatial
processing of objects and spatial working memory, we
have hypothesized that its extremely strong activation in
reading Chinese is associated with the unique square configuration
of characters. We further found that when Chinese-
English bilinguals process English words phonologically, the
left middle frontal cortex is most strongly activated. This
suggests that the processing of Chinese phonology (where
logographic characters are pronounced monosyllabically and
do not call for phonemic parsing) carries over to second
language processing. Finally, in a study with Chinese
dyslexics, we found less middle frontal and temporal activations
for dyslexic children, suggesting that they were facing a
great difficulty in phonological processing.
WS-3-3
Specific learning disabilities (SLD): advocacy
C.-W. Chan
Working Party on SLD, The Hong Kong Society of Child
Neurology and Developmental Paediatrics (HKCNDP),
Hong Kong, China
With advancement in our understanding of the neurobiological
bases, clinical features and evidence-supported
interventions for SLD, it is imperative that services for
and interests of affected individuals are safeguarded accordingly.
These are rights embodied under the UN Charter for
Children’s Rights. In order to achieve these we need alignment
of definitions among professionals, accurate identification
and diagnosis through powerful screening and
assessment tools and integrated multidisciplinary teams, as
well as specific and accountable management plans. In line
with these, there must be parents who understand their children’s
condition and needs, school teachers who have
appropriate preparation and ongoing in-service training,
enlightened education administrators, as well as wide spread
public awareness and acceptance of the disabilities. Adverse
complications associated with undiagnosed or improperly
managed children with SLD include school failure and
drop out, eroded self esteem, juvenile delinquency,
substance abuse, and a future life of unemployment and
underachievement. Effective legislation and government
policies, plus close partnerships between professionals,
stakeholders and the public are foundations for success.
Given the range of information and services that address
the full scope of SLD and appropriate measures to promote
their talents and potentials, every individual with SLD will
have the opportunity to lead a productive and fulfilling life,
from which society will ultimately benefit.
WS-3-4
Clinical diagnosis and management
C.C.C. LAM
Central Kowloon Child Assessment Centre, Kowloon, Hong
Kong, China
Abstract not submitted
FREE PAPERS-Oral Presentation
FO-1
Neuroscience
FO-1-1
Effects of developmental lead exposure on nitric oxide
synthase activity in different brain regions of rat
G.-J. Dong, Z.-Y. Zhao, Z.-W. Zhu
The Affiliated Children’s Hospital of Zhejiang University
School of Medicine, Hangzhou, China
Objective: To observe the influence of lead exposure on
nitric oxide synthase (NOS) in different brain regions of rat.
Methods: By establishing a series of rat models exposed to
different low levels lead during developing period, (drinking
water containing 0.025, 0.05 and 0.075% lead acetate) we
studied NOS activity in hippocampus, cerebellum, cerebral
cortical and brain stem. Results: For 0.025 and 0.05% groups,
NOS activity in hippocampus was obviously inhibited, had
significant difference with that in cerebral cortical and brain
stem (P , 0:05). For 0.075% group, NOS activity in cerebral
cortical was obviously lower than those in hippocampus and
brain stem (P , 0:05). On the 21st and 28th day after birth,
NOS activity in cerebellum of 0.025 and 0.05% groups was
lower than those in hippocampus, cerebral cortical and brain
stem (P , 0:05), while NOS activity in cerebellum of
0.075% group was lower than those in hippocampus and
brain stem (P , 0:05). On the 21st and 28th day after birth,
NOS activity in hippocampus and cerebellum of each experiment
group was obviously lower than that of control group
(P , 0:01). On different time points, NOS activity in Cerebral
cortical of 0.075% group was obviously lower than that
of control, 0.025 and 0.05% groups (P , 0:05). Lead exposure
had no influence on NOS activity in brain stem
(P . 0:05). Conclusions: NOS activities in hippocampus,
cerebral cortical and cerebella cortical of rat were inhibited
by lead exposure and the degree of the effect was related to Pb
exposure time and level of Pb.
FO-1-2
Developing cortical neurons injury following recurrent
epileptiform discharges induced by magnesium-free
culture
H.-Y. Cao, Y.-W. Jiang, T. Bo, X.-R. Wu
Division of neurology, Department of Pediatrics, Peking
University First Hospital, Beijing, China

Abstracts 387
Objective: To study developing cortical neurons injury
following recurrent epileptiform discharges induced by
magnesium-free culture. Methods: We cultured the cortical
neurons from 16 days rat fetuses. Cultures were exposed to
magnesium-free media for 3 h, returned to regular media
containing normal level magnesium, and maintained for 72
h. During and after the treatment, we used intracellular
recording techniques to detect the epileptiform discharges,
trypan blue staining and flow cytometry to present neuronal
viability and apoptosis, and MTT assay plus lactate dedhydrogenase
(LDH) activity to determine the cell function and
viability. Results: (1) Epileptiform discharges were induced
in all neurons sampled in the culture during the magnesiumfree
treatment, and spontaneous recurrent seizure activity
lasted 72 h in 77.8% of the sampled neurons. (2) At different
time (6, 12 and 72 h) after returning to regular media from
Mg 21 free media, no differences were found among neurons
in different cultured days (6, 12 and 17 days) on the rates of
cell death and apoptosis (P . 0:05). (3) Compared with
control, MTT metabolic activity decrease occurred at 6 h
after Mg 21 -free treatment (P , 0:05) in neurons cultured
for 6 days (85.69%), but at 24 h in neurons cultured for
12 (78.19%) and 17 days (64.00%). (4) LDH activity in
the supernatant of the culture medium decreased not significantly
at all time points after Mg 21 -free treatment
(P . 0:05). Conclusions: Our findings demonstrated that
the acute injury of neurons in this seizure cell model was
mainly functional, long term prognosis is during studying.
(This work is supported by National Natural Science Foundation
of China).
FO-1-3
Barrel field sizes are reduced in Down syndrome mouse
model and barrel plasticity is stunted in neonatally nBM
lesioned mouse
A. Nishimura a,b, *, M.E. Blue a,b , T.H. Moran d , C.F.
Hohmann e , G.T. Capone a,b,c , M.V. Johnston a,b,c
a Neuroscience Laboratory, Kennedy Krieger Institute,
b Department of Neurol,
c Pediatrics, and d Psychiatry,
Johns Hopkins University School of Med, Baltimore, MD,
USA,
e Department of Biology, Morgan State University,
Baltimore, MD, USA
*Present address: Department of Pediatrics, Kyoto Pref
University of Medicine, Kamigyo-Ku, Kyoto, Japan.
Previous studies have shown that neonatal lesion of basal
forebrain cholinergic projections (nBM) in mice leads to a
transient cholinergic depletion of neocortex and to permanent
alterations in cortical cytoarchitecture and in cognitive
performance. The present study examined whether neonatal
nBM lesions modify neocortical plasticity. The present
study also investigated whether Ts65Dn mouse, one of
Down syndrome models has an aberrant neocortical development.
Using cytochrome oxidase histochemistry, we
compared cross-sectional areas of individual barrels in
rows A–E and the Straddlers in the somatosensory cortex
of four groups of postnatal day 8 (P8) old mice that received
(1) right nBM lesions; or (2) left C row 1–4 whisker follicle
ablations; or (3) combined treatment on P1 (12–24 h after
birth) and in (4) untreated controls. And using young adult
Ts65Dn mice and control littermates, we compared crosssectional
areas of individual barrels in rows A–E. The plastic
response to whisker removal was stunted in nBM
lesioned animals. The present findings correspond to those
from a study of rats injected with the cholinergic immunotoxin,
IgG-saporin. We also found that the barrel field sizes
were reduced in Ts65Dn mouse whose nBM cholinergic
neurons degenerate at 6 months of age (Holtzman et al.,
PNAS 93 (23): 13333–8, 1996). These results suggest that
cholinergic inputs play a role in mouse barrel cortex development
and plasticity.
FO-1-4
Dioxin alters cell proliferation of neural progenitor cells
in the developing cerebral wall
T. Takahashi
Keio University School of Medicine, Tokyo, Japan
2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD), the most
potent dioxin, is an artificial environmental pollutant
known to disturb hormonal homeostasis and induce maldevelopment
of immune and urogenital systems in mice. In the
CNS, in utero and lactational exposure to TCDD causes
deficits in spatial learning and memory in rats. Several
reports in vitro indicate that TCDD may directly alter cell
cycle kinetics: TCDD retards G1 phase progression by inducing
p27Kip1 in a hepatoma cell line and in fetal thymocytes.
We have previously shown that G1 phase regulation
of proliferative neural progenitor cells plays a critical role in
the course of cerebral histogenesis. Thus, it is possible that
in utero TCDD exposure may affect cell cycle regulation,
particularly during G1 phase, of neural progenitor cells and
results in abnormal histogenesis of the neocortex. We investigated
effects of TCDD on cell cycle kinetics in the developing
mouse brain. Pregnant mice were given a single oral
dose of TCDD (20 mg/kg body weight) on embryonic day
(E) 7. On E14, after a continuous exposure to 5-bromodeoxyuridine
(BUdR) for 2, 8, 12 and 16 h, the BUdR labeling
index (LI; BUdR positive nuclei/all nuclei) was calculated
for the neural progenitor cell population of the proliferative
neuroepithelium in the developing cerebral wall. BUdR LI’s
of the neural progenitor cells were reduced in the TCDD
exposed embryos as compared to those in the control
embryos. All of the mitotic figures were BUdR positive
after 2 h exposure to BUdR both in the TCDD exposed
and control embryos, indicating that the combined length
of G2/M phases was not affected by TCDD. BUdR LI for 16
h-BUdR exposure was 1.0 for TCDD exposed embryos as in
control, indicating that all of the neural progenitor cells of
the proliferative neuroepithelium retained proliferative

388
Abstracts
activity even after TCDD exposure. These observations
taken together, the reduction in the LI’s after TCDD exposure
indicates that TCDD exposure caused G1 phase prolongation
in the neural progenitor cells. These results suggest
that in utero exposure to dioxin may affect cell cycle regulation
of the neural progenitor cells and thus, alter the process
of neuron production and eventually result in abnormal
development of the neocortex. Supported by Grant-in-Aid
from the Ministry of Health and Welfare, Pharmacia Fund
for Growth and Development Research, Science and Technology
Agency and CREST Tohyama Team, JST.
FO-2
Cerebral Palsy (1)
FO-2-1
Botulinum toxin type A in prevention of hip luxation of
bilateral cerebral palsy. Treatment of 65 patients
P.P. Si, P. Castroviejoi
Service of Pediatric Neurology, Hospital Infantile ‘La Paz’,
Madrid, Spain
Introduction: In bilateral CP, tetraplegic or diplegic, the
spasticity of flexor and adductor muscles of the hip often
produce hip dysplasia and/or progressive luxation. Objective:
To study the effectiveness of botulinum toxin type A
(BT-A) in prevention of external femoral head migration
and hip luxation in CP. Material and methods: All CP
patients treated with BT-A because pelvic girdle spasticity
were revised. Ages at the first injection were two–7 years.
The clinical and radiological evolution prior to BT-A treatment
and after it is analyzed. The drug was infiltrated in
adductors, medial hamstring muscles in every patient and
also in iliac psoas muscles if required. Post-treatment
follow-up ranged between 1 and 4 years. Results: Sixtyfive
children, 22% of 295 patients treated in our hospital
with BT-A because CP, suffered hip disorder. A total of 122
hips were treated. Pre-treatment evolution was bad, progressive
external femoral head migration out of Perkins’ line
was seen (10% of mean migration at 1 year of age, 21%
at 2, 26% at 3, 31% at 4 years of age). Nineteen/122 hip
were already luxated (more than 50% of migration) at the
onset of treatment, in spite of correct physiotherapy and
other treatments. After BT-A treatment, the progressive
impairment stopped. There was not statistical difference in
radiograph migration in patients group analysis, 10% of
femoral heads improved it position, 8% worsened and the
rest did not change during follow-up). Only one hip resulted
luxated after treatment. This is a very good result according
to the negative natural evolution of the disorder. Motor
function improved in every case whose hip was not luxated.
Conclusion: It is necessary to study clinically and radiologically
the hips of every child with bilateral CP before 3
years old. BT-A allows us to treat all the muscles implicated
in hip spasticity and avoids the progressive functional
impairment of hip mobility, so negative for the future physical
independence of CP patients.
FO-2-2
An epidemiological study of cerebral palsy in Henan
Province, China
L. Gao, Y. Meng, S.-Y. Zhao, C.-J. Zhou, C.-Z. Yuan, Y.-H.
Wang, M. Zhao, S.-L. Ma
Department of Pediatrics, Henan Provincial People’s
Hospital, Zhengzhou City, China
Objective: To investigate the prevalence, distribution of
different types and correlative etiologic factors and complications
of CP in Henan Province, China. Methods: From
January to December 2001, a random cluster sampling of
children aged 0 , 6 years were investigated in ten cities and
counties, including three districts – JinShui, ErQi,
MangShan in Zhengzhou City; JianXi district in Luoyang
City; JiYuan City and counties as YuanYang, XinYie,
TangHe, TaiKang, YuCheng, among others. The etiology,
clinical types, computed tomography and magnetic resonance
imaging of all cases were analyzed. Results: There
were 582 CP patients among 434 920 children investigated;
the total prevalence rate of CP was 1.58%, Of the 582 cases
of CP, spastic type was 56.87%, hypotonic type 21.13%,
mixture type 7.56%, tonic type 6.70%, ataxia type 5.33%,
tremor type 1.38%, and athetosis type 1.03%. The causes of
CP were perinatal asphyxia or hypoxic-ischemic encephalopathy
(HIE) (38.32%); premature or low birth weight
(16.84%); maternal infections during early pregnancy
(11.86%); hyperbilirubinemia (10.14%); intraventricular
hemorrhage (IVH) (8.25%). The incidence rate of complications
was 68.38%, 47.08% related to mental retardation
(MR). Conclusion: This investigation demonstrated the
approximate prevalence of CP in Henan Province. The spastic
type is the most common type of CP. Asphyxia and HIE,
premature and low birth weight, maternal infections during
early pregnancy and hyperbilirubinemia, among others were
high-risk factors. The main complication was MR. Most of
the causes could be prevented by health care in perinatal
period.
FO-2-3
Neonatal periventricular/intraventricular hemorrhage:
incidence and some risk factor
T.S. Al-Karagully
Department of Pediatrics, Saddam College of Medicine,
Baghdad, Iraq
Background: In a prospective study extending along an 8-
month period (April–November 1998), 100 newborns at the
University Hospital/Saddam College of Medicine, were
randomly selected and examined by cranial ultrasound (U/
S) to determine the incidence of periventricular hemorrhage/

Abstracts 389
intraventricular homorrhage (PVH/IVH) and some risk
factors. Methods: All sonograms were performed using
Real-time sector scanner with a 5-megahertz (5-MHz) Phasedarry
transducer. All examinations were performed by taking
the baby to the ultrasound examination room. No sedation
was used. Standard sonographic techniques, including axial
and occipital views, were used to obtain sections in coronal
and sagittal planes. The cranial U/S examination was
performed by the same doctor for all the babies included in
the study. Results: The incidence of PVH/IVH was 43.8% in
babies less than 1500 gm birth weight, 14.3% in babies 1500–
2500 g birth weight and no hemorrhage in babies more than
2500 g birth weight. Asphyxia was significantly (P ¼ 0:02)
related to PVH/IVH in babies less than 1500 g birth weight. A
total of 44.4% of the babies were clinically silent and the
most common presenting features were lethargy 55.5%,
convulsion 27.8%, bulging fontanel 22.2% and apnea
16.7%. Conclusions: (1) PVH/IVH is a major problem of
infants with birth weight of 1500 g; or less. (2) Asphyxia is
a major risk factor for the development of PVH/IVH in babies
with birth weight 1500 gm. or less. (3) A clinically silent
presentation may occur in the majority of cases of PVH/
IVH. (4) Lethargy, although non-specific, is the most
common presentation followed by convulsion, bulging
fontanel and apnea. (5) Sonography has enabled us to have
a better understanding of the morbid anatomy; and pathophysiology
of intracranial hemorrhage with the least invasion
and disturbance to the baby.
FO-2-4
DC-EEG unmasks very slow activity patterns during
sleep in infants
S. Vanhatalo a,b , P. Tallgren a , S. Andersson 3 , K. Sainio d ,J.
Voipio a , K. Kaila a
a Department of Biosciences, b Department of Child Neurology,
c Department of Pediatrics,
d Department of Clinical
Neurophysiology, University of Helsinki, Finland
Abundance of slow frequencies is the most prominent
feature of neonatal EEG. We used direct current electroencephalography
(DC-EEG) to test the hypothesis that the
immature human brain exhibits slow electrical activity
which is not detected by the conventional (i.e. high pass
filtered) EEG method. We performed recordings with DC-
EEG during sleep on 24 infants ranging from 32 to 46 weeks
of conceptional age. At all ages, DC-EEG revealed a significant
amount of oscillatory and other activity patterns that
are not seen with regular filter settings. Spectral analysis of
DC-EEG revealed prominent peaks at 0.01–0.2 Hz. Most
surprisingly, the activity pattern resembling delta-frequency
bursts in conventional EEG appeared to be, in fact, gigantic
negative transients. They had occipital maximum with a
very large amplitude (200–700 mV) and long duration (1–
5 s). They were also associated with bursts of high
frequency activity with intriguing similarity to the spontaneous,
synchronous neuronal activity described in recent
animal experiments. Our results demonstrate that neonatal
EEG contain significant amount of activity patterns that are
too slow to detect with a conventional EEG method. These
EEG waveforms are both quantitatively and visually very
prominent. We propose that their proper visualization with
DC-EEG may open a new avenue for non-invasive assessment
of physiological and pathological brain functions
during the neonatal period.
FO-3
Seizure Disorders (1)
FO-3-1
Higher incidence of posterior slow synchrony in EEG of
children with complex febrile seizures
V. Komárek, D. Pavlová, Z. Hrncir, M. Palus, T. Procházka,
P. Jiruska
Department of Child Neurology, Charles University Hospital
Motol, Prague, Czech Republic
Objective: Febrile seizures (FS) affect 3–5% of all children.
About 20% of FS are familial. The seizure type,
whether ‘simplex’ or ‘complex’, is important since it relates
to further prognosis. The role of EEG in differential diagnosis
of FS is still controversial (Maytal, 2000). The aim of our
study was to evaluate characteristic patterns in EEG of children
with different FS subtypes. Methods: The clinical and
EEG data were collected from 587 children suffering from
FS. In addition to visual evaluation we used linear spectral
(right-left coherence) and on-linear EEG analysis (phase
synchronization). Results: The normal EEG was found in
406 children. Specific epileptiform discharges were present
in six records. The excessive posterior slow (2–5 Hz)
synchrony (PSS) was found in 58 children (10%). The incidence
of PSS was significantly higher in children with
complex febrile seizures – 42 from 141 (27 %) than in
simplex febrile seizures – 16/446 (3.5%). Comments:
Doose (1997) reported PSS in 64% of FS with later epilepsy
and in 20% of FS with good prognosis. Our data suggest that
visual and mathematical evaluation of the EEG may be
important not only for electroclinical subclassification of
FS but also for further genotypical studies. Supported in
part by grant IGAMZCR-6258.
FO-3-2
A first locus for common simple febrile seizures
R. Nabbout a,b,c , J.F. Prud’Homme b , A. Herman a ,J.
Feingold a,e , A. Brice a,d,e , O. Dulac c , E. LeGuern a,d,e
a INSERM U289, Hôpital Pitié-Salpêtrière,
b Généthon,
Evry, c Service de Neuro-Pédiatrie, Hôpital Saint Vincent
de Paul, Paris, d Fédération de Neurologie, Hôpital Pitié-
Salpêtrière, e Departement de Génétique, Cytogénétique et
Embryologie, Hôpital Pitié-Salpêtrière, Paris, France

390
Abstracts
We report a large multigenerational French family with a
homogeneous phenotype consisting of isolated simple FS.
The FS trait did not show any linkage with the reported loci
for FS and GEFS 1 . After a genome scan, a new locus for FS
was identified. Patients and methods: This family was
obtained through a national campaign for familial epilepsy.
It consisted of 166 individuals in five generations. Affected
members presented a history of simple FS that segregates as
an autosomosal dominant trait. After exclusion of reported
loci for FS and GEFS 1 , a genome-wide search was
performed with 380 markers. Results: The clinical data
were obtained from the parents and/or medical files. In the
oldest generation, the status of all members was considered
as unknown since their parents were not alive at the time of
the present study. All FS occurred after 10 months of age
and ceased before 5 years. Only two affected patients had a
transitory anti-epileptic treatment. None presented a febrile
seizure or epilepsy and all family members have a normal
cognitive development. The genome wide search allowed
the identification of a new candidate region (Z max ¼ 3:31 at
q ¼ 0:00). Multipoint analysis and haplotypes construction
confirmed the genetic linkage of this region to simple FS
trait. Conclusion: Our family presents a homogeneous
phenotype consisting of isolated simple FS, the commonest
form of FS. A new locus is identified in this family and the
sequence analysis of potential candidate genes is in
progress.
FO-3-3
Infantile primary generalized epilepsy: benign epileptic
syndrome during infancy
E. Shahar a , S. Barak a , J. Andraus a , U. Kramer b
a Child Neurology Unit and Epilepsy Service, Rambam
Medical Center; b Haifa, Epilepsy Service, Dana Children’s
Hospital, Tel-Aviv, Israel
The present study delineates a benign generalized epileptic
disorder during infancy and early childhood, which we
termed infantile primary generalized epilepsy (IPGE), similar
to PGE. It includes infants under the age of 4 years
presenting with generalized clonic non-febrile seizures
associated with generalized epileptic EEG discharges,
favorable response to anti-epileptic drugs, and preserved
cognition and speech. Twenty-five infants who fulfilled
the inclusion criteria for IPGE, presenting at age 4–36
months (mean: 17) with recurrent generalized short-lived
clonic seizures, two also had status epilepticus. Eighteen
infants (72%) had accompanying recurrent, commonly
preceding, febrile seizures. Family history of seizures was
detected in eight (32%). EEG showed generalized 3–4 Hz
epileptiform discharges and normal background activity in
21 (84%), with a photosensitive response induced in three.
The larger group of 18 infants, mainly treated with valproate,
promptly responded to therapy terminated after 2 years,
with EEG normalization and no recurrence of seizures. A
smaller group of seven patients require prolonged therapy
that maintains them seizure-free, the EEG remains paroxysmal
and seizure may recur if treatment is discontinued.
Cognition and speech are intact in all children, but twelve
are irritable with a short attention span. The data presented
here delineates a unique generalized epileptic syndrome
during infancy with a benign course, rapid response to therapy
and preservation of cognitive skills, similar to adolescent
PGE.
FO-3-4
Non-convulsive status epilepticus (NCSE) in children
R. Koul, S. Qaboos
University Hospital, BW-1, Children ward one, Alkhod,
Oman
Non-convulsive status epilepticus is a condition characterized
by a cognitive or behavioral change, which lasts for
at least 30 min, with EEG evidence of seizures. NCSE is
uncommon in children and often seen after 10 years age.
This form of status epilepticus (SE) is often missed and is
picked up in epilepsy clinics, EEG laboratories and neurointensive
care units. One must keep this condition in mind in
patients with epilepsy who develop recent change in behavior,
memory, unexplained ataxia, prolonged speech loss
and prolonged inattention. The EEG is diagnostic. Various
types of NCSE are absence SE, complex partial SE,
Lennox-Gastaut SE, Landau-Kleffner SE, myoclonic astatic
epilepsy of Doose status and electrical epilepticus of slow
wave sleep. Eighteen patients of NCSE (26.5%) were seen
over last 8 years out of total 68 patients with SE. Complex
partial SE and Lennox-Gastaut SE formed six and five
patients, respectively, making 61% of cases. Majority of
literature supports aggressive management like convulsive
SE. Twelve children were treated with iv midazolam, nine
with additional phenytoin sodium while rest on oral antiepileptic
drugs. Outcome was good in six, poor in six and
recurrent attacks of NCSE were seen in four. Two patients
were lost to follow up. NCSE should be kept in mind in
children with epilepsy who develop change in behavior, loss
of speech, prolonged inattention or automatisms, or confusional
state. The management should be effective and
adequate.
FO-4
Neuroscience/Genetics
FO-4-1
Genotype-phenotype correlation of glucose transporter-
1 (Glut-1) deficiency syndrome
D. Wang, J.M. Pascual, P. Kranz-Eble, H. Yang, M.G.
Alvarez, R.P. Sun, D.C. De Vivo
Colleen Giblin Laboratories for Pediatric Neurology
Research, Columbia University, New York, NY, USA

Abstracts 391
Glut-1 deficiency syndrome (Glut-1 DS) was first
described in 1991. Since then, about 80 patients have been
diagnosed. Glut-1 DS is characterized by infantile seizures,
developmental delay, hypotonia, ataxia, acquired microcephaly,
and hypoglycorrhachia (30–35 mg/dl). The clinical
diagnosis can be confirmed by decreased glucose uptake
into erythrocytes (46.8%), fluorescence in situ hybridization
studies and molecular analysis of the GLUT-1 gene. We
have screened 36 patients and identified pathogenic mutations
including three cases of hemizygosity, 13 missense
mutations, four nonsense mutations, three insertions, six
microdeletions and three splice site mutations. The mutations
tend to be private with two exceptions. A missense
mutation (R333W) has been seen in three unrelated patients,
and several missense mutations have been seen in three
families at the R126 amino acid residue. There is a spectrum
of clinical severity in this patient population. Patients are
from relatively minor to severely disabled neurologically.
The phenotype-genotype correlations thus far have
remained elusive, although there is an emerging impression
that the patients with the milder phenotypes tend to have
less pathogenic missense mutations whereas the patients
with the more severe phenotypes tend to have more pathogenic
nonsense mutations or frame shift mutations that
produce a stop codon. These preliminary observations are
preliminary and subject to future modifications as more
patients are investigated.
FO-4-2
Fukutin protein expression in developing human
brainstem
Yoshiaki Saito a,b , Makio Kobayashi c , Kayoko Saito a ,
Masashi Mizuguchi d , Masayuki Itoh e , Sachio Takashima e ,
Makiko Osawa a
a Department of Pediatrics, Tokyo Women’s Medical
University, Tokyo Japan; b Division of Pediatric Neurology,
Yokohama Ryo-iku En; c Department of Pathology, Tokyo
Women’s Medical University; d Department of Pediatrics,
Jichi Medical School; e National Institute of Neuroscience,
National Center of Neurology and Psychiatry, Japan
FCMD results from fukutin gene defects and is characterized
pathologically by polymicrogyria of the cerebral and
cerebellar cortex, as well as brainstem anomalies including
the aberrant pyramidal tract. In accordance with its
predicted role in the regulation of corticogenesis, fukutin
is expressed in neurons of the developing cerebral and cerebellar
cortices. This time we examined immunohistochemically
its expression in the brainstem structures, which has
not been scrutinized. Antisera were raised in rabbits against
synthetic peptides that correspond to amino acid residues of
fukutin protein. Brainstem tissues were obtained at necropsy
from 14 control subjects aged 13 gestational weeks to 1 year
7 months. In the pons (n ¼ 9), fukutin was immunoreactive
in neurons of the facial, trigeminal and pontine nuclei as
well as the tegmentum areas including the locus ceruleus. In
medulla oblongata (n ¼ 9), positive labeling was noted in
the neurons of the vestibular, hypoglossal, ambiguus, arcuate
and inferior olivery nuclei. During the first trimester,
immunoreactivity (IR) was also noted in the subependymal
germinal cell layer and the roof attachment area of fourth
ventricle in the medulla oblongata. The IR in the pontine
nucleus was limited in the outer areas during this period. IR
was preserved in many of the above structures during early
childhood. The spatial and temporal pattern of fukutin
expression in the brainstem structures may suggest its role
in the morphogenesis of brainstem, as well as possible
abnormalities in brainstem function of FCMD patients,
including sleep state maintenance, respiration and deglutition.
FO-4-3
Effective international collaboration in rare disease
research: DNA banking in the interest of the community
P.F. Terry a,b , S.F. Terry a,c , M.E. Davidson c , C.T. Driscoll d ,
E.W. Johnson e
a PXE International, Inc., Sharon MA, USA; b Directory of
Consumer Advocacy, Genomic Health, Inc., Palo Alto, CA,
USA; c Genetic Alliance, Washington, DC, USA; d Technology
Transfer Office, NHGRI, NIH, Bethesda, MD, USA;
e Barrow Neurological Institute, Neurogenetics, Phoenix,
AZ, USA
Susceptibility to genetic disease crosses national borders.
International rare disorder laid advocacy groups accelerate
and focus research efforts. A diverse set of international
advocacy organizations, including those falling under the
umbrella of larger groups such as The Genetic Alliance in
the United States, have created small, niche repositories of
biological materials or ‘biobanks’. These biobanks provide
an invaluable asset to the research community by streamlining
what is perhaps the most logistically difficult aspects of
searching for a gene: local ‘red tape’, including accessing
and collecting appropriate DNA samples, protecting patient
confidentiality, recontacting/redrawing patients as needed,
and making sure the patient community is regularly
informed about progress in the field, often needing to be
done in an international context. It is also an exceptional
opportunity to empower these groups and the communities
that support them and foster the global effort to find the
causes and cures for these diseases. Pseudoxanthoma elasticum
(PXE) international is a lay advocacy group that has
served as a major player in advancing research into the
biology and genetics of a rare genetic disorder, PXE. PXE
International has been able to encourage a number of
productive international collaborations in 21 countries
throughout the world that have rapidly accelerated the
research on PXE. The organization is also uniquely able
to safeguard confidentiality by acting as both a bridge and
a ‘firewall’ between researchers, participants, and govern-

392
Abstracts
mental organizations. Lessons learned from biobanking
efforts, all born of advocacy, for different diseases, using
different models, will be discussed.
FO-4-4
MeCP2 expression in human neocortex
W.E. Kaufman, M.H. Jarrar, S.M. MacDonald
Departments of Pathology, Neurology, Pediatrics, Psychiatry,
and Radiology, The Johns Hopkins University School of
Medicine, and the Kennedy Krieger Institute, Baltimore,
MD, USA
MeCP2 is a member of the methyl-CpG-binding family
of proteins, which is involved in transcriptional repression.
Rett syndrome is a disorder characterized by mental retardation
and autistic features, which is associated in a majority
of cases with mutations within the coding region of the
MeCP2 gene. Considering that MeCP2 mutations are
mainly associated with neurologic disorders, we examined
the pattern of expression of MeCP2 in normal human frontal
neocortex by immunoblotting using a battery of antibodies
targeting different domains of the protein. In
addition to our recent demonstration of extra-nuclear,
mainly postsynaptic, MeCP2 immunoreactivity, we found
that MeCP2 is expressed as two distinctive bands of ,75
and ,100 kd. Both bands are better defined under higher
denaturing conditions, suggesting that MeCP2 has a
complex conformation in brain. The most abundant 75
kd MeCP2 band is the predominant one in nuclear fractions
and in bovine whole cortex, our technical control
mammalian sample, with 75:100 kd ratios of 2.59 and
2.23, respectively. In contrast, in human whole homogenates
the 100 kd is expressed in a higher proportion with
respect to the 75 kd (75:100 kd ratio: 1.80). This suggests
that the 100 kd MeCP2, at this point of uncertain origin
(posttranslational modification versus different isoform), is
a major component of MeCP2’s extra-nuclear pool. These
findings further support the concept that MeCP2 may link
synaptic activity and other signaling processes with transcriptional
regulation in neurons, a base for the predominant
neurologic involvement shown by individuals with
MeCP2 mutations.
FO-5
Cerebral Palsy (2)
FO-5-1
The normal tissue distribution of the neuroglobin gene
and its expression under hypoxic-ischemic brain damage
H.-Y. Wang, M.-Y. Deng, C.-G. Zhang
Department of Pediatrics the General Hospital of the PLA,
Beijing, China
The globins are proteins famous for their oxygen-carrying
capacity. Two types are known in vertebrates: hemoglobin
and myoglobin. Recently neuroglobin (NGB) has
been identified, mainly in the brain, which is a monomer
with a high oxygen affinity. The globin has a distinct function
similar to myoglobin. To investigate its function to a
greater degree, we initially studied the normal distribution
of the NGB gene in the brain. Using the nucleic acid in situ
hybridization (ISH) and immunohistochemistry method,
we found that NGB mRNA and NGB protein were abundant
in the brain of the normal adult rat. A large number of
NGB mRNA and NGB-immunoreactive (NGB-IR) positive
cells were distributed in the neurons of the rat brain. They
were both distributed in the cytoplasm of neurons. Notably,
the number of NGB mRNA and NGB-IR positive cells in
the cerebral cortex was much higher than in the hippocampus
and cerebellum, especially in the piriform cortex.
Secondly, the dynamic change of expression of NGB
mRNA in cerebral tissue was studied by using an adult
rat ischemic brain damage model and reverse transcription
(RT)-PCR technique. We discovered that the expression of
NGB gene showed a time-dependent pattern. It increased
rapidly at 1 min after ischemia, kept at a high level at 5
min, then returned to normal at 15 min. It then increased
again, but more slowly. Finally, we studied the expression
of NGB mRNA in 13 different tissues of humans. The
result showed that there was high expression in fetal
kidneys and normal adult livers, apart from the brain.
FO-5-2
Types and complications of 1192 cases with cerebral
palsy
Y.-K. Li a ,Q.Li b , Y.-J. Liu b , X.-S. Wen a
a Qingdao Children Hospital, Qingdao City, China; b Rehabilitation
Center for Cerebral Palsy, Jiamusi University,
China
We analyzed types and complications of 1192 cases with
CP, come around China and hospitalized in Rehabilitation
Center for Cerebral palsy, Jiamusi University during 1986–
1997. Ages of those patients varied from 5 months to 7
years 3 months, averaged 3 years 2 months. Purpose: This
report provides basic data for studies of cerebral palsy in
China. Results: On types of CP, spastic was 715 cases
(60.0%), athetosis 287 (24.1%), mixed types 113 (9.5%),
atonic 58 (4.9%), ataxia nine (0.7%), unclassified ten
(0.8%), similar to the investigation of Narabayashi. In the
spastic CP, quadriplegia was most, 375 cases (52.5%),
diplegia 135 (18.9%), hemiplegia 83 (11.6%), double
hemiplegia 77 (10.8%), triplegia 21 (7.3%), paraplegia
13 (1.8%) and monoplegia 11 (1.5%). In the athetosis,
quadriplegia was predominant (284 cases, 99.0%), only
three cases of hemiplegia (1.0%). Complications of CP
chiefly were MR, 567 cases (47.6%), principally found in
spastic quadriplegia (53.6%), triplegia (57.1%), double
hemiplegia (46.8%), athetosis (48.1%) and mixed type

Abstracts 393
(54.0%), caused almost always by anoxia or asphyxia. But
rates of MR in spastic hemiplegia, diplegia and paraplegia
were lower (respectively 31.3, 25.2 and 15.4%). Other
complications were dysopsia (114 cases, 9.6%), microcephalia
(77, 6.5%), epilepsy (53, 4.5%), dysphasia (51,
4.3%) and dysacousis (6, 0.5%). Dysopsia mainly occurred
in spastic diplegia and quadriplegia, then microcephalia
and epilepsy chiefly in spastic quadriplegia, and dysphasia
mostly in spastic quadriplegia and athetotic quadriplegia.
In the dysopsias, internal strabismus was most (73 cases,
6.1%), occurring mainly in spastic quadriplegia (21) and
diplegia (14). Others were external strabismus (13, 1.1%),
optic atrophy (7, 0.6%), nystagmus (7, 0.6%) and dislocate
of the lens (one case).
FO-5-3
A model of combination of pediatric clinical
rehabilitation and community rehabilitation
J.-N. Mai
Neurological and Rehabilitating Department, Guangzhou
Children’s Hospital, China
In the past 15 years, many pediatric hospitals have set
up rehabilitation department. A majority of children with
neurological disorders, such as cerebral palsy, have been
treated in the pediatric hospitals in the means of ‘comprehensive
rehabilitation service program on bed’. In some
hospitals, the rehabilitation service programs were carried
out by rehabilitation doctors and their multi-disciplinary
teams. Therefore, children with neurological damages
could enjoy the rehabilitation service from acute, chronic
and periods on beds and under the clinical condition, for
example, prevention of CP could start in neonatal stage
and the treatment of CP could be infantile period. Clinical
rehabilitation on bed plays an important role in the prevention
of CP and decreases the severity of disability in the
children with high risk factors. Since the rehabilitation of
motor dysfunction and disability must be long term or for
life time, only clinical rehabilitation on bed could not meet
the needs of these disabled children. Community and home
site rehabilitation can be an ideal model which disabled
children could benefit for life. A community rehabilitation
service and home rehabilitation program can be set up with
the help and long term supervision of experts from pediatric
hospital which is in the place of top level. Therefore, a
network which includes pediatric hospital, community
rehabilitation service and home rehabilitation program
can provide effective and economical service to children
with neurological disorders. the children with high risk
factors and abnormal function can receive early intervention
program and early rehabilitation service on the hospital
base, disabled children can enjoy long term or lifetime
service on the site of community and home. Neurological
and rehabilitation department in Guangzhou children’s
hospital which is a top level hospital in the south China
has played a positive role in constructing the network of
neurological rehabilitation service for disabled children in
the communities, home and the rural areas.
FO-5-4
Interrater reliability of a visual function checklist for
cerebral palsy children with severe grade mental
retardation
C.H. Ko a , C.Y. Ko b , L. Chia b , C.C.H. Lo a , P.W.T. Tse a
a Department of Paediatrics, b Department of Ophthalmology,
Caritas Medical Centre, China
Introduction: The visual function checklist (VFC) is an
innovative behavioral tool to assess visual function in children
with severe visual impairment or poor cooperation. It
assesses the child’s response to light perception, abilities of
visual exploration, fixation, following, distance viewing,
grabbing, orientation, and the presence of optokinetic
nystagmus. Methods: The subjects included 15 children
with cerebral palsy and severe to profound grade mental
retardation residing in the Developmental Disabilities Unit
of Caritas Medical Centre. Patients with underlying
syndrome disorders, poorly controlled epilepsy, respiratory
diseases requiring oxygen therapy, concurrent acute
ophthalmic or systemic infections were excluded from
the study. Each child received repeated independent assessments
by an ophthalmologist, paediatric neurologist, and
optometrist with the VFC. The assessment was conducted
in standardized settings. The results were converted into a
visual quotient (VQ), with a range from 0 to 1. Each rater
was blinded to the others’ scores. Intraclass correlation
coefficients (ICC) were computed to determine the interrater
reliability. Results: The mean VQs measured by the
ophthalmologist, paediatric neurologist, and optometrist
were 0.592 ^ 0.397, 0.597 ^ 0.390, and 0.615 ^ 0.431,
respectively. The overall ICC was 0.873 (95% C.I.
0.695–0.961). The ICC between the ophthalmologist and
optometrist was 0.954 (0.837–0.987). The ICC between the
ophthalmologist and neurologist was 0.747 (0.378–0.911),
and the ICC between the neurologist and the optometrist
was 0.838 (0.506–0.954). Discussion: The VFC has a high
degree of interrater reliability across different disciplines of
health care professions. It is particularly useful for children
with multiple disabilities precluding accurate determination
of visual acuity. It is also a useful tool to monitor
treatment outcomes in this group of patients. (Acknowledgement:
This Project is supported by a grant from S.K.
Yee Medical Foundation 2000).

394
Abstracts
FO-6
Seizure Disorders (2)
FO-6-1
Efficacy of ketogenic diet on intractable epilepsy in
children: a report of 215 cases
M. Ghofrani
Mofid Children Hospital, Tehran, Iran
The ketogenic diet is a high fat, low protein, low carbohydrate
diet. This study is conducted to determine the
effectiveness of ketogenic diet in epileptic children refractory
to medication. A total of 215 children, age 2–12 years,
who continued to have more than two seizure attacks
weekly despite adequate therapy with at least two anticonvulsant
medications, were enrolled in this study and
followed for 1 month to 3 years. Ketogenic diet’s effectiveness
was studied at the first, second and third follow
ups (1st, 6th and 12th month, respectively). Tolerance to
diet and adverse events secondary to ketogenic diet
consumption were recorded. Two hundred and fifteen children
with mean age of 5.1 years, averaged 235 seizures per
month before the diet, despite an exposure to a mean of 6.6
antiepileptic medications were enrolled. At 1 month follow
up after the initiation of diet, 68.8% were seizure-free and
11.7% had .50% decrease in seizure frequency. A total of
98.3% of the patients had adhered to the diet regimen and
reported at 1-month follow up. At 6 months, 44.5% were
seizure-free and 21.7% had .50% decrease in seizure
frequency. A total of 89.3% had observed the diet till the
6th month and reported for follow up. At 12 months, 14.8%
were seizure-free and 12.9% had .50% decrease in seizure
frequency. A total of 57.2% continued with the ketogenic
diet for 1 year and were observed. In conclusion, the ketogenic
diet should be considered as effective treatment for
epileptic children whose seizures remain refractory to treatment.
It seems that ketogenic diet is more effective and
cheaper than many new antiepileptic medications.
FO-6-2
Low-dose synthetic ACTH therapy for West syndrome:
initial effects and long-term outcome
M. Ito
Kyoto Multi-Institutional Study Group of Pediatric Neurology,
Shiga Medical Center for Children, Moriyama, Japan
Background: Most Japanese pediatric neurologists
attempt other treatments before using adrenocorticotropic
hormone (ACTH) therapy for West syndrome (WS), and
even then, they use only a low-dose synthetic ACTH to
avoid serious adverse effects. In this multi-institutional
study, we analyzed the initial effects, adverse effects and
long-term outcome in patients treated with low-dose
synthetic ACTH in Japan. Methods: We analyzed the medical
records of 138 patients with WS, who were treated with
low-dose synthetic ACTH therapy for the first time at our
institutions between 1989 and 1998. Results: At the end of
ACTH therapy, excellent effect on seizures was noted in
106 out of 138 (76%) patients, good effect in 23 (17%), and
poor effect in nine (7%). Initial effects on EEG were excellent
in 53 out of 138 (38%) patients, good in 76 (55%) and
poor in nine (7%). As for seizure prognosis at the time of
follow-up, 51 out of 99 (52%) patients were seizure-free,
while 48 (48%) patients had seizures. Mental outcome was
normal in six out of 98 (6%) patients, mild MR in 16
(16%), moderate MR in 26 (27%) and severe MR in 50
(51%). The initial effects of ACTH on seizures and longterm
outcome were not dose-dependent (daily dosage;
0.005–0.032 mg/kg, 0.2–1.28 IU/kg, total dosage; 0.1–
0.87 mg/kg, 4–34.8 IU/kg). The severity of adverse effects
correlated with total dosage of ACTH and the severity of
brain volume loss due to ACTH correlated well with the
daily dosage and total dosage of ACTH. Conclusion: Lowdose
synthetic ACTH therapy is as effective for the treatment
of WS as the higher doses used in previous studies.
The dosage of synthetic ACTH used in the treatment of
WS can be decreased as much as possible to avoid serious
adverse effects.
FO-6-3
Neurophysiological responses to novel stimuli in infants
with infantile spasms
K.G. Werner, T. Baldeweg, R.C. Scott, S. Boyd, B.G.R.
Neville
Neurosciences Unit, Institute of Child Health, London,
United Kingdom
Objectives: Infantile spasms (IS) are strongly associated
with cognitive and social impairment, possibly related to
abnormalities in frontal/temporal neuronal networks. In
adults and older children novel environmental sounds elicit
prominent event-related potentials (ERPs) in temporal and
frontal cortices. The aims of this study were to determine
whether similarly robust novelty ERPs can be recorded in
infants and if they are abnormal in infants with IS. Methods:
Twenty-four full term infants (age range 3–10 months)
and 13 infants with IS (range 3–10 months) were recruited.
EEG was recorded continuously from 19 electrodes (10–20
system). An oddball paradigm was used with frequent
tones (80%, 1 kHz), deviant tones (10%, 1.5 kHz) and
brief novel environmental sounds (10%, 200 ms long),
delivered binaurally (interstimulus interval 700 ms) via
speakers at a distance of 30 cm. Infants were either asleep
in stages I to II or awake and feeding. Three blocks were
recorded, with 180 deviants and 180 novels. Results:
Robust and reproducible ERPs to novels were detected in
all normal infants, with peak to peak amplitudes of up to 25
mV. They consisted of two components over the temporal
(latency 250 ms) and fronto-central scalp (500 ms), respec-

Abstracts 395
tively. In all patients with IS the novelty-ERPs showed
markedly reduced amplitudes or delayed latencies in
comparison with controls. Conclusions: Novelty-ERPs
can be recorded reliably from 3 months of age. ERPs are
different in children with infantile spasms compared with
controls. The preliminary results support the hypothesis
that there is altered temporal lobe processing in children
with infantile spasms.
FO-6-4
Practice parameter: evaluating a first non-febrile
seizure in children
D. Hirtz, S. Ashwal, A. Berg, D. Bettis, C. Camfield, P.
Camfield, P. Crumrine, R. Elterman, S. Schneider, S.
Shinnar
National Institute of Neurological Disorders and Stroke,
Bethesda, MD, USA
Objective: The Quality Standard Subcommittee of the
American Academy of Neurology and the Practice Committee
of the Child Neurology Society develop practice parameters
as strategies for patient management based on
analysis of evidence. For this practice parameter, the authors
reviewed available evidence concerning the value of diagnostic
testing after a first non-febrile non-provoked seizure
in a child. Neonatal seizures and seizures lasting more than
30 min or more were excluded. Methods: Multiple searches
revealed relevant literature and each article was reviewed,
abstracted, and classified. Recommendations were based on
a three-tiered scheme of classification of the evidence.
Results: There was sufficient evidence to provide a recommendation
that an EEG be obtained routinely as part of the
diagnostic evaluation. Other studies were recommended as
based on specific clinical circumstances. Conclusions:
Further studies are needed using large, well-characterized
samples and standardized data collection instruments.
Collection of data regarding appropriate timing as well as
the choice of evaluations would be important.
FO-7
Neuroimaging (1)
FO-7-1
Prognostic value of combined use of general movement
assessment and proton magnetic resonance spectroscopy
in term infants affected by hypoxic-ischemic
encephalopathy
G. Rapisardi a , M. Cappellini b , M. Luce Cioni a , C. Ernst a ,C.
Fonda b
a NICU, Department of Pediatrics; b Pediatric Radiology
Unit, A. Meyer Hospital, Florence, Italy
Objective: To evaluate the neurodevelopmental (ND)
prognostic value of the early combined use of Prechtl’s
method of qualitative assessment of general movements
(GMs) and proton magnetic resonance spectroscopy (1H
MRS) in term infants affected by hypoxic-ischemic encephalopathy
(HIE). Subjects and methods: 24 infants born at
term transferred to our NICU for suspected HIE. All the
infants had a 1 H MRS within the 1st week after admission,
repeated GM assessments before discharge and then
between 9 and 16 weeks post-term ( fidgety age) and the
neurological outcome assessed at 12 months of age. Results:
The 11 infants with a poor ND outcome (death or CP) had a
significantly higher mean Lac/Cr and lower NAA/Cr at 1 H
MRS than the 13 infants with a normal ND outcome
(P , 0:05). All infants with normal GMs at 9–16 weeks
had a normal ND outcome. Within the 15 subjects with
signs of moderate to severe hypoxic-ischemic insults at 1 H
MRS, the four who had a normal ND outcome had normal
GMs before the discharge. Within the 18 subjects with
abnormal GMs in the 1st week after admission, the seven
who had a normal ND outcome had lower Lac/Cr and higher
NAA/Cr in the first week of life. Conclusion: In term infants
affected by HIE the combined use of GMs and 1 H MRS
increases their specific separate ND prognostic value.
FO-7-2
Non-invasive focus localization using dipole modeling
and EEG-assisted functional MRI in benign childhood
epilepsy with centrotemporal spikes
R. Boor, G. Vucurevic, G. Kutschke, T. Bauermann, S. Boor
University Children’s Hospital, Department of Child
Neurology, Mainz, Germany
The localization of epileptic foci is an important issue in
children with extratemporal epilepsies. However, the value
of non-invasive methods such as dipole modeling of EEG
spikes, and the EEG-assisted fMRI has not been sufficiently
investigated in children. As a model of extratemporal
epilepsies, we studied nine patients aged 4–13 (median
10) years with benign rolandic epilepsy. Interictal EEGs
were recorded with 23 channels and included four lower
temporal electrodes. The spikes were averaged and the
dipoles were modeled with the BESA 2000 program, and
then imported into the anatomical 3D-MRI (T1-weighted
3D-MPRAGE). Additionally, interictal spikes were
recorded during the fMRI acquisition (BOLD, 1.5 Tesla,
EPI T2-sequence, TR 6000 ms, TE 66 ms, matrix
128 £ 128, slice thickness 6 mm, 16 transversal slices) on
a MR-compatible battery-powered digital EEG system with
16 channels. The fMRI sequences were correlated off-line
with the EEG spikes and analyzed with the SPM 99 software.
EEG source analysis demonstrated the spike onset in
the central region (face or hand area) in all patients. The
fMRI results were consistent with the modeled dipoles in
five patients; we could not demonstrate fMRI activation
despite active spiking in two patients, and two patients did
not produce sufficient spikes (under sultiame treatment) for

396
Abstracts
fMRI analysis. In conclusion, we localized the interictal
spikes in the central region in patients with rolandic epilepsy
and confirmed these results by EEG-assisted fMRI. These
techniques appear to improve focus localization in children
with extratemporal epilepsies
FO-7-3
Quantitative measurement of hippocampal volume in
children with epilepsy and clinical analysis
L.-M. Ye
Department of Neurology, Tianjin Children’s Hospital,
China
Objective: We aim to measure normal hippocampal
volume of Chinese children with an age of 6–14 and the
hippocampal volume loss of patients with epilepsy, to
compare the reliability of two assessment methods, and to
study patients with hippocampal volume loss. Methods:We
selected 30 members for experiment group and 40 members
for comparison group. We used Marconi 1.5 T.MR scanner
to get hippocampal images, measured the right/left hippocampal
volume, and then performed P testament after differential
analysis. We analysed the relation between eight
clinical data and different treatment effects. We examined
the therapeutic effect of Topamax, the newly developed
medicine for epilepsy. Result: Normative hippocampal
volume: right: 0.5530 ^ 0.0458 cm 3 , left:
0.5500 ^ 0.0495 cm 3 . Right/left differential value:
0.0033 ^ 0.0250 cm 3 . The P for self-differential value and
self-differential ratio: 0.000.Visual estimation for hippocampal
loss: left: 0.0895 ^ 0.0243 cm 3 ; right:
0.07 ^ 0.0183cm 3 . Diagnosis limit: right .0.05, left
,20.05 cm 3 . Only 24.4% of traditional medicines for
epilepsy are effective. Topamax presents obvious effects
for controlling the attack of epilepsy and reaching normal
EEG. Conclusions: Selective performance of hippocamal
volume measurement is conducive to the diagnosis, treatment
and prevention of epilepsy.
FO-7-4
The utility of neuroimaging in the evaluation of pediatric
migraine and migraine-like headache
V. Farkas, A. Nyquist
Semmelweis University, Children’s Hospital, Budapest,
Hungary
The aim of this study was to collect data about the
usefulness of neuroimaging in the diagnosis of pediatric
patients suffering from migraine with aura as well as
migraine-like headache accompanied by neurological deficient
symptoms. Children and adolescents (range 6–15
years) with a complaint specifically of migraine with
aura (130 patients) classified according to the International
Headache Society (IHS) criteria and migrainous disorder
not fulfilling the IHS criteria of migraine (80 patients) were
investigated. They had attacks with severe neurological
deficient symptoms like sensory disturbances, dysphasia,
dysarthria, unilateral short lasting hemiparesis with
decreased level of consciousness. All patients underwent
at the diagnosis a skull CT scan with intravenous contrast
agent. During the follow-up period (3–8 years) 59 patients
had a further neuroimaging by using of MRI because of
progressive worsening of headache symptoms (a); longlasting
unilateral slowing of the EEG background activity
(b); newly developed headache symptoms (c): basilar
migraine, ophtalmoplegic migraine and thunderclap headache.
At the diagnosis CT scans in both group of patients
revealed various non-significant pathologies of the CNS,
but three otherwise healthy young children had findings
thought to be clinical significant: Arnold-Chiari malformation
I–II (one patient), cerebral arteriovenous malformation
(two patients). By using MRI techniques during the followup
period in 53 patients findings were normal or irrelevant
to the headache, but in two patients with long lasting
ophthalmoplegia a swollen oculomotor nerve with contrast
enhancement was present and in four additional patients
deep venous abnormality was detected. According to our
results the greater resolution and discrimination of MRI,
appeared to be of little clinical importance in the evaluation
of pediatric migraineurs. Although there are some rare,
unique headache types like ophthalmoplegic migraine,
non-aneurysmal thunderclap headache that remain a diagnosis
only by exclusion.
FO-7-5
Clinical and MR imaging study in term children with
spastic diplegia
Y. Kobayashi, S. Tanaka, A. Onuma
Division of Pediatric Neurology, Takutoh Rehabilitation
Center for Children, Sendai, Miyagi Japan
In preterm children with spastic diplegia (preterm SD),
the brain lesions on MRI have predominantly shown periventricular
leukomalasia (PVL). Lesions of SD in term birth
children (term SD) are, however, heterogeneous on MRI
and distinct from preterm SD, although both have clinically
been categorized as SD. We studied MR images of the brain
in 61 terms SD (57 singletons, four twins, 36 males, 25
females, mean age 14.2 ^ 5.1 years). The mean birth weight
was 3027 ^ 326 g. The correlation was examined between
clinical profiles and MRI findings. Our results showed: (1)
MRI findings in term SD showed various lesions, PVL in
seven, posthemorrhagic porencephaly in five, dysmyelination
in four, brain infarction in three, brain anomalies in one,
and others. No lesions on MRI were seen in 32 of 61 term
SD (52.5%). (2) Perinatal complications were not so
common, asphyxia in 14, respiratory distress in 5, seizures
in 5 and feeding difficulty in 15. (3) Mental retardation was
found in 44 children with term SD (72.1%). (4) Seizures and

Abstracts 397
EEG abnormalities were more often observed in children
with various lesions on MRI. MRI findings in term children
with SD were more heterogeneous. The most common finding
was no lesion on MRI. A different approach may
provide some answers in term SD with normal MRI finding.
Term SD seems to be associated with more severe disability
than preterm SD. The pathogenesis of term SD remains to
be studied further.
FO-7-6
Magnetic resonance imaging patterns of immediate
brain damage and their evolution following ‘Shaken
Baby Syndrome’
R.A. Minns a , T.-Y.M. Lo a , M. McPhillips b , R.J. Gibson b
a Department of Child Life and Health, University of Edinburgh,
Edinburgh UK; b Department of Paediatric Radiology,
Royal Hospital for Sick Children, Edinburgh UK
The pathological consequences of acute rotation-deceleration
injury that occur in ‘shaken baby syndrome’ are
most comprehensively seen on MRI (Barlow et al., 1999).
As part of an ongoing prospective study, a total of 72 MRI
scans were obtained from a cohort of children who were
scanned during the acute encephalopathic phase (20 infants)
and during the follow-up period (38 children). The acute
phase scan evaluation was based on the frequency of the
following observations: (1) subtemporal, suboccipital and
interhemispheric subdural haematoma; (2) tearing of pericerebral
veins; (3) compartmentalisation and varying signal
intensity of the subdural haematoma; (4) contusion and
lacerations; (5) petechiae of the corpus callosum and the
grey-white interface; (6) cerebral oedema; (7) intraventricular
and subarachnoid haemorrhages; and (8) asphyxial
injury. Seventeen of the 38 patients with long-term
follow-up scans had equential MR imagings (a mean of
four scans per child). Microencephaly occurred in 94% of
these children on follow-up and diffuse or focal white matter
atrophy was responsible for more than 50% of these cases.
FO-8
Infection/Immunology
FO-8-1
Effect of dexamethasone on nitric oxide synthase gene
expression in endotoxemia in neonate rats
H. Wang, X.-H. Du, Y.-B. Wu, B.-M. Wu
Department of Pediatrics, The Second Clinical College,
China Medical University Shenyang, China
NOS played a role in endotoxemic brain injury, which
has been increasingly concerned. The expression of three
types of NOS mRNA in the brain and effects of dexamethasone
on NOS and caspase-3 mRNA expression
were investigated by RT-PCR in postnatal 7-day Wistar
rats with acute endotoxemia established by intraperitoneal
administration of Escherichia coli LPS. The results showed
neuronal nitric oxide synthase (nNOS) and caspase-3
mRNA were faintly expressed in the brain of normal
control rats, while iNOS and endothelial nitric oxide
synthase (eNOS) mRNA could not detected. The expressions
of three types of NOS mRNA were weak at 2 h after
acute endotoxemia (LPS 5 mg/mg), peaked at 6 h, thereafter
reducing gradually to 24 h. The intensity of expression
was nNOS . iNOS . eNOS. It was found that
expression of NOS mRNA was inhibited in rats when
dexamethasone was administered together with LPS (5
mg/mg) with intensity of iNOS . nNOS . eNOS.
Caspase-3 mRNA expression was increased at 2, 4 and 6
h after acute endotoxemia, peaked at 24 h, and inhibited
partially by dexamethasone administration. The results
suggest that LPS-induced NO production induces apoptotic
death of neurons through mechanism involving the
caspase-3 activation, which may play an important role
in the pathogenesis of brain injury during endotoxemia,
and neuro-protective effects of dexamethasone may be
partially realized through inhibiting the expression of
NOS mRNA.
FO-8-2
Dynamic changes of brain derived neurotrophic factor
(BDNF) mRNA after experimental bacterial meningitis
and antibiotic treatment
L. Li, Q.-X. Shui
Affiliated Children’s Hospital, Zhejiang University School
of Medicine, Hangzhou, China
To investigate why mortality and neurologic deficits still
occur following bacterial meningitis (BM) in children with
newer and more potent antibiotics, we used in situ hybridization
to study the mRNA expression of BDNF in the brain
during the course of experimental meningitis and after antibiotic
treatment in the rats (n ¼ 42). We established the
animal models by inoculating Streptococcus pneumoniae
(SP) intracisternally, and inoculating the same volume of
normal saline suspension in uninfected control rats. BDNF
protein was also evaluated by immunohistochemistry in the
brain of SP inoculated rats. BDNF mRNA expression was
obviously up-regulated at 24 h post-inoculation (p.i.)
(0.13320 ^ 0.0275) (P , 0:01), then declined and still
stronger (0.09403 ^ 0.00383) than that of control rats
(0.06495 ^ 0.01241) on 5 days p.i. (P , 0:05), but downregulated
and nearly undetectable in the brain of rats on 5
days by antibiotic treatment for 3 days (0.03814 ^ 0.0120)
(P , 0:01). Immunochemistry showed similar changes to
mRNA expression, but the differences from mRNA expression
was that BDNF protein had declined to normal levels
on 5 days p.i. The results of the study support the hypothesis
that BDNF might play a neuroprotective role in brain
damage process of bacterial meningitis in rats. The expres-

398
Abstracts
sion of BDNF mRNA expression might be inhibited after
antibiotic treatment, and this may weaken the endogenous
neuroprotective ability. It might be one of the mechanisms
of mortality and neurologic deficits following BM. Supplementary
BDNF might be a possible therapeutic strategies to
prevent brain damage.
FO-8-3
Spectrum of movement disorders in children with
Japanese encephalitis: a clinicoradiological correlation
U.K. Misra, J. Kalita
Department of Neurology, Sanjay Gandhi PGIMS, Lucknow,
India
Background: A systematic evaluation of movement disorders
in children with Japanese encephalitis (JE) is lacking.
Aim: To evaluate the spectrum of movement disorders in
children with JE and correlate these with MRI findings.
Methods: Consecutive children with JE admitted to our
institute were included. The diagnosis of JE was based on
clinical, radiological and serological criteria. CT and MRI
were carried out. The movement disorders were documented
and their severity was graded on a 0–IV scale. Outcome
was defined at 3 months on the basis of the Barthel Index
score. Movement disorders were correlated with radiological
findings and outcome. Results: Thirty consecutive children
(eight females) with JE whose mean age was 11.2 years
were included. In the acute stage mean Glasgow coma scale
(GCS) score was 7.4 and 13 had seizures. CT scan was
abnormal in nine out of 26 revealing thalamic lesion in
nine, basal ganglia, substantia nigra and pons in one each.
Cranial MRI was abnormal in 22 out of 27 patients revealing
thalamic lesions in 19, basal ganglia nine, substantia nigra
16 and pons two. Movement disorders were noted in 25
patients after 1–4 weeks of ictus. Parkinsonian features
were present in all and dystonia in 18 patients. Dystonia
involved trunk and limbs mainly but three patients had
mouth open dystonia. Dyskinesia was present in nine
patients. There was no statistical correlation of the various
movement disorders with MRI changes (P . 0:05). Patients
with movement disorders had poorer outcome at 3 months
compared to those without (P , 0:01) and patients with
dystonia had worse outcome compared to parkinsonian
features only. Conclusion: A wide variety of movement
disorders are common in children with JE. Presence of
dystonia is associated with poor outcome.
FO-8-4
Acute necrotizing encephalopathy of childhood:
radiologic and pathologic features of thalamic lesions
M. Mizuguchi a , M. Hayashi b , Y. Nakai c , M. Itoh c ,S.
Takashima c
a Department of Pediatrics, Jichi Medical School, Minamikawachi,
Tochigi; b Department of Clinical Neuroscience,
Tokyo Metropolitan Institute for Neuroscience, Fuchu;
c Department of Mental Retardation and Birth Defect
Research, National Institute of Neuroscience, Kodaira,
Japan
Acute necrotizing encephalopathy of childhood (ANE), a
fulminant parainfectious disorder prevalent in Japan and
Taiwan, is characterized by multiple, symmetrical brain
lesions affecting the bilateral thalami, putamina and cerebral
white matter. To elucidate the pathomechanism of ANE, we
studied cranial CT and MRI findings of 14 patients, and
necropsy findings of four of them. In all the cases, CT
demonstrated thalamic hypodensity as soon as coma developed.
After the 3rd day of illness, the center of thalamic
lesions of most cases became hyperdense, producing a
concentric appearance which was further evident on MRI.
Neuropathologic examination revealed laminar changes of
vascular and parenchymal pathology. Excessive permeability
of blood vessels and resultant vasogenic edema became
more prominent with increasing depth from the cerebral
surface. The deep portion of the lesions showed severe perivascular
hemorrhage, accounting for the central high
density on CT. These results indicate that brain lesions of
ANE are caused by local breakdown of the blood–brain
barrier.
FO-8-5
The outcome of severe pediatric Guillain-Barre
syndrome seen in a tertiary hospital: supportive care
alone vs. intravenous immunoglobulin treatment and
supportive care
A.L.F. Luat
Child Neuroscience Division, Philippine Children’s Medical
Center, Quezon City, Philippines
Objective: To compare the outcome of the pediatric
patients with severe Guillain-Barre syndrome (GBS) who
received supportive care alone with the outcome of those
who received intravenous immunoglobulins plus supportive
care. Methods: This was a 6-year retrospective, descriptive,
cross-sectional study. Among the 48 patients diagnosed to
have GBS during the study period, 37 were included.
Twenty four received supportive care alone, while 13
received intravenous immunoglobulins in addition to
supportive care. The main outcome criterion was the
improvement by at least one functional grade within 28
days after the onset of illness. The secondary outcome
criterion was the ability walk unaided within 42 days of
the onset of illness. Other outcome measures were the
mean length of hospitalization, mean length of intensive
care unit stay and mean duration of intubation. Results: A
total of 69% of the patients in the IVIG group improved by
one functional grade within 28 days after the onset of illness
compared to 41% of the patients who received supportive
care alone. Similarly, 69% of the patients in the IVIG group

Abstracts 399
were able to walk unaided within 42 days, compared to 37%
of the supportive care group. The results however did not
reach statistically significant difference with p values of
0.0873 and 0.0652, respectively. There was also no statistically
significant difference in the duration of hospitalization
between the two groups (IVIG-17 days, supportive care-19
days; P-value ¼ 0.5569). Similarly, although there was a
trend towards shorter days of intubation and ICU stay in
the IVIG group, the results did not reach statistical significance.
In the supportive care group, one had hypoxic
ischemic encephalopathy and one died due to sepsis.
Conclusions: This restropective study showed that though
there was a trend towards benefits in favor of the IVIG group
in all outcome measures, the difference did not reach statistical
significance.
FO-8-6
Neuroradiological diagnostic and prognostic features of
tuberculous meningitis in childhood
S. Andronikou a , B. Smith a , R. van Toorn b , H. Douis a , J.M.
Wilmshurst b
a Department of Pediatric Radiology, and b Department of
Pediatric Neurology, Red Cross Children’s Hospital,
School of Child and Adolescent Health, University of
Cape Town, Cape Town, South Africa
We present our clinical experience of affected patients
and correlating neuroradiological features. Methods: The
study composed of a retrospective review of all CT scans,
clinical and laboratory findings in children presenting with a
provisional diagnosis of TBM between 1998 and 2001. CT
scans were assessed with regard to the presence of imaging
features such as basal meningeal enhancement, hydrocephalus,
infarction and tuberculomata. Patients were defined as
those with definite proof of TBM (TB CSF culture positive),
those with circumstantial proof of TBM (clinical presentation
and consistent CSF features) and those without
evidence of TBM. Correlation was made between the
presence and severity of meningeal enhancement with
presentation, proof of TB and outcome, initially concentrating
on the CSF culture positive group and then including the
probable group. Results: Seventy-two patient records were
reviewed, from the group 19 patients (seven M:12 F) were
CSF culture positive and 40 (24 M:16 F) were clinically
consistent with a diagnosis of TBM. The youngest patient
was 3.3 months and the oldest was 12 years with a median of
16 months for the culture positive group. The clinical
features in the two groups were summarized and statistically
compared with no significant difference found. The
commonest positive CT finding of the affected patients
was basal meningeal enhancement, followed by hydrocephalus,
cisternal high-density exudate prior to IVI contrast,
infarction and TB granulomas. Conclusion: From this an
inclusion criterion has been devised, including the neuroradiology,
to enhance the early confirmation of TBM.
Current established guidelines are lacking this.
FO-9
Genetics/Molecular Pathology
FO-9-1
Docosahexaenoic acid induced opening of voltage-gated
K channels prevents repetitive firing in neurons
D. Erichsen a , X.-P. Xu b , F. Elinder a
a Department of Neuroscience, and The Nobel Institute for
Neurophysiology, Karolinska Institute, Stockholm, Sweden;
b Department of Pediatrics, the First Hospital of Harbin
Medical University, Harbin, China
Epilepsy is a brain disorder characterized by recurrent
seizures caused by the synchronous firing of large groups
of neurons. About 25–30% of epileptic children are intractable
to the conventional anti-epileptic drug treatment.
However, the ketogenic diet (KD) could be an effective
option for them. In order to investigate the mechanism of
the KD anticonvulsant action from the effect of free fatty
acids (FFAs) and ketone bodies on voltage-gated K channels,
we carried out the study with the two-electrode voltage
clamp technique on the Shaker K channel expressed in
Xenopus oocytes and measured currents through channels
with the concentrations changed from those before treatment
to those during treatment. The results showed docosahexaenoic
acid (DHA), eicosapentaenoic acid (EPA) and
linoleic acid (LA) within clinically relevant concentrations
for the KD open the K channel by shifting the G (V) in
negative direction. A total of 30 mV DHA shifts the G (V)
curve with 22.3 ^ 0.4 mV (n ¼ 3). In experiments with
200 mg/l albumin, 100 mM DHA did not shift the G (V)
curve. At the concentration of 100 mM, EPA and LA,
respectively, shift the G (V) curve with 25.0 ^ 2.2
(n ¼ 3) and 24.0 ^ 0.4 mV (n ¼ 3). In contrast, similar
experiments performed for ketone bodies (b-hydroxybutyric
acid and acetoacetate) showed no effect at all. Computer
simulations of a simple excitable system showed that repetitive
firing is completely abolished at all stimulating
currents when the voltage dependence of the K channels
in shifted as little as 22 mV. The present findings open
up a new mechanism for anti-epileptic treatment.
FO-9-2
Glucose transporter (GLUT1) deficiency without
epilepsy, an unusual cause for developmental delay
W.C.G. Overweg-Plandsoen a , J.E.M. Groener b , O.F.
Brouwer c , H.D. Bakker d , D.C. De Vivo e
a Department of Pediatric Neurology,
b Department of
Pediatrics, Leiden University Medical Center, LEIDEN,
The Netherlands; c Groningen University Center: Department
of Pediatric Neurology; d Academic Medical Center

400
Abstracts
Amsterdam: Department of Pediatrics; e Columbia Presbyterian
Medical Center New York: Department of Neurology,
USA
The case of a 7.5 year old boy will be presented. His
psychomotor development was delayed and especially his
ability to walk remained poor due to imbalance. His
parents reported fluctuations during the day. He never
had epileptic seizures. The neurological and psychological
examinations showed a TIQ (WIPPSI-R) of 61, a cerebellar
syndrome, signs of involvement of the pyramidal tract
and dystonic posturing of the arms while walking. Examinations
for detecting the cause of the retardation were
performed. The EEG and MRI-brain were without abnormalities.
The results of the metabolic screen showed a serum
lactate of 2.3 mmol/l. Lactate in CSF was low (0.85 mmol/
l). The CSF/plasma ratio of glucose was low twice (0.31
and 0.34, normal value .0.6) indicating a deficiency of
glucose transport across the blood–brain barrier. In De
Vivo’s laboratory the GLUT1 deficiency was confirmed
in erythrocytes, DNA analysis is underway. Although this
patient does not have epilepsy he has been treated with a
ketogenic diet to provide an alternative energy source and
to try influencing his neurological symptoms. There seems
to be amelioration of the neurological symptoms after 3
months of using the diet. An overview will be presented
of the symptoms of ‘De Vivo’ disease. Epilepsy is present
in all patients except in this one. The disease is autosomal
dominant, the gene is located on chromosome 1. This is the
first case of GLUT1 deficiency without epilepsy.
FO-9-3
Immunological pathogenesis of the allergic neuropathy
induced by lipopolysaccharide of Campylobacter jejuni
Y.-X. Gao, F.-C. Cai
Department of Neurology, Chongqing Children’s Hospital,
Chongqing University of Medical Science, Chongqing,
China
Objective: To investigate the immunological pathogenesis
of neuropathy induced by Campylobacter jejuni (CJ)
LPS. Methods: (1) The specific anti-CJ LPS IgG antibody
was purified from the sera of immunized rats by affinity
chromatography; (2) pathologic examination of sciatic
nerve was performed after perineural injection by specific
anti-CJ LPS IgG antibody; (3) the affinity of the specific
anti-CJ LPS IgG antibody to the sciatic nerves of normal
rat or human was detected by immunohistochemistry
(streptavidin-biotin-peroxidase complex (SABC) and fluorescein
isothiocyanate (FITC)-immunofluorescent method);
(4) expression of the specific CJ LPS IgG in the pathological
nerves was detected by immunohistochemistry; and
(5) Expression of tumor necrosis factor alpha (TNF-a)
mRNA in damaged nerves was detected by in situ hybridization
histochemistry. Results: (1) Significant lesion of
sciatic nerves caused by the specific antibody of CJ LPS
was observed. The incidence of pathologic fibers (20.7%)
was much higher than that of control group (4.8%),
P , 0:01; (2) strong conjugation of the specific anti-CJ
LPS antibody with the sciatic nerves of normal rat or
human was confirmed by immunohistochemistry; (3) the
specific IgG was strongly deposited in nerves with axonal
degeneration and mixed lesion. However there was sparse
IgG deposition in 60% nerves with demyelination; (4) there
was high expression of TNF-a mRNA in 84% of pathological
nerves after repeated immunization by CJ LPS and no
expression of TNF-a mRNA in that of control group.
Conclusion: CJ LPS could induce peripheral neuropathy,
result in more expression of specific antibody and TNF-a
on pathological fibers. This might be an important factor in
the pathogenesis of patients of CJ-associated GBS.
FO-9-4
Platelet-derived growth factor and its receptor in
muscular dystrophies
Y.-J. Zhao a,b , K. Haginoya a , K. Iinuma a
a Department of Pediatrics, Tohoku University School of
Medicine, Sendai, Japan;
b Department of Pediatrics,
Second Clinical College of China Medical University,
Shenyang, China
We examined the immunological localization of platelet-derived
growth factor (PDGF)-A, PDGF-B, and PDGF
receptor PDGFR alpha and beta to clarify their role in the
progression of human muscular dystrophy. Biopsied
frozen muscle from patients with DMD, BMD, and
CMD were analyzed immunohistochemically using antibodies
raised against PDGF-A, PDGF-B, PDGFR alpha
and beta. In normal control muscle, neuromuscular junctions
and vessels were positively stained with antibodies
against PDGF-A, PDGF-B, PDGFR alpha and beta. In
dystrophic muscle, PDGF-A, PDGF-B, PDGFR alpha
and PDGFR beta were strongly immunolocalized in the
regenerating muscle fibers and infiltrating macrophages.
PDGFR alpha was also immunolocalized at the muscle
fibers sarcolemma and endomysial connective tissue. The
most significant finding in this study was a remarkable
over-expression of PDGFR beta in the endomysium in
DMD and CMD muscles. Double immunolabeling
revealed that activated interstitial fibroblasts were clearly
positive for PDGFR beta. These findings indicate that
PDGF and its receptors have an important role in the
proliferation of fibroblasts, which leads to muscle fibrosis
in DMD and CMD. They also have role in muscle fiber
regeneration and signaling at neuromuscular junctions in
normal and diseased muscle.

Abstracts 401
FO-9-5
A novel autosomal dominant hereditary motor and
sensory neuropathy with intermediate conduction
velocities in six children
Y. Pan a , F.P. Thomas a,b , F. Gondim a , L.J. Kinsella a , M. Al-
Lozi c , J.A. Antenor a , T.J. Geller a , S.S. Scherer d ,P.De
Jonghe e , V. Timmerman e
a Department of Neurology, Saint Louis University School of
Medicine, St. Louis, MO, USA; b St. Louis Veterans Administration
Hospital, Institute for Molecular Virology, Department
of Molecular Microbiology and Immunology, Saint
Louis University; c Department of Neurology, Washington
University, St. Louis, MO, USA; d Department of Neurology,
University of Pennsylvania Medical Center, Philadelphia,
PA, USA; e Peripheral Neurology Group, Department of
Neurology and Molecular Genetics, University of Antwerpen,
Antwerpen, Belgium
Charcot-Marie-Tooth disease (CMT) encompasses multiple
heterogeneous hereditary neuropathies linked to numerous
genetic loci and genes. We diagnosed CMT in five girls
and one boy, ages 5–14, among 20 children from a fourgeneration
family with a novel genotype and phenotype.
Walking age was normal; disease onset was in the 1st decade.
The central and autonomic nervous systems and cranial
nerves were uninvolved. Heel walking was impaired in
five; otherwise strength was normal. Tremor was present in
four, distal atrophy in one. Hyporeflexia was present in three.
Sensation was impaired in five. Romberg sign, nerve enlargement
and foot deformities were absent. Neurophysiologic
studies were normal in two children (ages 5 and 9). One child
(age 7) had borderline sural nerve conduction velocities
(NCV, m/s) of 41; a 2nd (age 8) had abnormal NCVs of 42
(peroneal), 39 (tibial) and 40 (sural); a 3rd (age 10) had a
borderline motor peroneal amplitude; a 4th (age 14) had
NCVs of 45 (median), 32 (peroneal) and 37 (tibial), low
sensory nerve action potential amplitudes and distal muscle
denervation. No peripheral myelin protein 22, myelin protein
zero, connexin-32, early growth response gene 2, or neurofilament
light chain gene mutations were found. Linkages to
the CMT1A, CMT1B, CMT1D, CMT2A, CMT2D, CMT2E,
CMT2F loci and the intermediate NCV loci on chromosomes
19p12–p13.2 and 10q24.1–q25.1 were excluded. A genomewide
search is in progress. This study describes the clinical,
electrical and genetic features of a novel, axonal and demyelinating
autosomal dominant polyneuropathy with intermediate
NCV; a new gene locus is likely.
FO-9-6
The value of serum neuron specific enolase and SPECT
rCBF imaging in children with status epilepticus
Y. Ouyang, Q. Peng, F.-G. Mu
Sichuan Provincial Hospital, No32, first ring road in
western, Chengdu, China
Objective: To explore the value of serum neuron specific
enolase (NSE) and SPECT rCBF imaging in the evaluation
of brain injury of SE in children. Methods: Serum NSE was
detected through ABC-ELISA method. Serum NSE was
performed in 28 status epilepticus within 24 h after seizure
attacks, and in 40 normal controls. Ten of SE cases had
serum NSE detection at 1, 3 and 7 days. SPECT rCBF
imaging was performed in 15 of status epilepticus. Results:
The mean serum NSE in SE patients was 15.13 ^ 6.22 mg/l,
which was higher than that of normal controls(mean
2.635 ^ 0.765 mg/l, P , 0:001); the serum NSE returned
to normal at 7th day and these children’s long-term outcome
was better. The SPECT rCBF imaging abnormal rate in 15
SE was 73.4%. In SE patients with acute etiological factors,
rCBF abnormal region was larger than those with chronic
etological factors. Conclusion: Status epilepticus in children
can cause brain injury. The serum NSE is a reliable marker
of brain injury. Observation of serum NSE may predict
long-term neurological outcome. SPECT rCBF imaging
abnormalities in childhood status epilepticus might be of
help in the evaluation of brain injury. Therefore, serum
NSE and SPECT rCBF imaging in children with SE may
be very useful to assess the degree brain injury and longterm
outcome.
FO-10
Neuroimaging (2)
FO-10-1
Variability in clinical and neuroimaging presentation in
congenital bilateral perisylvian polymicrogyria (CBPP)
in pediatric patients
R.M. Valério, F.N. Arita, S. Rosemberg
Neuropediatrics Division, Department of Pediatrics, Santa
Casa de Sao Paulo Medical School, Sao Paulo, Brazil
CBPP refers to a clinicopathological syndrome characterized
by a particular type of neuronal migratory disorder
easily detected through MRI. The most frequent clinical
signs include dysarthria, abnormal tongue movements,
dysphagia, pyramidal signs, epilepsy and mental retardation.
We report ten patients calling attention to the variability
of clinical presentation and MRI findings in CBPP.
There were five males and five females. There were no
familial cases. The pregnancy was uneventful in eight.
Toxoplasmosis was detected and treated during the 4th
month of pregnancy in one case and in the remaining, the
mother was drug addicted. Age at first consultation ranged
from 7 months to 14 years (mean: 5.2 years). Eight patients
were referred for developmental delay, one for seizures and
one for speech disturbance. Four patients were microcephalic,
and macrocephaly was observed in one. Three patients,
all under 2 years of age were severely handycaped. Motor
examination was normal in two patients. Pyramidal and/or
extra-pyramidal signs were observed in the remaining five.

402
Abstracts
Pseudo-bulbar sings were detected in nine patients. Six
patients had epilepsy whose onset varied between ages of
10 months and 12 years (mean: 3.5 years). MRI revealed
relatively symmetrical bilateral perisylvian polymicrogyria
in nine patients and in one the alteration predominated on
the right side. In addition, bifrontal periventricular nodular
heterotopia, bifrontal schizencephaly, and biparietal schizencephaly
were associated in three cases. In conclusion,
CBPP is not a homogeneous cortical developmental malformation.
It is worth mentionning that in this series of no
familial cases, the incidence of intractable epilpsy was
30% and CBPP may be accompanied by other disorders
of cortical development.
FO-10-2
Attention deficit hyperactivity disorder: subtypes and
1 H-magnetic resonance spectroscopic characteristics
Z. Jin a , Y.-W. Zeng a , L. Sun b , G. Liu a , Y. Wang a , Y.-F.
Wang b
a fMRI Center, Hospital 306, Beijing, China; b Mental Institute,
Peking University, Beijing, China
Background: According to DSM-IV, the patients with
ADHD could be divided into three clinical subtypes, those
were different in demographic characteristics, functional
impairment, and comorbidity with other disorders. Thus,
we hypothesized that there would be different neurochemical
status among the patients. Methods: Thirty-four
untreated school children suffering from ADHD (inattentive
subtype ADHD-IA, n ¼ 19; hyperactive/impulsive subtype
ADHD-HI, n ¼ 4; combined subtype ADHD-C, n ¼ 11)
were investigated by using 1 H-MRS. Spectra were acquired
bilaterally in the globus pallidus. Peaks of NAA, Cho, myoinositol,
glutamate and creatine (Cr) were measured and
their ratios were calculated and compared with data from
matched controls. Results: All three subtypes showed significant
decreased NAA/Cr ratios comparing with normal
controls. Among them the ADHD-C subtype had the lowest
NAA/Cr value. ADHD-IA and ADHD-C subtypes appeared
mild increased Cho/Cr ratios, while the value in ADHD-HI
group was decreased. Conclusion: These findings suggested
that striatal neuronal death or dysfunction exits in all the
three ADHD subtypes. Among them ADHD-C subtype was
the most severe one. There seemed to be a mild hyperactivity
of the cholinergic system in ADHD-IA and ADHD-C
subtypes, but not the ADHD-HI subtype.
FO-10-3
Single photon emission computed tomography (SPECT)
in attention deficit hyperactivity disorder (ADHD) –
baseline and post intervention
S. Gulati a , V. Kalra a , C.S. Bal b , H. Zamir a
a Departments of Pediatrics and Nuclear Medicine; b All
India Institute of Medical Sciences, New Delhi, India
Background: ADHD is a common childhood neurobehavioral
disorder. Objective: To study the cerebral perfusion
abnormalities in children with ADHD. Materials and methods:
A randomized double blind placebo controlled trial to
evaluate the efficacy of a polyherbal formulation, Mentat
(Bacopa monnieri, Withania somnifera, Centella asiatica,
Nardostachys jatamanasi) in 60 children with ADHD
(DSM IV criteria). Subjects were randomised into Mentat
(30) and placebo groups (30). Cerebral perfusion was evaluated
using 99mTc ethyl cystine dimer brain SPECT in a
subset of 37 children whose parents gave consent. SPECT
images were acquired using a dual-headed gamma camera
system with fan beam – collimator (Elscint varicans).
SPECT scans were performed at baseline (37) and if abnormal
(26), repeat at 6 months. Consent for post intervention
repeat scan was obtained in 12/26 with abnormal SPECT.
Results: Thirty-seven ADHD children age range 5–14
years, boys predominated (30). Abnormal cerebral perfusion
was seen in 26/37 (70.2%) (P ¼ 0:006). Twenty-four/
37 (64.8%) had hypoperfusion in thalamus and/or basal
ganglia. Two had temporal and basifrontal hypoperfusion
respectively. Among 12 follow up scans, decoding at the
end of the trial revealed six from either group. In Mentat
group 3/6 (50%) showed post treatment normalization of
perfusion abnormality versus 1/6 (16.6%) in placebo group
(P ¼ 0:11). Conclusions: A statistically significant proportion
of children with ADHD had thalamic and/or basal
ganglia hypoperfusion. The area identified may help to
understand the anatomic substrates in this entity. SPECT
abnormalities may in future be used to evaluate and monitor
therapy outcomes in ADHD.
FO-10-4
Correlation of long-term intellectual and
neuropsychological effects of closed head injury in
infants and children using proton MR spectroscopy
S. Ashwal, B.A. Holshouser, T. Brenner, M.C. Freier, T.
Burley
Radiology and Psychology, Departments of Pediatrics,
Loma Linda University, Loma Linda CA, USA
The ability to predict long-term neurologic and neuropsychological
outcome in 22 children, ages 1 week to 14
years at the time of closed head injury (CHI), were investigated
using proton magnetic resonance spectroscopy
(MRS) acquired post injury and compared to standardized
neurologic, intellectual, and neuropsychological testing
done 1–7 years later. Clinical indicators of acute injury
severity including age at injury, electroencephalography,
MRS variables of NAA/Cho, Cho/Cre, and lactate presence
accurately classified children as functioning above or
below the average range for most outcome measures.
Combined clinical and MRS variables accounted for
approximately 50% of the variance in cognitive and
neuropsychological outcome confirming the validity of

Abstracts 403
their predictive use. Of the injury severity indictors,
presence of lactate is a particularly important prognostic
marker of poor long-term intellectual and neuropsychological
functioning. Our findings indicate the potential for
providing accurate estimates of long-term intellectual and
neuropsychological functioning after CHI in infants and
children using proton MRS in combination with clinical
variables.
FO-11
Morphogenesis
FO-11-1
Partial trisomy 16 mice model for Down syndrome: an
analysis of brain morphology and physiology
P. Belichenko a , A. Kleschevnikov a , E. Masliah b ,W.
Mobley a
a Department Neurology and Neurological Science, Stanford
University Medical Center, Stanford; b School of Medicine,
UCSD, La Jolla, USA
Partial trisomy 16 mice (Ts65Dn) are a genetic model
for Down syndrome. The medial and lateral septum, fascia
dentata (FD), CA1 area of hippocampus, motor and somatosensory
cortices were studied. Sections for confocal
microscopy were immunostained for synaptophysin (p38),
or were examined after neuronal microinjection with Lucifer
yellow. Electrophysiological experiments were
performed on hippocampal slices. Relative to controls,
the area of p38-IR was significantly increased in cortex,
hippocampus, FD and medial septum of Ts65Dn mice at 21
days, 3, 6 and 16 months of age. In granule cells of FD, the
number of dendritic spines was decreased in Ts65Dn mice
at 21 days, 3 and 6 months of age. In Ts65Dn mice, spine
head area was significantly increased while the length of
spine neck was decreased. In all areas investigated in
Ts65Dn mice, EM showed enlarged and tortuous neurites
with abundant electrodense, laminated bodies and occasional
synaptic vesicles and spines were distended with
vacuolization of the endomembrane system. There was a
defect in induction of long-term potentiation (LTP) of the
perforant path input to the FD in Ts65Dn mice: LTP was
suppressed at 45–60 min after the first tetanus. Furthermore,
paired-pulse depression of the population spike
observed in FD was significantly stronger in the Ts65Dn
mice, suggesting enhanced efficiency of the feed-back inhibitory
system. Our data show dramatic changes in both
morphology and function of synapses in Ts65Dn mice.
The defects are seen in both developing and mature
Ts65Dn mice. Further studies are required to show whether
or not these abnormalities are also present in the brain of
Down syndrome patients. Supported by NIH grants
AG16999 and NS38869.
FO-11-2
Clinical spectrum of holoprosencephaly: a clinicalneuroradiologic
analysis
J.S. Hahn, L.L. Plawner, M. Delgado, V. Miller, E. Levey,
S.L. Kinsman, A.J. Barkovich, E. Simon, N. Clegg, V.
Sweet, E. Stashinko
Stanford University School of Medicine, Department of
Neurology, Stanford, Texas Scottish Rite Hospital, Dallas,
Kennedy Krieger Institute, Baltimore, University of California
San Francisco, San Francisco, Children’s Hospital of
Philadelphia, PA, USA
Holoprosencephaly (HPE) is a brain malformation that
results from incomplete cleavage of the prosencephalon
into two hemispheres. We evaluated 68 children with HPE
with history, developmental assessment, and physical examination.
Neuroimaging studies were assessed for the grade of
HPE (from least to most severe: lobar, semilobar, and
alobar), the degree of non-separation of the deep gray nuclei,
and presence of dorsal cyst or cortical malformation. In
general, the severity of clinical problems and neurological
dysfunctions paralleled the grade of HPE. Dystonia was
correlated with the degree on non-separation of the caudate
and lentiform nuclei, as well as, the grade of HPE (P , 0:05).
Hypotonia was correlated with the grade of HPE (P , 0:05).
Mobility, upper extremity function, and language were all
significantly correlated with the degree of non-separation of
the caudate, lentiform, and thalamic nuclei and grade of HPE
(P , 0:01). Seizures occurred in approximately half of the
children with HPE. The presence of cortical malformation
was associated with seizures that were difficult to control.
Endocrinologic dysfunction was noted in 72% of the patients
with all having at least diabetes insipidus. The severity of
endocrine abnormality correlated with the degree of
hypothalamic non-separation (P ¼ 0:029). There was a
correlation between the severity of the facial malformation
and the grade of HPE (P ¼ 0:032), but there were many
exceptions. In conclusion, this study shows that by combining
the detailed neuroradiologic features of HPE with clinical
assessments, we were able to develop a more accurate classification
scheme for predicting outcome and clinical
problems.
FO-11-3
Role of intrauterine infection for the pathogenesis of
epilepsy in children at the early stage of life
S.A. Gulyaev, A.A. Ovchnnickova, S.E. Gulyaeva, I.V.
Archipenko, L.V. Viborova
Vladivostok State Medical University, Vladivostok, Russia
Distinctive clinical signs of epilepsy in 79 children at the
age up to 5 years born from mothers infected by sexual
relations and having high percentage of miscarriages, stillborn
fetus, pathology in pregnancy and delivery were

404
Abstracts
studied. It was found that among all examined children 71%
of babies were carried to full term, 57% had signs possibly
due to intrauterine hypoxia, or perinatal asphyxia, 73.4%
had signs of intrauterine infection, and 16.5% had slight
traumatic destruction (of the nervous system). At the early
neonatal period incidence of epilepsy reached 27.5%.
Among all kinds of epilepsy generalized form was prevalent
(63.6%). Hemangioma was found in 45.5%, and subarachnoidal
hemorrhage – in 41.4%. Central nervous system
symptoms were observed only in 1/3 of all patients at this
stage. No further information was obtained. The pathological
lesion at the early years of life was most precisely
recorded by neurosonography that was useful to see changes
of brain structure (77.3%). The findings were characterized
as a stable increase of echodensity in parenchyma in 17.6%,
and as inborn defects of brain structure development in
5.8%. This research demonstrated not only early clinical
signs of epilepsy in children born to mothers infected by
sexual relations but also severe pathology with changes in
the brain structure caused by infectious agents, influencing
the nervous tissue. We conclude that prevention of brain
pathology in these prenatally affected children is an urgent
issue in child neurology.
FO-11-4
Epileptic chromosomopathies: epileptic syndromes of
childhood due to chromosomal disorders
P.A. Hwang, L. Chen, J. Shaw, P. Wyatt
Departments of Paediatrics and Genetics, North York
General Hospital, Bloorview Epilepsy Research Program,
University of Toronto, Toronto, Canada
A retrospective review of over 1500 patients in a community-based
practice revealed about 2% of children with chromosomal
disorders, detected by karyotype, fluorescent in-situ
hybidization (FISH), methylation studies or specific DNA
probes. In addition to the usual trisomy 21 in Down syndrome,
two had balanced Robertsonian translocation, 15 had Angelman
Syndrome, with and without deletions at 15q11–12 loci,
ten had Rett syndrome and one each had Smith-Magenis with
17p and Wolff-Hirschorn with 4p deletions respectively. The
clinical features were quite characteristic although not specific:
dysmorphic features, developmental delay, and multiple
seizure types: partial and generalized, often refractory to antiepileptic
drugs. The EEGs were variable: disorganized background
activity, multifocal epileptiform discharges, and
characteristic features in certain syndromes: central spikes,
pseudoperiodic discharges of Rett syndrome, and stimulussensitive
central-parietal spikes of Angelman syndrome.
Certain chromosomal disorders have a high risk of epileptic
seizures: Angelman (but not Prader-Willi), Rett and Wolff-
Hirschorn, but not Prader-Willi, Smith-Magenis or Down
syndrome. Only in the Angelman syndrome does the deletion
at 15q11–12 involve GABA-A receptor subunit loci and may
be considered to constitute an epileptic chromosomopathy.
FO-12
Genopathies
FO-12-1
The frequency of common mutations among patients
with mucopolysaccharidosis types I, II and IIIA
diagnosed in Austria over the last 17 years
Th. Kroepfl, K. Paul, B. Plecko, E. Paschke
University of Graz, Department of Pediatrics, Graz, Austria
The MPS are a hereditary, clinically heterogeneous group
of 11 disorders caused by the deficiency of lysosomal
enzymes involved in mucopolysaccharide catabolism.
Their phenotypes include growth retardation, dysostosis,
enlargement of visceral organs, progredient psychomental
retardation and reduced life span. In populations originating
from Northern European and Anglo-American countries,
three enzyme defects comprise the majority of cases,
namely a-l-iduronidase, MPS I, iduronate-2-sulphatase,
MPS II, and heparan N-sulphatase, MPS IIIA. Several mutations
have been shown to be common among European
cases and genotyping has become essential for genetic counselling
and possible prediction of clinical phenotype. Therefore,
we started to define the genotype of all cases diagnosed
enzymatically in Austria between the years 1983–2000.We
initially tested 66 cases for common mutations in MPS I, II
and IIIA. Similar to other European countries the majority
of cases were MPS I (23), MPS II (21) and MPS IIIA (22).
Taken together, the frequency of common mutations in
MPS I and IIIA among patients diagnosed in Austria was
found to be slightly below the average distribution found in
other European countries with a reversed ratio of the
frequency of the main common mutations Q70X and
W402X in MPS I. Moreover, S66W, a mutation common
in Italian populations, was found to be unexpectedly
frequent in MPS IIIA. The methods for the discovery of
these mutations are simple and reliable. We therefore
suggest that W402X and Q70X in MPS I as well as R74C,
R245H and S66W in MPS IIIA should be assayed routinely
after identification of the enzyme defect. This would help to
avoid time-consuming cell cultivation procedures and thus
be helpful in situations of late amniocentesis.
FO-12-2
Spectrum of mutations and phenotype/genotype
correlations in muscle-eye-brain (MEB), another defect
of glycosylation
H. Pihko, C. Diesen, J. Dieguez-Lucena, W. Dobyns, P.
Santavuori, A.-E. Lehesjoki
Hospital for Children and Adolescents, HUS, Helsinki, and
Department of Medical Genetics, University of Helsinki,
Finland
MEB disease is a recessively inherited disorder charac-

Abstracts 405
terized by a severe brain malformation, ocular abnormalities
and muscular dystrophy. The disease was first described in
Finland 20 years ago, but after the localization of the gene to
1p32, we have been able to identify MEB-patients from
several countries around the world. Although the clinical
severity of MEB is more variable than previously thought,
we were able to exclude patients with Walker-Warburg
syndrome from the MEB locus, thus showing that these
two disorders are caused by different genes. The defective
gene, O-mannose b-1,2-N-acetylglucosaminyl transferase
(POMGnT1), places the disease into the increasing group
of inherited disorders of glycosylation. We have analyzed
mutations of POMGnT1 in Finnish as well as in patients
from different nationalities. So far, one mutation, G to A at
position 5750, has been found in 18 Finnish patients studied.
The results of this mutation analysis as well as genotypephenotype
correlations will be presented.
FO-12-3
Septo-optic dysplasia: a new phenotype associated with a
heteroplasmic mutation in the mitochondrial
cytochrome B gene
M. Schuelke a , H. Krude b , B. Finckh c , E. Mayatepek d ,J.
Smeitink e
a Department of Neuropediatrics, b Department of Pediatric
Endocrinology, Charité University Hospital, Berlin,
Germany; c Children’s Hospital, University of Hamburg,
Germany; d Division of Metabolic and Endocrine Diseases,
University Children’s Hospital, Heidelberg, Germany; e Nijmegen
Center for Mitochondrial Disorders at the Department
of Pediatrics, University Medical Center, Nijmegen,
The Netherlands
Septo-optic dysplasia (de Morsier syndrome, MIM
182230) is characterized by hypoplasia of the optic nerve,
agenesis of the septum pellucidum, and hypothalamic
dysfunction. The most frequent endocrine defect is growth
hormone deficiency. Most cases occur spontaneously and
their genetic basis is unknown. Recently a missense mutation
in the homeobox gene HESX1 was detected in one familial
case. Here we present a patient in whom a mutation in HESX1
was excluded but in whom we detected an isolated respiratory
complex III-deficiency due to a new heteroplasmic
mutation (14849T . C) in the mitochondrially encoded
cytochrome b gene. The 25-year-old patient suffered from
septo-optic dysplasia, retinitis pigmentosa, lactic acidosis,
exercise intolerance, hypertrophic cardiomyopathy and
rhabdomyolysis. The mutation causes a replacement of the
serine35 by proline at the ubiquinone (Q i ) binding site. This
interrupts the electron flow from complex II to cytochrome c.
Lack of ATP due to malfunction of the respiratory chain
could explain the exercise intolerance and lactic acidosis
but not the septo-optic dysplasia or the retinitis pigmentosa.
The latter condition hints to free oxygen radicals as putative
pathogenic agents. A low a-tocopherol concentration in the
muscle, a reduced total radical trapping parameter of the
plasma and an increased excretion of leukotriene E 4 in the
urine are compatible with increased reactive oxygen species
(ROS)-generation. Morphogenesis of the brain demands a
fine tuning between proliferation and apoptosis. We hypothesize
that in our patient a disturbance of this balance due to
increased ROS-concentration and a subsequent increase in
apoptosis might have resulted in deranged fetal brain development
such as seen in septo-optic dysplasia.
FO-12-4
Identification of the genetic defect in an Asian-Indian
community with megalencephalic leukoencephalopathy
and cysts
B.S. Singhal a , E.P. Hoffman b , F.-F. Wu b , D. Stephan b ,S.
Naidu c , R. Gorospe b
a Bombay Hospital, Department of Neurology, Institute of
Medical Science, Medical Research Center, Bombay,
India;
b Children’s National Medical Center, Research
Center for Genetic Medicine, Washington, DC; c Kennedy
Krieger Institute/Johns Hopkins Medical Institute, Division
of Neurology and Pediatrics, Baltimore, MD, USA
We screened the entire coding region of the MLC1 gene in
a group of 23 unrelated Asian Indian patients from one ethnic
group-Agarwals-who presented with a clinical syndrome
characterized by early-onset megalencephaly, leukoencephalopathy
with cysts, progressive spasticity, seizures, and
learning problems. The majority of the patients (22/23, 96%)
were homozygous for an insertion of cytosine in exon 2
between nucleotides 250 and 251, indicative of a recessive
disorder with a founder effect. One patient (1/23, 4%) was
homozygous for C585A; however, he was adopted and
parental details were unknown. Despite the commonality
of the gene defect in this ethnic group, there was significant
phenotypic variability indicative of possible environmental
factors or individual genetic background as contributors.
These novel mutations in MLC1 gene indicate that this clinical
syndrome among Asian Indian Agarwals is the same as
that among Turkish and European patients. Individuals from
this Indian community live in various parts of the world.
When a patient from this ethnic group presents with the
above-mentioned clinical and neuroimaging features, mutations
in the MLC1 gene should be sought.
FO-13
Higher Cortical Functions
FO-13-1
Reactus: a new cognitive function diagnostic device
B. Scott, S. Freiger, J. Rehrmann
Trifolium, ideas and solutions, Kassel, Germany
The ‘Reactus’ consists of integrating medical test proce-

406
Abstracts
dures into a comprehensive system where visual reaction,
audio reaction, psycho-motor abilities, visual-motor coordination,
attentiveness and concentration are examined. The
doctor will be able to analyze the cognitive function of
children and adults in a 5-min test procedure and the results
can be printed in an easy to understand graph. The Reactus
evaluates various abilities of an individual including: *starting
reflex, *surprise reactions, *visual and auditory reaction,
*visual and auditory differentiation, *visual and auditory
reliability, *constancy of reactions, *attentiveness,
*concentration, *strategy, *psycho-motor abilities,
*visual-motor coordination, *short-and long-term endurance.
The user-friendly software can enable the doctor to
perform further analysis using SPSS data. Researches in the
field of ADHD, LD, Turner, diabetes mellitus, effects of
medicine and very low birth weight (VLBW) had been
performed in Japan (National Children’s Hospital Tokyo,
Teikyo University, Saitama Municipal General Center,
Tokyo Metropolitan University of Health Sciences, Jichi
Medical University and Tokyo Gakugei University); in
China (Hua Shan Hospital, Shanghai); in Germany (Leipzig
University). All doctors are connected via the Internet and
the research activities were printed in a bi-monthly email-
Newsletter. The results of Reactus display a more specific
picture about the abilities of children and adults.
FO-13-2
Environmental mercury exposure in children with
autistic spectrum disorder (ASD): A case-control study
P. Ip, H.K. Ho, J. Lee, W. Wong, V. Wong
Division of Neurodevelopmental Paediatrics, Department
of Paediatrics, Queen Mary Hospital, The University of
Hong Kong, Hong Kong SAR, China
Objective: To study whether there is any increased
environmental mercury exposure in children with ASD.
Methods: A cross-sectional study was performed from
April to September, 2000. Simultaneous hair and blood
mercury levels were analysed in 2 groups of children: (i)
ASD; and (ii) control. A questionnaire on sociodemographic
data, dietary habits and other risk factors for environmental
mercury exposure was completed. Results:
Altogether 82 autistic (aged 7.2 years) and 55 normal children
(aged 7.8 years) were recruited. There was no difference
in the mean hair mercury level of autistic group (2.3
ppm) as compared with control group (mean 2.1 ppm)
(P ¼ 0:79). The mean blood mercury level of autistic
group (19.5nmol/l) compared to control group (14.7
nmol/l) (P ¼ 0:056). The mean hair mercury level in
both groups of children (n ¼ 137) was 2.2 ppm, even
higher than the levels of adults in Europe (1.2 ppm) and
U.S. (1.5 ppm). Hair mercury levels correlated well with
blood mercury levels in our children (r ¼ 0:88). The
frequency of fish consumption was the only independent
variable correlated with hair (r ¼ 0:51) and blood
(r ¼ 0:54) mercury levels. For those children who
consumed fish more than three times per week, their hair
and blood mercury levels were twice than those who
consumed fish less than three times per week and three
times of those who never consumed fish. Five autistic children
had toxic blood mercury levels (.45 nmol/l). Their
family members were screened and half of them also had
toxic mercury levels. However, some had features of
mercury poisoning. The mean blood mercury level of
those with mercury intoxication dropped from 70.5 to
26.8 nmol/l (P ¼ 0:0001) after reducing fish consumption
for 3 months. Conclusions: There was no significant difference
in the hair and blood mercury levels between autistic
and normal children. All our children had high hair
mercury levels which correlated well with blood mercury
levels. Frequency of fish consumption correlated with hair
and blood mercury levels in Hong Kong children. Thus
there is an overall increased risk of environmental mercury
exposure.
FO-13-3
A study to use modern IT device in facilitating written
communication of children with developmental dyslexia
C.W. Chan, K.Y. Cheng, C.C. Lam, T.F. Ho, C.K. Leong
Working Party on Specific Learning Disabilities (SLD), The
Hong Kong Society of Child Neurology and Developmental
Paediatrics (HKCNDP), China
The aim of this study was to explore the feasibility and
effectiveness of using Cantonese speech-to-text input technology
as a tool for dictating Chinese characters into word
processing applications in facilitating written communication
of children with dyslexia. Fourteen Hong Kong children
with dyslexia, aged 9–14, and three without dyslexia,
aged 10–15, were trained. In the six training sessions, participants
were familiarized with the use of the input device
and guided to personalize the associated speech template,
input specially designed reading texts into the computer,
and complete two oral pictorial composition exercises.
These sessions were conducted with the help of parents
and volunteer workers. Usefulness of the technology was
evaluated with regard to both the written product and to
process factors. Participants’ written output was analyzed
for recognition accuracy rate for different types of writing
tasks. Through analysis of structured feedback from helper
observations, parent questionnaires, and interviews, difficulties
encountered and factors noted to contribute to
success in the training and trial processes were studied.
Results indicated high individual variability in the effectiveness
of using this tool. Implications for the improvement
of this Cantonese speech-to-text technology as well
as its potential application for facilitating the learning of
children with dyslexia at home and school will be
discussed.

Abstracts 407
FO-13-4
The ‘learning brain’ and environment: Pavlovian
philosophy
K.B. Kulkarni
Sanjivani Nursing Home, Gwalior (M.P.), India
The ‘learning brain’ and environment implies unity of
organism and its social and ecological environment which
becomes manifest during the early phase of neuropsychic
development of a growing child. This has important applications
in neonatology, developmental Pediatrics, disorders
of affect, even environmental Pediatrics. Interrelationships
between body and environment are realised through conditioned
reflexes which by perseverence and practice can be
transformed into instinctive, even herd reflexes (genetic
transmission). Relationship between interoceptors from
viscera and exteroceptors from environment is inseparable.
We must appreciate the claims put on newborn CNS by
adjustment to environmental influences. The newborn
cortex is under protracted protective inhibition resulting in
continuous sleep. Powerful stimuli for conditioned reflexes
inducing sleep are fresh air or breeze, quiet environment,
certain body positions, light clothing and even mother’s
sweet song or words. Even the experimental model of cat
newborn for studying the ontogenesis of breast feeding
related behaviour draws parallels from Pavlovian ethos
which has great bearing on the human situation of breast
feeding. Principal Pavlovian idea is the process of ‘imprinting’
which answers ‘No’ for the question: should parents
prevent children from being exposed to life’s dangers and
difficulties? Pavlov’s observations lay the ground work for
this by observing behaviour of animal newborns under
adverse circumstances. Birdies under comfortable environment
loath to build nests later. Pavlov further thought that
Nature has worked out detailed programme of behaviour for
each animal species including Man but has consciously left
numerous gaps to be filled by ‘imprinting’ –a form of
conditioned reflex activity.
FO-13-5
Conversion disorder: under diagnosed? Over
investigated?
P.M. Leary
Bristol Royal Hospital for Children, Bristol, United Kingdom
The term ’conversion disorder’ (DSM-IV) is applied
when symptoms and deficits involving voluntary motor
and sensory function suggest a neurological or other physical
condition, which in fact is not present. In Britain and
almost certainly elsewhere as well such cases are regularly
encountered by paediatric neurologists and place significant
demand on consulting time and diagnostic resources. The
widely diverse manner in which organic neurological disorder
may present creates a dilemma in the diagnosis and
management of conversion disorders. While there is real
need to exclude organic disease the thoroughness with
which this is often done may strengthen patient and parental
conviction that there must be a physical explanation for the
symptoms. Management may be further complicated by
anxiety about litigation, parental refusal to accept an
emotional cause for symptoms, ’Gillick competent’ patient
refusal to accept intervention and a community shortage of
resources for treating children and adolescents with
emotional problems. In this study illustrative cases are
presented, diagnostic procedures reviewed, management
strategies discussed and guidelines suggested.
FO-13-6
Trial of risperidone in children with conduct disorders
A. Dayan-Nahmad, S. Mann-Shvili, A. Gonzalez-
Astiazaran, M.-A. Collado-Corona, C. Martínez-Wbaldo
Centro Nacional de Rehabilitación, Instituto de la Comunicación
Humana, México City, Mexico
Objective: To study the use of Risperidone in children
with behavior dysfunctions. Method: An open-labelled
prospective trial was conducted for the use of risperidone
from July 2000 to December 2001 in 220 subjects with
conduct disorder. A total of 112 were excluded as they
received another drug or abandoned the treatment. Thus,
108 (80 boys, 28 girls) were enrolled in the study 95 had
additional language problems and 30 also had hypoacusia.
The ages were 2–13.11 years. Patients were seen monthly
throughout the trial. Risperidone was increased at monthly
intervals with an initial dose of 0.027 mg/kg per day,
depending on symptom control. Results: Risperidone was
associated with clinical improvement at a dose of 0.027–
0.15 mg/kg per day. Almost all patients showed minor
adverse effects, (initial drowsiness and weight gain).
None-developed extrapyramidal side effects. Conclusions:
These data provide preliminary evidence that risperidone
efficacy in the treatment of conduct disorders in children.
FO-14
Treatment
FO-14-1
Effect of topiramate on body mass index and
hyperphagic behaviour of morbid obese children, after
brain tumour treatment
C.E. Catsman-Berrevoets a , E.L.T. Van den Akker b , F.K.
Aarsen a , S.L.S. Drop b
a Departments of Child Neurology and 2 Child Endocrinology,
University Hospital Rotterdam/Sophia Children’s
Hospital, Rotterdam, The Netherlands
Reduced appetite and weight loss were found in clinical

408
Abstracts
trials of topiramate for epilepsy. In children treated for brain
tumour and especially craniopharyngioma hyperphagia and
therapy resistant morbid obesity frequently occurs. In a pilot
study we explored the effectiveness and the tolerability of
topiramate in children with obesity, after treatment of brain
tumour. We evaluated the response after 3, 6 and 12 months
of treatment with topiramate up to 7 mg/kg per day. Eating
behaviour, weight, effect on hypothalamic dependent
hormones and neuropsychological side effects were evaluated.
Two boys and four girls (aged 8.7–17.2 years) with a
BMI 2.75–4.4 SD above the age related were included. Five
had been treated for craniopharyngeoma, one for primitive
neuroectodermal tumor (PNET). After starting topiramate
treatment, at doses ranging from 2.7 to 3.7 mg/kg per day
parents of all five children reported a marked reduction of
the obsessive hyperphagia. On the child behavioural checklist
they all scored better on items of self-esteem. BMI at 3
months ranged from 10.15 to 3.78 (mean 21.38) in
comparison to BMI at the start of the study. After 6 months
BMI ranged from 11.44– 2 4.47 (mean 20.94) and after
12 months from 11.36 to 24.38 (mean 20.3) with BMI at
the start of the study. No important changes in blood parameters
were detected. Neuropsychological evaluation in
comparison to that at the start of the study mainly showed
a decreased performance on tests of attention and concentration,
verbal short memory, verbal sequential memory and
a reduced verbal fluency. These problems started to occur
with a dosage exceeding 4 mg/kg per day and were reversible.
Results of this small pilot study suggest that topiramate
stabilizes weight in children with morbid obesity,
which have been treated for a brain tumour, but does not
lead to important weight loss. Doses of topiramate exceeding
4 mg/kg per day mainly have adverse, but reversible
effects on performance on attention, concentration, verbal
memory and verbal fluency tasks. Topiramate seems to have
a positive effect on the obsessive, ‘craving’ behaviour of the
child in respect to food and subsequently on feelings of selfesteem.
FO-14-2
Curative effect in cerebral palsy with acupoint injection
of drug
Q.-F. Yuan, W.-C. Nan, S.-S. Yun, H.-L. Rong
Woman and Child Health Center, Shenyang, China
Objective: To investigate the effect of acupoint injection of
drug in children with cerebral palsy. Sixty children from 40
days to 3 years were divided into two groups. Both groups
adopted physical treatment, massage and other therapy. In
the treatment group acupoint injection of drug was added,
which included cerebral and body acupoint injection, 10-
day-injection is a course. Then 10 days break, three courses
consecutively. Drugs include Lizhusaile injection and
dansen injection and vitamin B12, B1 and ATP. Injective
acupoints on head area, feeling area and equilibrium area
and so on. Clinic Curative rate in the treatment group was
94.95%, clinic curative rate in the control group is 78.4%
(P , 0:01). So comprehensive treatment is better than single
motor treatment and reduces the treatment course.
FO-14-3
Effect of cerebroprotein hydrolysate injection and
conductive education in children with cerebral palsy
D. Wu, J.-L. Tang, D.-Y. Liu, J.-X. Wu
Pediatric department, the first Hospital Affiliated to Anhui
Medical University, Hefei City, China
To find a new way to treat children with cerebral palsy.
One hundred eighty three children with cerebral palsy, aged
3 months to 5 years were divided into three groups: CHI
group, conductive education (CD) group, CHI plus CD
group and control group. They were followed-up for 5
years. CHI was intravenously injected, 5 ml/day for 1-
year-old children and 10 ml/day for children older than 1-
year-old. We defined 3 months as a course of treatment. We
regularly give a short course of CD for the children with
cerebral palsy and their parents from May 1997. Children
with cerebral palsy and their parents attended the course.
Some simply training methods of CD were introduced to the
parents – home conductors, and then the parents began to
train the cerebral palsy children at home. The results are as
follows: (1) CHI plus CD was most effective in all ways. (2)
Both CHI group and CD method group were more effective
than control group (P , 0:01). (3) CHI group and CD group
were more effective than CD group (P , 0:05).
FO-14-4
Quantum neurodynamics and biofeedback efficiency in
treating neurological disorders
J. Pop-Jordanov a , N. Pop-Jordanova b , D. Dimitrovski a ,E.
Solov’ev a , N. Markovska a
a Research Center of Energy, Informatics and Materials,
Macedonian Academy of Science and Arts, Skopje, Macedonia;
b Department of Psychophysiology, Pediatric Clinic,
Faculty of Medicine, Macedonia
As a technique for learning self-control by back-reported
biological signals, biofeedback is ultimately based on
mental-neural information flow. All standard attempts to
explain the mechanism of the subtle two-way interaction
between non-material mental agencies and neural events
were confronted with violation of the energy-matter conservation
laws of physics. One non-classical hypothesis was
defined by Eccles (Nobelist for Physiology and Medicine) in
his paper with a provocative title: ‘do mental events cause
neural events analogously to the probability fields of quantum
mechanics?’ In this paper, the consecutive steps of
biofeedback signal flow are investigated, in the light of
the fundamental Eccles hypotheses. An advanced quantum

Abstracts 409
mechanical modeling is performed, to calculate the probabilities
for quantum transitions within neural molecules in
cortical electric field. The results indicated the brain-wave
frequency as a physical quantity associated with perception
and memory processing. In addition, limiting the probability
of transitions as to maximize the information entropy, the
relevant number of molecules is calculated for the
frequency of electric fields the cytoplasm is exposed to.
Consequently, some basic rationales for biofeedback efficiency
in treating neurological disorders, like seizure, attention
deficit, as well as neuromuscular computer related ones,
are deduced and discussed. In particular, the effects of brainwave
frequency ‘filtering’ by neurofeedback are considered.
FO-14-5
Randomized treatment study of inosiplex versus
combined inosiplex and intraventricular alpha
interferon in subacute sclerosing panencephalitis (SSPE)
G.G. Gascon, B.A. Anlar, M. Fojas, V. Udani
The International Consortium on SSPE, and Devol E Brown
University, Providence, RI, USA: Haceteppe University,
Ankara, Turkey; Makati Medical Center, Manila, Philippines;
Hinduja National Hospital and Research Center,
Bombay, India, King Faisal Specialist Hospital and
Research Centre, Riyadh, Saudi Arabia
Methods: One hundred and twenty-one patients, mainly
from Ankara, Manila and Bombay, who met diagnostic
criteria for SSPE were randomized into oral inosiplex
alone (Group A) and combined oral inosiplex and intraventricular
alpha interferon (Group B), delivered through an
Ommaya reservoir. Inclusion criteria were Stage II SSPE
or better. The Stage, Neurological Disability Index (NDI),
and Brief Assessment Exam (BAE) were used for clinical
rating of neurological status. The principal laboratory value
followed was the CSF IgG Synthesis Index (Tourtellotte).
Dose of inosiplex (Isoprinosine, Newport Pharmaceuticals,
Dublin) was 100 mg/kg per day in divided doses, for 6
months. Alpha interferon 2b (Intron A, Schering Plough,
NJ, USA) started with 100 000 units per meter squared
and escalated to 1 000 000 units per meter squared over 5
inpatient days, then 1 000 000 units/meter squared twice a
week for 6 months. Baseline and follow-up data flow sheets
were filled at 3, 6, 12, 18, 24 and 30 weeks, and at longer
intervals till at least 2 years. Results: After excluding 35
cases whose data sheets were not received and another 18
disqualified for various reasons, mainly protocol noncompliance,
68 cases remained for analysis, 40 in Group
A and 28 in Group B). Survival analysis, carried out so
far through 30 weeks, showed five observed deaths during
treatment in Group A and one in Group B, total 6. There was
82% survival at 30 weeks in Group A, 90% in Group B. The
Log-Rank test between Groups A and B, showed a chisquare
of 1.0134, with P ¼ 0:31. Conclusion: No evidence
of difference in survival at 30 weeks between treatment with
inosiplex alone, versus combined inosiplex-alpha interferon.
Commentary: Survival analysis will be carried out
further to 2 years post-institution of treatment. Morbidity
over time will be analyzed similarly for NDI, BAE and
Staging. Toxicity and complications will be analysed.
FO-14-6
Population pharmacokinetic model of valproate in
children with epilepsy and prediction of valproate serum
concentrations
D.-C. Jiang, L. Wang
Department of pediatrics of Peking University 1st Hospital,
Beijing, China
Objective: Using therapeutic drug monitoring data of
valproate serum concentrations to set up the population
pharmacokinetic model of valproate for pediatric patients
with epilepsy in China and to predict serum concentrations
for new patients by Bayesian approach. Method: We
collected valproate serum concentrations of 50 pediatric
patients with epilepsy who were taking valproate in monotherapy
regimens, and had no hepatic or renal disease. They
were divided into two groups (n ¼ 25). Each patient had at
least two steady state concentration points. This study used
the NPEM2 software to estimate valproate population pharmacokinetic
parameter values and distributions in a first
group of patients. On a second group, we used these parameters
to predict valproate blood concentrations with Bayesian
approach by the USC*PACK PC Program. Accuracy
and precision were assessed by mean prediction error (ME)
and mean squared prediction error (MSE). Results: We
successfully set up the population pharmacokinetic model
of valproate. Estimated population parameter values
(mean ^ SD) were Kel, 0.0868 ^ 0.0507 h 21 ; Ka,
0.143 ^ 0.120 h 21 ; and Vs 0.0419 ^ 0.025 L/kg.
ME ¼ 22.18; MSE ¼ 16.82. Using these parameters, we
can accurately predict valproate blood levels in new patients
with epilepsy by Bayesian approach. Conclusion: The adoption
of the previously mentioned population pharmacokinetic
model of valproate and Bayesian approach can
accurately predict steady state concentrations. It must be
essential for a better use of valproate in clinical practice.
FO-15
Headache/Vasculopathy
FO-15-1
Idiopathic intracranial hypertension in childhood
D.I. Zafeiriou a , C. Basiakos b , E. Maratou a , E. Vargiami a ,G.
Katzos a , N. Gombakis a , E. Kontopoulos a
a 1st Department of Pediatrics, Aristotle University,
‘Hippokratio’ Hospital, Thessaloniki, Greece; b Department
of Ophthalmology, ‘Hippokratio’ Hospital, Thessaloniki,
Greece

410
Abstracts
Idiopathic intracranial hypertension (IIH) is an unusual,
but important cause of headache, which can result in loss of
vision. In a partly retrospective partly prospective study, the
etiology, the presenting symptoms and the clinical course of
24 patients with IIH (ten females, 14 males; age range 4–16
years) who were admitted to our Department between 1991
and 2001. Predisposing factors included acute otitis media
(N ¼ 7 children), upper airway infection (N ¼ 3), obesity
(N ¼ 2), frontal sinusitis (N ¼ 1), previous use of corticosteroids
(N ¼ 1), growth hormone use (N ¼ 1), peptic ulcer
(N ¼ 1), systemic hypertension (N ¼ 1), marfanoid habitus
with mitral valve prolapse (N ¼ 1) and allergy (N ¼ 1),
while in five patients no association could be found. Most
frequent signs at presentation included headache (91.7%),
vomiting (37.5%), nausea (29.2%), vision blurring (27.5%),
abducens nerve palsy (20.8%), diplopia (16.6%) and photophobia
(12.5%). Nineteen patients (79.2%) presented with
the classical clinical picture with headache, papilledema and
raised CSF pressure. Two patients had increased CSF pressure
without papilledema and three patients had papilledema
without increased CSF pressure. In 15 patients, IIH
resolved with the use of acetazolamide, in two patients with
corticosteroids, in four patients with acetazolamide and
corticosteroids and in two patients with acetazolamide and
furosemide. Only one patient was treated with repeated
lumbar punctures, while in four of them, IIH relapsed
(range 2–4 relapses). Other ophthalmologic findings
included decreased visual acuity (6/24), abnormal visual
field examination (4/20) and abnormal visual evoked potential
testing (3/15); however, only one patient continues to
have a mild decrease of his visual acuity to date (follow-up
range 6 months–4 years). In none of the patients was either a
lumboperitoneal shunting or an optic nerve sheath fenestration
required. From our series, we conclude that acetazolamide
alone or in combination seems to be the treatment of
first choice for most patients with IIH, since it is associated
with a relative low risk (4.2%) for long-term visual loss.
However, careful and frequent ophthalmologic follow-up,
as well as prospective multicenter randomized studies, is
needed in order to establish evidence-based treatment protocols
associated with minimal long term residuals.
FO-15-2
Changes in the visual crowding effect as a function of age
E. Vandenbussche a , G. Van Hamme a , T. Reinehr a ,H.
Maes a , L. Lagae b
a Laboratory of Neuropsychology K.U. Leuven, Campus
Gasthuisberg, Leuven, Belgium; b Department of Neuropediatrics,
U.Z. Gasthuisberg, Leuven, Belgium
Introduction: Visual crowding is a form of contour interaction
due to spatial lateral masking. Originally, Flom et al.
(1963) used Landolt-C and four surrounding bars to quantify
thistypeofcontourinteraction.Theyvariedtheangularsizeof
a single black four-position Landolt-C in a larger white field,
so that the C was monocularly recognised in less than 100% of
the time. Although the literature mentions that crowding
decreased by age, no data are yet available. Aim of the study:
To assess the visual crowding effect in binocular vision, in
children of 60–146 month of age. Method: We used the procedureofFlom
withaLandolt-Cat aspecific angularsize,sothat
it was recognised in 70% of the time. The Landolt-C was
presented 1 sec at maximal contrast. The distance of the four
surrounding bars to the Landolt-C was varied in 5 steps (0.1,
0.2, 0.4, 0.8, 1.6 times the size of the Landolt-C). During the
crowding assessment the refraction of both eyes was optimally
corrected Results: (1) The crowding effect is maximal
at a distance 0.4 times the size of the Landolt-C. In children
younger than 72 months there is even a tendency to reach the
maximal crowding effect at a smaller distance. (2) The crowdingeffect
decreases gradually with age. (3) The distance range
withinwhichthecrowdingeffectoccurred,decreasedsymmetrically
around the distance with optimal crowding.
FO-15-3
A clinical study on spontaneous intracranial
hemorrhage in children
J.-L. Cai a , K.-R. Bao a , J.-M. Yu b
a Department of Pediatrics, b Department of radiology, The
Xinhua Hospital of Shanghai Second Medical University,
China
Objective: To study the causes, clinical manifestations,
neuroimaging features and prognosis in children with spontaneous
intracranial hemorrhage. To seek for the methods to
reduce the morbidity, mortality and sequelae. Methods: A
retrospective study was performed on 49 cases with spontaneous
intracranial hemorrhage confirmed by clinical manifestation
combined with neuroimaging features and/or
cerebrospinal fluid examination. Results: Delayed vitamin
Kdeficiency (36.7%) and cerebral vascular malformation
(22.4%) were the common causes of spontaneous intracranial
hemorrhage in children. No risk factors were found in
22.4% cases. The main clinical manifestations were headache
and/or vomiting, seizure and disturbance of consciousness.
Hemorrhages in cerebral parenchyma were commonly
seen in children with spontaneous intracranial hemorrhages
and had relatively better prognosis. Conclusion: Prevention
of delayed vitamin K deficiency, hemorrhage relapse and
ischemic damage of cerebral vascular malformation can
effectively reduce the morbidity, mortality and sequelae of
spontaneous intracranial hemorrhage in children.
FO-15-4
Stroke: outcome, risk factors and aetiology in Pakistani
children
Z. Habib, S. Ibrahim, S. Mithani, B.S. Hasan
Department of Pediatrics, Aga Khan University Hospital,
Karachi, Pakistan

Abstracts 411
Objectives: This is the first descriptive study that addresses
factors for stroke outcome, clinical features and etiology in
Pakistani children. Aims of the study were to (a) describe age
groups, gender and selected clinical variables in ischemic (I)
and hemmorhagic (H) stroke; (b) classify causes of strokes;
and (c) investigate poor prognostic indicators for stroke
mortality within hospital and morbidity (length of hospital
stay). Methods: Data from 138 children (ages 2 days–192
months; 92 (67%) males, 46 (33%) females) was gleaned
from chart reviews of the past ten years from the pediatric
department of the Aga Khan University Hospital. Descriptive
statistics, chi-square, odds ratios, 95% confidence intervals
and multiple logistic regression analyses were used.
Results and conclusions: The mortality was higher in hemorrhagic
(64%) versus ischemic (36%) strokes at P ¼ 0:002.
Overall, the percentage of strokes (I 1 H) was higher in the
infant age group (40%). The percentage of (I) strokes was
higher in the neonate (83%), preschool (74%) and toddler age
groups (73%). The occurrence of seizure activity was higher
in (I) stroke, odds ratio 2.45, P ¼ 0:01. Anemia occurred
with higher frequency in (H) strokes with odds ratios of
5.3, P ¼ 0:000. Presence of seizures dictated morbidity
(bivariate analysis). However, morbidity was not affected
by age at onset of stroke, gender, level of consciousness,
seizure or stroke type at the multivariate level of analysis.
The odds of the child dying from hemmorhagic stroke was
three times higher than ischemic stroke. Plausible pathophysiological
mechanism in the etiology of both (I) and
(H) strokes were presented.
FO-15-5
Visual failure without headache in paediatric ‘Benign’
intracranial hypertension
M.J. Lim, A. Penn, D. Calver, J.P. Lin
Paediatric Neurology and Ophthalmology Department,
Guy’s Hospital, London, UK
Background and objective: We have recently described a
small group of children (n ¼ 7) with benign intracranial
hypertension (BIH) with no headaches, presenting with
focal neurological signs, visual problems and an increased
risk of visual failure. We have continued to recruit more
patients to further assess the differences between the headache
and non-headache groups in BIH. Results: (Table 1)):
A significant proportion present without headaches (31%),
within the paediatric BIH group, In our current bigger
sample size, the atypical presentation group continues to
exhibit more visual abnormalities (blurred vision, diplopia,
visual field defects, larger blind spots and poor visual
acuity) and focal neurological signs (6th and 7th nerve
palsy) when compared to the headache group. Four children
in the non-headache group have marked visual impairment
at follow up. The recognised risk factors for BIH (e.g.
weight) are also less commonly seen within the non-headache
group. Conclusion: These children without headaches
represent a subgroup that have different characteristics to
the headache group and are at risk of visual failure, warranting
early recognition, aggressive management and very
close follow up.
FO-15-6
Confusional migraine: revisited
D. Ghosh, E. Varley, K. Busch, A.D. Rothner
Division of Pediatric Neurology and Otolaryngology and
Communication Disorders, The Cleveland Clinic Foundation,
Cleveland, OH, USA
Confusion is defined as disorientation with or without
altered sensorium. It is the cardinal symptom in acute
confusional migraine (ACM). Our experience and review
of the literature suggest that: (a) confusion is seen in
several migraine variants; and (b) may represent either a
receptive or expressive aphasia. The charts of 20 patients
with ‘confusional’ migraine were reviewed. Twelve were
males. Ages were 5–20 years (14.25). Six had ACM, five
basilar artery migraine, three hemiplegic migraine, three
trauma triggered migraine, two ‘complicated’ migraine
and 1 migraine with aura. One to four episodes per patient
occurred lasting 20 min to 36 h (6.5 h). All had throbbing
headache. Eleven had aphasia. Two were examined during
the attack. One had an expressive aphasia (dysfluent speech
with preserved comprehension), the other a receptive aphasia
(with reading, writing and naming difficulties). EEG in
both showed focal slowing over the dominant cortex. Focal
slowing was seen in six of 11 cases. Interictal CT and/or
MRI were normal. In one child the MRI showed middle
cerebral artery spasm and cortical T2-hyper-intensity over
the dominant cortex. In no patient was an underlying etiol-
Table 1
Headache group (n ¼ 24) Non-headache group (n ¼ 11)
Age (95% CI) 9.8 (^3.3) 7.6 (^3.4)
Sex M:F 7:5 4:7
Associated risk factors (%) 62 27
Visual failure (acuity worse than 6/36 in either eye) (%) 8 36
Blind spot affected (%) 5 (n ¼ 20) 25 (n ¼ 8)
Focal neurological signs (%) 12 45

412
Abstracts
ogy found. In conclusion, confusion can be a prominent
feature of the different migraine variants. Awareness of this
may help avoid invasive investigations. Aphasia as a
component of ACM is common. A detailed language
evaluation during the attack may be useful. Reversible
vasospasm of intracranial vessels causing ischemia contributes
to the mechanism for confusion and aphasia in
migraine.
FREE PAPERS-Poster Presentation
FP-A
Developmental/Basic Neuroscience
FP-A-001
Significance of Pavlov’s work on the physiology of
neuropsychic behaviour of man
K.B. Kulkarni
Sanjivani Nursing Home, Gwalior (M.P.), India
Pavlov’s epoch making observations and animal experiments
provide the edifice for understanding neonatology,
developmental pediatrics, psychology and overall higher
nervous activity. His concept of integral nature of organism,
nervism, unity of organism with social and ecological environs,
the complex toxicosis involving CNS excitation and
inhibition, his idea of multitudinous reflexes as basic reserve
of higher nervous activity, the concept of sleep and internal
inhibition as part of the same process of excitation are only a
few examples. The Pavlovian thought underscores not just
conditioned reflexes but also the instinctive reflexes of
dynamic stereotypes, conditioned switching and reflex
chains. Ukhstomsky’s dominance focus or the dominant
and Pavlov’s conditioned reflex is the elementary functional
unit of central organisation of behaviour. In fact, many
mechanisms of mental activity are a part of the second
and third signalling system of conditioned reflex activity:
even speech, writing and developing communication skills
in the era of information technology. According to him, by
forming numerous two ways connections – forward and
backward – we form mental images of the world around
and likewise, respond reflexively or voluntarily. His observations
on young birds and mammals provide the answer
whether our children should be exposed to life’s dangers and
difficulties. He therefore immensely valued the process of
‘imprinting’. Pavlov stressed on ‘reinforcement’ mechanism
on which elaboration, existence, discontinuation and
extinction of conditioned reflex depends. He took pains to
differentiate between a biologically significant stimulus
from useless one, both in the laboratory and in real life.
Without Pavlovian theory of emotions it would not have
been possible to extricate psychology so early from physiology.
FP-A-002
Aminophylline aggravates long-term morphological and
cognitive damages in status epilepticus in immature rats
L.-T. Huang a , S.N. Yang a , M.-C. Lai b , P.-L. Hung a
a Department of Pediatrics, Chang Gung Memorial Hospital,
Kaohsiung, Chinese Taipei; b Department of Pediatrics,
Chi Mei Foundation Hospital, Chinese Taipei
The aim of this work was to investigate whether aminophylline,
an adenosine receptor antagonist used usually as a
treatment for premature apnea, had synergistic effects on
status epilepticus in the developing brain. On the postnatal
day 14 (P14), four groups of rats intraperitoneally received
saline, aminophylline, lithium-pilocarpine (Li-PC), and Li-
PC plus aminophylline, respectively. Subsequently, Morris
water maze task was performed at P80. The brains were then
analyzed with cresyl violet stain for histological lesions and
evaluated for mossy fiber sprouting with the Timm stain. No
seizures were elicited in the saline-treated or aminophyllinetreated
rats. Both the Li-PC-treated and aminophylline plus
Li-PC-treated rats exhibited seizures and there was no
significant difference in mortality between the two groups.
More interestingly, as in adulthood (P80), aminophylline
aggravated the spatial deficits and histological damages in
hippocampus seen in Li-PC-treated rats. In summary, this
present study suggests that the use of adenosine receptor
antagonists, such as aminophylline, exacerbates seizureinduced
damage in the developing brain.
FP-A-003
Immunohistological evaluation of the diencephalon and
basal ganglia in alobar holoprosencephaly
M. Hayashi a , K. Hamano b , S. Araki c , T. Kohji a ,S.
Kumada b , M. Itoh a , K. Tamagawa c , J. Satoh a ,Y.
Morimatsu a
a Department of Clinical Neuropathology, Tokyo Metropolitan
Institute for Neuroscience, Tokyo, Japan; b Tokyo
Metropolitan Medical Center for SIMDS, Tokyo, Japan;
c Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
Holoprosencephaly (HPE) is caused by the impaired cleavage
of the embryonic prosencephalon and in the severest
type, alobar HPE, the bilateral diencephalon and basal ganglia
are usually fused and tend to intermingle with the upper
brainstem. Therefore, their detailed neuropathological
features have not fully been investigated, although disturbed
regulation in body temperature, electrolyte balance and/or
muscle tonus is frequently observed in HPE patients. We
immunohistologically examined the expression of hypothalamic
markers such as vasopressin and orexin A, neurotransmitters,
neuropeptides and calcium binding proteins in six
female autopsy cases of alobar HPE, in addition to six agematched
controls, who showed preserved immunoreactivities
for these markers from the neonatal period. The HPE

Abstracts 413
cases consisted of two preterm babies, two infants and two
cases over 1 year in age. The neurons immunoreactive for
either vasopressin and/or orexin A were found on both sides
of the diencephalic midline cleft in five cases, with the
exception of the preterm infant case of 35 weeks of gestational
age. The tyrosine hydroxylase-immunoreactive fibers
and neurons were observed in the mixture of diencephalon
and basal ganglia and the mesencephalic substantia nigralike
structure in all the HPE cases. The parvalbumin-immunoreactive
structures mimicking the red nucleus was identified
in one preterm infant case and two cases over 1 year,
while the immunoreactivity for methionine-enkephalin was
found only in cases over 1 year in the mixture of diencephalon
and basal ganglia. These data suggest that the
jumbled diencephalon and basal ganglia showed some
degree of functional development in alobar HPE, and
incomplete or delayed expression of the markers examined
here may partly reflect the diencephalic and motor disturbances
in HPE.
FP-A-004
Developmental changes in EEG multifractal
characteristics of normal and abnormal brain
E.L. Wasserman a , R.I. Polonnikov b , N.K. Kartashev b
a V.M. Bekhterev Psychoneurological Research Institute,
Saint-Petersburg, Russia;
b Institute of Informatics and
Automation of RAS, Russia
One hundred-ten males and females (6.5–19.5 years old)
were investigated (48 healthy persons, 51 ones with hemiplegic
cerebral palsy and 11 ones with similar acquired
cerebral defect). Monopolar 12-channel EEG were
processed and then regarded as multifractal processes.
Power spectrums of 30 successive 1-s fragments of background
activity in each channel were approximated by k·f b
model ( f-frequency in 2–30 Hz band, k and b-model parameters).
Model parameters were averaged in all 30 fragments
and 12 channels and then analysed. Stable
correlations (accordant to Spearman, r s , P , 0:0001) of
both parameters with the age of person were discovered:
k (r s ¼ 20:63) and b (r s ¼ 20:58). These dependences
were approximated well by exponential functions and
observed in healthy persons (r s ¼ 20:55, r s ¼ 20:56) as
well as in patients (r s ¼ 20:67, r s ¼ 20:64). It was
concluded: (1) parameter b (which is an informational
characteristic of EEG as multifractal) and parameter k
(which is a power characteristic of EEG) demonstrate regular
changes with age reflecting the process of cerebral
maturation; and (2) this regularity takes place not only in
the cases of normal development of brain but also in the
cases of its abnormal development caused by early or later
injury.
FP-A-005
The expression of PRL, GFAP and Fos in the cerebral
cortex and amygdala of acute heat stress model of
seizure in weanling rats
H. Ni, Q.-X. Shui
Department of Neurology, Children’s Hospital, College of
Medicine, Zhejiang University, Hangzhou, China
Many investigations have suggested the possibility that
febrile convulsions (FC) in early childhood are an etiologic
factor in human temporal lobe epilepsy. In order to explore
the morphological basis of pathogenesis in FC, the expression
of prolactin, glial fibrillary acidic protein (PRL, GFAP)
and Fos immunoreactivity in cerebral cortex and amygdala
of weanling rats was examined. We studied this by using the
warm-water-immersion model and immunohistochemistry
method. Thirty rats were randomly divided into two groups:
normal control group (n ¼ 6) and hyperthermia-treated
group (n ¼ 14). The latter was further divided into FC
(n ¼ 6, score reaching Grade 5, two rats were abandoned
for only reaching Grade 2) and hyperthemic (non-convulsion)
groups according to the reaction to hyperthemia treatment.
Our results demonstrated that there was a large
number of positive cells with PRL, GFAP and Fos immunoreaction
in both cerebral cortex and amygdala in
hyperthermia-treated group (P , 0:01). Moreover, the
expression of GFAP and Fos were markedly high in FC
group than in hyperthemic (non-convulsion) group, meanwhile
the expression of PRL did not change significantly
between the hyperthemic and FC group (P . 0:05). These
results indicated that the increased prolactin immunoreactivity
is involved in the progress of heat stress activity, but
may have no significant relationship with seizure activity.
However, GFAP and Fos are not only involved in pathogenesis
of heat stress progress, but may also associate with
seizure activity.
FP-A-006
Different expression of c-Fos and prolactin in neonatal
rat with hypoxic-ischemic encephalopathy
H. Ni a , Q.-X. Shui a , Y.-C. Wang b , C.-C. Shi b
a Department of Neurology, Children’s Hospital, College of
Medicine, Zhejiang University, Hangzhou, China; b The
Peoples’ Hospital of Jinhua, Zhejiang Provence, China
Recent studies have showed that prolactin is not only
synthesized in the pituitary gland, but also within the central
nervous system to control a variety of behaviors. The
expression of c-fos like protein is regarded as a marker for
neuronal activity following noxious stimulation in the rat.
The present study was to investigate the relationship
between c-fos and prolactin expressions in neonatal rat
with HIE. Twenty 7-day-old SD rats were randomized
divided into two groups: normal control group (n ¼ 8, with-

414
Abstracts
out hypoxic-ischemic treatment) and hypoxic-ischemic
treatment group (HIE, n ¼ 12). After ligation of the left
common carotid artery, the rats of HIE group were exposed
to 8% oxygen-92% nitrogen at 378C for 3 h. At the end of
the hypoxic period, they were removed from the container
and placed in room air for 60 min before being decapitated.
Immunohistochemistry was used to detect the expression of
PRL and Fos. Compared with controls, the number of Fos
positive neurons elevated significantly in hypothalamus,
supramammillary nucleus, suprachiasmatic nucleus, thalamic
pavaventricular nucleus, the thalamic midline nucleus,
and amygdala (P , 0:01). On the other hand, cortical and
hippocampal PRL immunoreactive cells were significantly
elevated compared with the control (P , 0:01). The
increased expression of Fos and PRL might closely related
to hypoxic-ischemic processes. The different expression
region of Fos and PRL suggest differential role of them in
HIE.
FP-A-007
Ontogenic expression of colony stimulating factor-1
receptor on neurons
S.-Q. Qu, Y.-Q. Wang, L. Zuo, X.-H. Hu
Navy general hospital, Beijing, China
The mice with different ages, from embryonic day 14, 18
(E14, E18) to newborn and postnatal 7, 14, 21, 28 (P7, P14,
P21, P28) were used in our experiment. Immunochemical
staining and immunoblotting were used to examine the
expression of CSF-1 receptor (CSF-1R) on neurons in
CNS. The results showed that the expression of CSF-1R
could be detected on brain neurons of mice from fetal age
14 days and increased gradually along with the growing of
fetal mice and reached the peak value in newborn mice.
The expression decreased gradually after birth and reached
plateau 4 weeks later showed by Western blot. The
numbers of CSF-1R positive neurons also showed the
same pattern as western blot in immunochemical study. It
was indicated that there might be a close relationship
between the level of CSF-1R expression and the ontogeny
and development of neurons in CNS in mice. Ya-qi
Wang’s work showed that expression of CSF-1R were
only observed in a few of neurons in the brainstem in
normal state, but up-regulated not only at the vicinity of
the lesion but also in distant areas after the ischemic lesion.
It was suggested that neurons have the potency of expressing
CSF-1R during the development, which might be
down-regulated somehow after birth and up-regulated in
the lesion’s conditions in our experiment. We supposed
that CSF-1R might play an important role in the development
course of neural system in mice and was one of the
factors in response to the stimulation.
FP-A-008
Effect of nerve growth factor on neuronal injury in
strychnine-nitrate induced seizure rats
M.-Y. Hu, Z.-M. Ren, M. Jiang
Department of pediatric Second Affiliated Hospital of
Harbin Medical University Harbin, China
Objective: To probe whether exogenous nerve growth
factor (NGF) might have an active effect on the encephalo-neuronal
apoptosis induced by acute seizures. Methods:
With the model of strychnine-nitrate-induced epilepsy,
the morphological changes of brain cells were observed
under the photomicroscrope with hematoxylin and eosin
(HE) dying as well as the amount of apoptotic neurons
and the effect of NGF on that were detected by the method
of immuno-histochemistry with bax and bc1-2 protein.
Result: Apoptotic neurons in CA1 of the hippocampus and
the cortex were seen 24 h after epilepsy was induced, which
increased significantly at 48 h and reached the peak at 72 h,
then decreased toward 7 days to the valley. There are far
fewer apoptotic neurons in brain cells of the rats injected
with NGF into their abdomen than those without injection
killed at the same time expectedly (P , 0:05). Conclusions:
Exogenous NGF could inhibit the encephalo-neuronal apoptosis
induced by acute seizures, which means that NGF may
have a neuroprotective effect against brain damage owing to
epilepsy.
FP-A-009
Levels of CSF neurotrophic factors in patients with Rett
syndrome and children with autism
R.S. Riikonen, R. Vanhala, U. Turpeinen
Children’s Hospital, University of Kuopio, Kuopio, Finland
Autism and Rett syndrome (RS) are both developmental
disorders of unknown origin. Differentiation of RS from
infantile autism in the very early stages is not always
easy. The aim of the present study was to determine whether
CSF NGF or CSF insulin-like growth factor-I (IGF-I). (1)
Distinguish patients with RS from autism; and (2) could
have a role in the pathogenesis of the syndromes. Method:
We measured CSF NGF levels with two-site ELISA method
and CSF IGF-1 levels with radioimmunoassay (RIA).
Results: Patients with RS had low to negligible levels of
CSF NGF. Patients with autism had normal CSF NGF
levels. CSF IGF-I of patients with RS did not differ from
controls. Patients with autism had low levels of CSF IGF-I
compared to controls. NGF acts specifically on cholinergic
neurons of the basal forebrain. In RS the frontal cortex is
more severely affected than the other cortical areas. IGF-1 is
important for cerebellar development. In autism, many
studies show hippocampal or cerebellar pathology. Conclusions:
Our findings are in agreement with the different
morphological and neurochemical findings (brain growth,

Abstracts 415
affected brain areas, neurotransmitter metabolism) in the
two syndromes.
FP-A-010
Monoamines in brain tissues of newborn rabbits with
cerebral palsy
X.-J. Li, Y.-Q. Li, Z.-M. Jiang, L. Li
Prevention, Treatment and Rehabilitation Center for Child
Cerebral Palsy in Heilongjiang Province, Jiamusi, Heilongjiang,
China
To study the role of monoamines (DA, 5-hydroxytryplamine,
5-HT, NE) in pathogenesis of CP by measuring the
levels of dopamine (DA), 5-HT, nonepinephrine (NE) in CP
rabbit models. Twenty newborn rabbits (2–3 days) were
randomly divided into two groups: the control group (C
n ¼ 10), the model group (M n ¼ 10). Rabbits in group M
were administered intraperitoneal bilirubin 100 mg/kg, total
dose was 300 mg/kg. Rabbits in group C were administered
saline as the same dose as those in group M. Animals were
killed after being fed naturally for 45 days, hippocampus,
cerebral cortex, basal ganglia and brainstem were separated
respectively. Each region was divided into two parts: one
part was fixed and examined with light microscope. The
levels of monoamines in the other part were determined.
The levels of NE, 5-HT in basal ganglia and NE in brainstem
in group M were significantly lower than those in
group C (P , 0:05). There was positive correlation between
DA, 5-HT in basal ganglia and NE in brainstem
(r ¼ 0:8979, r ¼ 0:7112, P , 0:05), and between 5-HT
and DA in basal ganglia (r ¼ 0:6178, P , 0:05). The results
suggest that bilirubin may be responsible for the decreased
DA, 5-HT, NE in brain tissues. DA, 5-HT may participate in
the damage of basal ganglia induced by bilirubin. The levels
of NE may be related to the symptoms of CP.
FP-A-011
Pilocarpine-induced status epilepticus produces
neuronal death: implications for programmed cell death
mechanisms
R.-Z. An, Y.-R. Yin, C.-J. Jin, G.-H. Li
Department of Pediatrics, College of Medicine, YanBian,
University, Yanji, China; New Era Children Hospital,
Yanji, China
Purpose: Prolonged and continuous epileptic seizures
(SE) induced by pilocarpine activate programmed cell
death mechanisms. In order to test this hypothesis, we
investigated the morphology of neuronal death (necrotic
and apoptotic) from pilocarpine-induced SE, and the
expression of proapoptotic and antiapoptotic protein bax
and bcl-2, respectively. Methods: Pilocarpine induced
status epilepticus lasting 2 h was induced in Sprague-
Dawley adult male rats, which were allowed to recover
for 24 or 72 h before perfusion-fixation. Neuronal death
was assessed by light microscopy with the haematoxylinand
-eosin stain and with in situ terminal deoxynucleotidyl
transferase dUTP nick-end labelling (TUNEL stain). Bax
and bcl-2 protein expression were examined by immunohistochemistry.
Results: Twenty-four and 72 h after 2 h
pilocarpine induced SE, neuronal death in hippocampus
(CA1 and CA3) was morphologically necrotic, but there
were TUNEL-positive and morphologically necrotic cells
in the hippocampal CA1 and CA3 regions at 72 h, but not
at 24 h, after SE. Bax protein expression was also increased
in the hippocampal CA1 and CA3 regions at 72 h after SE.
Bcl-2 protein expression was not detected. Conclusion: Our
results suggest that programmed cell death promoting
mechanisms are activated by SE in neurons that become
necrotic and could contribute necrotic, as well as apoptotic,
neuronal death.
FP-A-012
Expression of GABA A receptor a 1 subunit in rat
hippocampus following kainic acid-induced temporal
lobe epilepsy
G.-F. Lei, R.-P. Sun, Y.-L. Wang, B.-M. Li, J.-W. Wang,
R.-M. Hu, S.-H. Guo
Pediatric Department, Shandong University Qilu Hospital,
Shandong, China
Intrahippocampus injection of kainic acid (KA) in the rat
represents a widely used animal model of human TLE, the
etiology of which is controversial. This study evaluated by
immunohistochemistry for the expression of GABA A receptor
a 1 subunit (major a subunit expressed in adult brain) in
the contralateral hippocampus of rats 12, 24, 72 h, 7 and 30
days after injection of KA into right hippocampus. At early
intervals (within 24 h after KA injection) a 1 subunit expression
on hippocampus was decreased. The decrease was
followed by increase of a 1 subunit expression after 7–30
days. The results indicate that the decreased GABA A receptor
a 1 subunit expression on hippocampus at early interval
may play an important role in the induction and maintenance
of epilepsy, and the decrease in GABA A receptor a 1
subunit expression may be compensated partly by increased
expression at late interval. Thus the compensatory mechanism
is operative in epileptic hippocampus.
FP-A-013
Effect of radix salviae miltiorrhizae on inducible nitric
oxide synthase activity in rat astrocytes
F. Gao, J.-F. Lu, Q.-X. Shui, Z.-Z. Xia, B.-L. Zhou
Department of Neurology, Children’s Hospital, Zhejiang
University Medical College, Hangzhou, China
Objective: The present study was undertaken to examine
the effect of radix salviae miltiorrhizae (RSM) on the indu-

416
Abstracts
cible nitric oxide synthase (iNOS) activity induced by endotoxin
in cultured rat astrocytes. Method: We detected iNOS
activity in normal astrocytes, LPS induced astrocytes, and
RSM treated LPS induced astrocytes by Griess methods.
Results: (1) The effect of LPS induced iNOS activity expression
in astrocytes was significant among the different LPS
concentrations within the range of 0.01–50 mg/ml, the maximum
stimulation for iNOS activity was obtain with 10 mg/
ml LPS (40.00 ^ 2.52 mM). (2) The effect of different incubation
time on iNOS activity of astrocytes: the iNOS activity
of astrocytes in the control group was no significant
change from 0 to 48 h of incubation time. However, when
it was stimulated with 5 mg/ml LPS, the iNOS activity
increased to be 6.02 ^ 0.52, 6.93 ^ 0.52, 27.65 ^ 1.47,
29.20 ^ 6.09, 29.40 ^ 1.60 mM with the time of incubation
at 3, 12, 24, 36 and 48 h, respectively. The induction of
iNOS activity by endotoxin was not influenced by RSM
incubation within 12 h, otherwise RSM exhibited strong
inhibition at 24, 36 and 48 h, respectively, as compared
with that of LPS group. (3) The effect of various concentrations
of RSM on 5 mg/ml LPS induced iNOS activity: iNOS
activity attenuated gradually, with the increase of RSM
concentrations. Conclusions: It was suggested that the
iNOS of astrocytes might take part in the pathology of
bacterial infection in central nervous system, and its role
could be decreased by RSM.
FP-A-014
Gene therapy for rat hypoxic-ischemic encephalopathy
by neurotrophin-4-engineered marrow stromal cell
Y.-J. Jia, Y.-J. Yang, X.-R. Zheng, J.-B. Liu, J.-H. Song
Xiangya Hospital, Central South University, Changsha,
China
Objective: Marrow stromal cells, which have many characteristics
of stem cells, populate various non-hematopoietic
tissues including the brain. In the present study, we explored
the protocol of gene therapy for hypoxic-ischemic encephalopathy
by neurotrophin-4(NT-4)-engineered marrow stromal
cell. Methods: The recombinant pcDNA3.1(1) NT-4
plasmids were constructed and transduced into marrow stromal
cells by lipofectin. The newborn rat hypoxic-ischemic
model was successfully established, and NT-4-engineered
marrow stromal cells were transplanted into brain. Results:
The plasmids could express active NT-4 protein and mRNA
in marrow stromal cells presented by immunohistology and
in situ hybridization. Four weeks after transplantation, more
NT-4 immuno-reaction positive cells were observed in the
transplanted group compared with the control group. In addition,
significant difference in motor activity and histological
changes were detected between the two groups. Conclusion:
These results suggested that NT-4-engineered marrow stromal
cells were effective in gene therapy for hypoxicischemic
encephalopathy.
FP-A-015
Changes of nestin expression in marrow stromal cells
differentiate to neuron and islet
Y.-J. Jia, Y.-J. Yang, J.-B. Liu, X.-R. Zheng, J.-H. Song
Xiangya Hospital, Central South University, Changsha,
China
Objective: Marrow stromal cells (MSCs) exhibited multiple
traits of a stem cell population. In the present study, we
explored changes of nestin expression during marrow stromal
cells differentiating into neurons and islets. Methods:
MSCs from adult rats were induced by baicalin in serumfree
medium for 6 h, and post-induced by baicalin for neuron
or by kinetics gain factor (KGF) for islet. After inducement,
neuron and islet were evaluated by immunocytochemistry
staining. And insulin released was measured by radioimmunoassay.
The expression of nestin was measured by immunocytochemistry
and RT-PCR. Results: After induction,
MSCs expressed neuronal special proteins, such as NSE,
NF and NeuN, and islet special proteins, such as insulin
and glucagons. In addition, cumulative quantities of insulin
with 24 h and the stimulation index maintained at high level.
The expression of nestin was appeared in 6 h of differentiating
into both neuron and islets. After 24 h, the expression was
not detected. Conclusions: The results showed that nestin
may play a role in MSCs differentiate into neuron and islets.
They suggested that there may be a common path of neuron
and islets differentiation.
FP-A-016
Effects of phenytoin on primary cortical neuron cultures
Y.-J. Jia, Y. Zhou, Y.-J. Yang
Xiangya Hospital, Central South University, Changsha,
China
Objective: By observing the effects of phenytoin (PHT)
on cortical neuron, we tried to give an explanation of the
mechanism of PHT-induced neurotoxicity. Methods: After
established the method of primary rat cortical neuron
culture, neuron membranous fluidity, percentage of surviving,
LDH releasing ratio, and morphologic changes in the
various doses of PHT (2.5, 5, 12.5, 25 mg/ml and control
groups) were measured. Moreover, the conformation of
membrane proteins was calculated by circular dichroism
spectra. Results: There were no significant differences
between the control group and low-dosed groups (2 and
12.5 mg/ml). On the contrary, in the high-dosed groups
(25 and 50 mg/ml), there were significant decrease of
membranous fluidity and the percentage of neuron surviving,
and increase of LDH releasing ratio (P , 0:05). a-helix
content in the membrane proteins of control group was
48.3%. After treated by high-dosed PHT, the content
increased to 53.7 and 55.9%, respectively. Conclusions:
The results showed that the high-dosed PHT may play a
role in decrease of membranous fluidity, increase of LDH

Abstracts 417
efflux and a-helix content in the membrane proteins. They
suggested that there is a relationship between PHT-induced
neurotoxicity and changes of membranous structure.
FP-A-017
Isolation and cultivation of neural stem cells from
newborn rats
Y.-J. Jia, Y.-J. Yang, J.-H. Song, J.-B. Liu, X.-R. Zheng
Xiangya Hospital, Central South University, Changsha,
China
Objective: To study the isolation and cultivation of
neural stem cells from newborn rats. Methods: Cell culture,
indirect immunofluorescence cytochemistry and gene
transfection techniques were used. Results: The neural
stem cells isolated from newborn rats had the potential to
form clones, express neuroepithelial stem cell protein
(nestin) and differentiate into mature neurons and astrocytes.
Moreover, green fluorescent protein vector could
be efficiently transfected into this cell line. Conclusion:
The results demonstrated that our neural stem cells can
only be characterized on a critical functional basis in
terms of their undifferentiated features, capacity for selfrenewal,
proliferation, pluripotentiality, and ability to efficiently
express ex vivo gene.
FP-A-018
The dose-dependents and time-dependents in induced
brain heat shock protein 70 (HSP70) expression with
curcumin
R. Huang, F. Luo, Y.-J. Yang
Department of pediatrics, XiangYa Hospital, Central South
University, Changsha, China
Objectives: To study the dose-dependents and timedependents
in induced HSP70 expression with curcumin.
Methods: I The dose-dependents. SD rats were randomly
divided into seven groups: (1) blank group (B); (2) DMSO
group (DMSO); (3) heat shock group (HS); (4) curcumin
80 mg group (C80); (5) curcumin 40 mg group (C40); (6)
curcumin 20 mg group (C20); (7) curcumin 10 mg group
(C10); animals were killed 24 h after HS or injection
DMSO or curcumin. Brain HSP70 were tested by Western
blot analysis. II The time-dependents. SD rats were all
injected with curcumin 40 mg/kg into peritoneal. Then
randomly divided into eight groups according the killed
time, 0, 2, 4, 6, 12 and 16, 24 and 48 h. Brain HSP70
expression was detected by the Western blot analysis.
Results: The Brain HSP70 expression of HS, C40, C80
group were increased significantly, especially in C40
group (P , 0:01). HSP70 expression was increased gradually
with the time going, and the top in 16H group; then,
there was a plateau in 24 and 48 h group. Conclusions:
Brain HSP70 expression can be induced by pretreatment
with curcumin, and there are dose-dependents and timedependents.
FP-A-019
Effects of developmental lead exposure on N-methyl-daspartate
receptor mRNA expression in rat
hippocampus
Z.-W. Zhu, Z.-Y. Zhao, G.-J. Dong
The Affiliated Children’s Hospital of Zhejiang University
School of Medicine, Hangzhou, China
Objective: To investigate the effect of developmental
lead exposure on NMDA receptor mRNA expression in
rat hippocampus. Methods: We established a series of
developing rat models exposed to low level lead (drinking
water containing 0.025, 0.05 or 0.075% lead acetate), and
examined mRNA expression levels of the subunit NR2A
and NR2B in rat hippocampus with RT-PCR method.
Results: There were no differences in body weight of rat
pups between any two groups at any age (P . 0:05). The
blood lead level of Pb-exposed rats changed regularly: at
age of 14 days, it was lower than that of 7 days, and
increased to a highest level at 21 days, then returned to
lower level at 28 days. There was difference between 21
days and any other age groups (P , 0:05). The expression
levels of NR2A mRNA of 0.05 and 0.075% groups at age
of 7 and 14 days were higher than those of control group
(P , 0:05), but in 0.025% group, the expression level of
NR2A mRNA was higher than that of control group at 7
days (P , 0:05). There were no significant differences in
expression level of NR2B mRNA between any two groups
at any age (P . 0:05). Conclusions: Developmental low
level lead exposure of rats can raise the expression level
of NR2A mRNA in hippocampus. Developmental low
level lead exposure of rats does not affect the expression
level of NR2B mRNA in hippocampus.
FP-A-020
Development of the prefrontal layer IIIc pyramidal
neurons in fetuses and infants with Down’s syndrome
M. Vukšić, J. Bošnjak, A. Cepika, Z. Petanjek, I. Kostović
Croatian Institute for Brain Research, Salata 12, School of
Medicine, University of Zagreb, Zagreb, Croatia
In this study we have analysed the development of associative
layer IIIc pyramidal neurons of the prefrontal cortex
in children with DS during the late fetal and early postnatal
period. Basal dendritic tree of rapid Golgi impregnated
pyramidal neurons was quantitatively analysed (dendritic
length; number and morphology of dendritic spines) in the
postmortal brains from 3 subjects with DS (fetus 36 weeks
of gestation, infants aged 2.5 and 4.5 months) and in 5 age
matched controls. During the perinatal period there is the
most intensive dendritic growth and spinogenesis. No differ-

418
Abstracts
ences were present between DS infants and control subjects
concerning the pattern of the dendritic elongation and
segment outgrowth. Increase in spine number followed in
DS the normal curve up to third postnatal month. At 4
months decreased spine density was observed only on the
most distal terminal branches. Some morphological alterations
of the dendritic spines are present in DS subjects from
the beginning of their occurrence. It was proposed that the
basis of cognitive dysfunction in DS lies in the disordered
development of their associative cortex. These data suggest
that children with DS begin their life with morphologically
normal layer IIIc prefrontal pyramids, which are the key
cellular elements of the corticocortical connectivity. Since
the first regressive changes are not present before the fourth
postnatal month, it seems reasonable to conclude that the
application of some therapeutic procedures in the early
period of life could mitigate a cognitive decline present
later in these patients.
FP-A-021
Reelin and calretinin immunohistochemistry in
ibotenate-induced cortical dysplasia in newborn hamster
T. Takano, Y. Takeuchi
Department of Pediatrics, Shiga University of Medical
Science, Otsu, Japan
Reelin is an extracellular matrix protein synthesized and
secreted by Cajar-Retzius (CR) cells in the marginal zone,
and plays a pivotal role in cortical lamination. Calretinin, a
calcium-binding protein, has been postulated to have a
correlation with calcium-dependent events such as synaptogenesis,
neurite elongation or neuroprotective process. The
present experiment reported the model of cortical dysplasia
induced by intracerebral inoculation of ibotenate in
newborn hamsters. In order to investigate the role of reelin
and calretinin in the development of cortical dysplasia,
immunohistochemical localization of these proteins was
analyzed. Main types of cortical dysplasia detected were
microgyria (Mg), leptomeningeal glioneuronal heterotopia
(LGH) and subcortical nodular heterotopia (SNH). Reelin
immunohistochemistry showed evenly spaced CR-like
cells, mainly in the more superficial aspects of the marginal
zone. Focal increases of reelin immunoreactive CR-like
cells were observed in the lesions of Mg and LGH. In the
area of SNH, reelin immunoreactivity was not detected.
Calretinin immunoreactivity was mainly observed in the
following areas; small number of horizontally oriented
cells in the marginal zone, intensively stained cells and
fibers in the subplate, and large number of neurons in the
piriform cortex. Focal accumulation of calretinin positive
fibers and cells was observed in the lesions of Mg and LGH.
Small number of calretinin immunoreactive cells were also
detected in SNH. Overexpression of reelin or calretinin in
the areas of MG, LGH and SNH was thought to be related to
the abnormal tangential neuronal migration.
FP-A-022
Study the protection role of TGF-b1 in acute ischaemia
brain damage of mice
B. Xue, F. Li, X.-Z. Fang
Department of Pediatrics, Sheng Li Hospital of Sheng Li
Petroleum Administrative Bureau, Dong Yin, China
Objective: To investigate the protection role of TGF-b1in
acute ischaemia brain damage. Method: One hundred and
eighty mice were randomly divided into control group,
model group, TGF-b1 prevention and treatment group, cytidine
5 0 -diphosphocholine (CDPC) prevention and treatment
group. Mice in the prevention group were injected TGF-b1
25 mg/20 g or CDPC 62.5 mg/20 g into abdominal cavity an
hour before acute ischaemia brain damage model was established,
while mice in the treatment group were injected the
same medicines as the prevention group an hour after model
was established. Ten mice of each group were killed after 24
and 48 h, the brain tissues were collected to observe the
changes of pathology using light microscope and electric
microscope. Nerve cells of hippocampus CA1 area were
counted. Expression of TGF-b1 mRNA in neuron and glial
cells were detected by in situ hybridization. The death condition
of the rest mice was observed. Results: TGF-b1 mRNA
was not expressed in control group. It was completely
expressed and most III degree in model group. Although
there was also expressed in TGF-b1 prevention group and
treatment group, the degree was lower than those in model
group. TGF-b1 mRNA was completely expressed in CDPC
prevention group and treatment group. But the degree was
lower than those in model group and more than those in TGFb1group.
There were more hippocampus CA1 nerve cells in
TGF-b1 prevention group and treatment group than in model
group and CDPC group. The change of morphology was
lower than in model group and CDPC prevention and treatment
group. The survive time of mice was prolonged in TGFb1
prevention group and treatment group. Conclusion: TGFb1
can down-regulation TGF-b1 mRNA expression, lighten
the extent of damage in hippocampus CA1 area, prolong the
survive time of mice, play a protection role in acute ischaemia
brain damage. The effect of TGF-b1 prevention is superior
to the effect of treatment.
FP-A-023
Building a mouse model hallmarking the congenital
human cytomegalovirus infection in central nervous
system
J.-L. Tang a , M.-L. Wang b , J.-J. Qiu a ,D.Wu a ,W.Hu b
a Pediatric Department, The First Affiliated Hospital, Anhui
Medical University, Hefei, China;
b The Microbiology
Teaching and Researching Section, Anhui Medical University,
Hefei, China
To investigate the mechanisms that human cytomegalo-

Abstracts 419
virus (HCMV) can vertically transmit from the placenta of
mice to infect their offspring in the CNS and cause congenital
anomalies, in addition provide basic research for
preparing HCMV vaccine, we have developed a new type
of mouse model of HCMV congenital CNS infection. Pure
strain mice were propagated after being infected with
HCMV, then we looked for evidences that CNS of their
filial generations were infected by HCMV. The experiment
shows that in the infection groups the mortality of fetal mice
and the fatality of neonatal mice in 1 week are higher than
that of in the control groups (P , 0:01). At the same time
we research the CNS of fetus’s mice whose mother were
infected by HCMV, the results are as fellows: (1) the virus
was successfully isolated from their cerebral cortex. (2) The
signal of HCMV hybridization print was found in their
nervous cell through in situ hybridization. (3) Especially
human herpes virus-like particles and inclusion bodies in
the cytoplasm of nerve cell were found in the tissue of
their brain under the electronic microscopy. This new type
of mouse model of HCMV inherent CNS infection will help
us to prepare HCMV vaccine and research HCMV congenital
infection in CNS.
FP-A-024
Cerebrolysin protects cortical neurons from
extracorporal HSV-1 infection
J.-L. Tang, M.-L. Wang, D. Wu, Y. Hu
Pediatric department, the First hospital affiliated to Anhui
medical University, Hefei City, China
The effects of cerebrolysin on isolated mice cortical
neurons in a herpes simplex virus HSV-1 infection model
has been examined. First prepared culture solution of
newborn mouse’s cerebral cortex neurons (CCNs) and
cultured them, then 0.4 ml cerebrolysin for each aperture
(provided by Austrian Ebewe Medicine Factory) was added
to CCNs culture solution respectively on the 2nd, 4th, 6th
and 10th day. At the same time one group of normal culture
solution of newborn mouse’s cerebral cortex neurons was
utilized as control group. On the 12th day, each aperture was
received 3.0 log TCID50 suspension of HSV-1. The results
are as follow: (1) the culture groups that receiving cerebrolysin
on the 4th or 6th day were found to suffer lower CCNs
pathological damages ratio (25–40%) after 24 h later, while
other culture groups receiving cerebrolysin on the 2nd, 8th
or 10th day suffered higher CCN’s pathological damage
ratio (75–90%). (2) In five kinds of culture solution with
different cerebrolysin concentration showed no obviously
pathological damage, which indicated that cerebrolysincan
protect CCNs from the harm of HSV-1. This conformed that
adding cerebrolysin to CCN culture on the 4th or 6th day
could intensify CCN’s HSV-1 resistance. But why the
culture groups that receiving cerebrolysin on the 2nd day
shows no enough protective efficacy is worthy studying in
the future.
FP-A-025
Experimental study in the effect of platelet activating
factor and its antagonist on hypoxic ischemic
encephalopathy
W. Wang, D. Li, S.-Y. Li, Y. Gao
Children Hospital of Changchun, Changchun, China
Objective: To investigate the relationship between platelet
activating factor (PAF) and hypoxic ischemic encephalopathy
(HIE), and new method of therapy. Methods: Serum
PAF in Wistar rats and 20 patients with HIE were assessed
by improved Henson’s method. The PAF antagonist
(BN51021) was used to treat test rats with HIE. Results:
The level of serum PAF in test Wistar rats and patients
with HIE was significantly higher than that of normal
control group P , 0:001). The level of serum PAF in tests
Wistar rats with higher PAF was reduced by ginkgolides
antagonist (BN51021). Conclusion: The PAF is related to
HIE, can be considered as a marker to assess HIE severity
and prognosis. The ginkgolides antagonist (BN51021) can
obviously inhibit the production of PAF in test Wistar rat
with HIE and decrease serum PAF (0.001). This study may
help for future treatment of HIE.
FP-A-026
Stimulation of N-methyl-d-aspartate (NMDA) receptors
inhibits neuronal migration in the embryonic cerebral
cortex
M. Kihara a , H. Yoshioka b , K. Hirai b , M. Murata b ,K.
Hasegawa b , Z. Kizaki b , T. Sawada c
a Kyoto First Red Cross Hospital; b Department of Pediatrics,
Kyoto Prefectural University of Medicine, Kyoto,
Japan; c Kyoto Second Red Cross Hospital, Japan
The role of the NMDA receptor in neuronal migration in
the cerebral cortex is still unclear. We performed a tissue
culture study using embryonic rat brain to investigate the
effect of excessive stimulation of NMDA receptors on the
neuronal migration in the cerebral cortex. Four significant
cellular zones were identified in the cultured cerebral wall:
the ventricular zone, the inner intermediate zone, the outer
intermediate zone, and the cortical plate. After we labeled
progenitor cells in the ventricular zone of E16 cerebral
cortex explants by [ 3 H] thymidine, the explants were
cultured for 48 h, and distribution of labelled cells was
autoradiographically evaluated. Stimulation of NMDA
receptors, by adding the NMDA receptor agonist, NMDA
(10 mM) or ibotenate (1 mM), to the culture medium caused
a significant percent decrease of labeled cells in the intermediate
zone and an increase in the ventricular zone. This
suggests that stimulating NMDA receptors by agonists inhibits
neuronal migration in rat cerebral cortex. It is known
that blocking NMDA receptors also inhibits neuronal migration
in the cerebral cortex. These results suggested that acti-

420
Abstracts
vation of NMDA receptors within a relatively narrow
window is essential for a normal rate of neuronal migration.
FP-A-027
The effect of pentylenetetrazol on the activation of NFkB
in cultured primary hippocampal cells
K.-Y. Wang, X.-Z. Ruan, S.-Q. Zhu, W. Wang
Department of Neurology, Tongji Hospital, Tongji Medical
College, Huazhong; University of Science and Technology,
Wuhan, China
Objective: To investigate the role of pentylenetetrazol on
the activation of NF-kB in cultured primary hippocampal
cells. Methods: We measured the apoptosis and inhibiting
rate initiated by pentylenetrazol (PTZ) in both PB-treated
and PB-untreated primary cultured hippocampal cells
respectively by a FACScan side scatter analysis and MTT
method. The activation of NF-kB was detected by laser
scanning confocal microscopy with FITC-tagged p65 antibody
and PI-staining DNA. Results: NF-kB/p65 associated
green fluorescence can be seen in the nuclei of PB-untreated
hippocampal cells after PTZ stimulation, but were not seen
in the nuclei of cells without PTZ stimulation and in the
nuclei of PB-treated cells after PTZ stimulation. After PTZ
stimulation, surviving rates of PB-treated hippocampal cells
were not significantly higher than those of PB-untreated
hippocampal cells (P . 0:01). The percentages of apoptosis
in PTZ stimulated PB-untreated cells were not significantly
higher than those without PTZ stimulation and PB-treated
cells after PTZ stimulation (P . 0:01). Conclusion: These
results demonstrated that PTZ can activate NF-kB of hippocampal
cells, and not induced excitotoxicity. PB may interfere
the effect of PTZ through the pathway of the activation
of NF-kB.
FP-A-028
The mechanism and pathway of cytokine-induced
apoptosis in astrocytes of rats
J.-B. Liu, Y.-J. Yang
Department of Pediatric, Xiangya Hospital, Changsha,
Huna Province, China
Objective: To explore the mechanism of LPS, interleukin
1b (IL-1b), and TNF-a-induced apoptosis and the pathway
of the apoptosis in astrocytes of rats. Methods: The astrocytes
were cultured in vitro and treated with sole or combination
of LPS, IL-1b, and TNF-a. Cell viability was
demonstrated by MTT method. Apoptosis was observed
by fluorescence microscope using acridine orange, EB,
and anti-annexinV-cy3 monoclonal antibody and by electron
microscope. Apoptosis rates of astrocytes were evaluated
by flow cytometry; NO 2 2 /NO 2
3 were assayed by
spectrophotometer; cytochrome c in cell plasma was determinated
by Western blot; caspase-3 mRNA was observed
by in situ hybridization. Results: LPS 1 IL-1b 1 TNF-a
can decrease the cell viability to 0.46 ^ 0.15. The amounts
ranking of MTT in cells were LPS 1 IL-1b 1 TNF-a treated
.LPS 1 IL-1b treated .LPS 1 TNF-a treated.
Amount of NO 2 2 /NO 3 2 produced by astrocytes stimulated
by LPS 1 IL-1b 1 TNF-a was the highest at 72 h. Amount
of NO 2 2 /NO 3 2 and MTT of astrocytes had negative relation,
l-NMMA pretreatment can attenuate NO 2 2 /NO 3 2 produced
by astrocytes stimulated by LPS 1 IL-1b 1 TNF-a and
relieve the cell viability. Cytochrome c in cell plasma of
astrocytes stimulated by LPS 1 IL-1b 1 TNF-a and the
expression of caspase-3 mRNA were higher than normal
control. l-NMMA decreased the amount of cytochrome c
in cell plasma of astrocytes and the expression of caspase-3
mRNA. Conclusion: Cytokines decreased the viability of
astrocytes because of autoendocrine of NO. Inhibition of
NO by l-NMMA relieved the viability of astrocytes stimulated
by LPS 1 IL-1b 1 TNF-a. NO can cause astrocytes
apoptosis, this may relate to releasing the cytochrome c
from mitochondria cell plasma, and activating the expression
of caspase-3 mRNA.
FP-A-029
Treatment of Rett syndrome patients with
transplantation of embryonic nervous tissue
V.I. Tsymbaliuk, N.A. Pichkur, L.D Pichkur
Institute of Neurosurgery Named Academician A.P. Romodanov,
Academy of Medical Sciences, Ukrainian Children’s
Specialized Hospital ‘OKhMATDET’, Kyiv, Ukraine
RS is a neurodevelopmental disorder occurring exclusively
in girls. D. Armstrong et al. (1995) found striking
decrease in the dendritic trees of selected cortical areas,
chiefly projection neurons of the motor, association, and
limbic cortices. They suggested that the pathologic changes
were based on the trophic factors deficit. Degenerating
axons in the caudate nucleus were identified, which suggests
dysfunction in the dopaminergic nigrostriatal system. The
authors suggested applying the method of RS treatment with
transplantation of embryonic nervous tissue into the motor
area of cerebral cortex. Treatment and assessment of five
patients with classic RS phenotype aged 3–10 years was
conducted. Chromosomes and metabolic screening results
of these patients were normal. The patients were observed
6–12 months post-operatively. Positive dynamics in clinical
status was identified in five patients: reductions in stereotypic
behaviors (body rocking, hand mannerisms). Purposeful
hand use improved in two patients. They re-acquired
self-service feeding skills and were able to grasp objects
and transferred them from hand to hand. Signs of spasticity
with rigidity in lower extremities and truncal ataxia diminished
and gait improved (in four patients). Bursts of hyperventilation
stopped in two patients. Significant
improvement was noted in the youngest patients (3 and 4
years old). The changes were stable. Embryonic nervous

Abstracts 421
tissue transplantation could be a potential method of RS
treatment.
FP-A-030
Expression of adhesion and extracellular matrix
molecules in the developing human brain
B. Anlar, P. Atilla, A.N. Çakar, M.F. Kose, M.S. Beksaç,A.
Dagdeviren, Z. Akçören
Hacettepe University Department of Pediatric Neurology,
Ankara, Turkey
Cell adhesion molecules (CAM) and extracellular matrix
molecules (ECM) have important roles in cell migration and
connection. Their developmental expression has not been
fully described in human brain. In this report, CAM and
ECM immunohistochemistry was examined in frontal tissue
samples from 14 to 28 weeks old fetuses aborted for obstetrical
reasons (n ¼ 22) and four fetuses with nervous system
abnormalities. Neural adhesion molecule (NCAM) and
fibronectin were present at all gestational ages examined
while laminin and tenascin expression started at 17 weeks.
As gestational age progressed, expression of NCAM and
glial fibrillary acidic protein tended to increase while N-
cadherin and laminin decreased. The distribution was predominantly
vascular for laminin and fibronectin, and in the
neuropil for NCAM, tenascin and thrombospondin. The
expression of thrombospondin and fibronectin shifted from
periventricular to outer cortical layers with advancing gestational
age. This time- and site-related expression supports
the role of these molecules in tissue differentiation and
response to injury. The descriptive data obtained in this
study might constitute a basis for further studies investigating
the role of CAM and ECM in developmental abnormalities
of the CNS.
FP-A-031
Transient intrauterine hypotension causes apoptosis in
fetal brain and affects learning
M. Tombakoglu, M. Durakoǧlugil, G. Kale, H. Orer, B.
Anlar
Hacettepe University Departments of Pediatric Neurology,
Pediatric Pathology and Pharmacology, Ankara, Turkey
Hypotensive episodes are frequent during pregnancy, and
their functional effect on fetal brain has not been studied.
We produced systemic hypotension for 30 min during midgestation
in pregnant rats and examined their offsprings on
postnatal days 1 and 28. When compared to sham controls,
the brains of the hypotensive group contained more
TUNEL-positive cells in the hippocampal and periventricular
regions on both time points. Spatial learning was
impaired in 28 day-old pups of the hypotensive mothers,
and correlated with the number of apoptotic cells. These
results indicate that transient maternal hypotension induces
apoptotic cell death in fetal brain and affects learning. Similar
mechanisms might be involved in the pathogenesis of
human learning disorders.
FP-A-032
Use of fluorescent proteins expressed in specific neural
cell types to trace differentiation of neural progenitor
cells
M. Maletic-Savatic a,b , J. Mignone a , R. Malinow a ,G.
Enikolopov a
a Cold Spring Harbor Laboratory, 1 Bungtown Road, Cold
Spring Harbor, NY, USA;
b School of Medicine, State
University of New York, HSC T12-020, Stony, Brook, NY,
USA
Neurogenesis, a process first thought to be limited to the
prenatal period, occurs throughout life in distinct brain
regions, such as the subventricular zone and the dentate
gyrus subgranular zone. Over the past decade, a considerable
amount of data has been accumulated regarding the identification,
isolation, propagation, and pluripotency of neural
progenitor cells (NPC). However, little is known about the
specific mechanisms that commit differentiation of neural
progenitor cells toward a specific cell lineage, such as the
neuronal or glial lineage. To investigate the in vivo differentiation
of neural progenitor cell, we developed a unique
approach, which enabled us to visualize differentiation of
neural progenitor cells into different neural cell types,
under a variety of experimental conditions. We generated
transgenic mice that express different fluorescent proteins,
specific to a neural cell type, and we observed differentiation
of NPCs into these cell types using two-photon microscopy.
We studied this process in organotypic slice cultures derived
from the mature (.3-week-old) transgenic mice. This
culture model is a very-well studied model that closely
mimics in vivo conditions, with preserved hippocampal
circuitry. The therapeutic potential of adult hippocampal
neurogenesis may be profound. Identification of specific
conditions that promote neurogenesis will increase the
potential use of NPCs as agents for brain tissue reorganization
and repair.
FP-A-033
Changes of calcium calmodulin and phasphodiesterase
in the brain damage of newborn pigs of hypoxic ischemic
encephalopnthy
L.-P. Hu
Beijing Military Medical College, Beijing, China
Objective: The changes of calcium (Ca 21 ), calmodulin
(CaM) and phosphodiesterase (PDE) were tested in the
brain damage model of newborn pigs HIE. Methods: PDE
indirect determine of improvement Thompson CaM, PDE
and CaM were removed in the response solution and atom

422
Abstracts
spectrophotometry. Results: The brain Ca 21 content was not
change at unit volume of fresh cerebral tissue, CaM content
and PDE activity rose significantly at that time and 12 h of
the brain damage. Conclusion: The changes of CaM content
and PDE activity were related to the brain damage of HIE.
FP-A-034
Resistance to brain damage caused by prolonged
seizures in rats with different maturational stages
L. Jiang, F.C. Cai, X.P. Zhang
Children’s Hospital, Chongqing University of Medical
Sciences, Chongqing, China
Objective: To demonstrate the special ability to resist brain
damage from prolonged seizures and convulsive status
epilepticus (SE) in premature animals. Methods: Megimide
was injected in 80 healthy and adult rats respectively to evoke
prolonged seizures and SE. The incidences of injured
neurons were analyzed at the slices of hippocampus CA3,
dentate gyrus and parietal cortex. Results: (1) Adults rats
(ARs) only had less than 20 min prolonged seizures with
the same dose of megimide. However 71.4% baby rats
(BRs) experienced SE. (2) The average duration from the
onset to death during the period of seizures was 44 ^ 24
min in BRs, and 7 ^ 5 min in ARs. (3) Comparing the incidences
of dead neurons at different cortical areas, a typical
pathologically pattern of selective neuron damage was
shown predominately at the hippocampus and dentate
gyrus. (4) On the 4th day after the seizure in BRs, the number
of necrotic neurons counted in the hippocampus and dentate
gyrus was 60 ^ 21 in the group with seizures lasting #29
min; 167 ^ 51 with seizures lasting 30 ^ 59 min. However
there were 227 ^ 34 min of necrotic neurons in ARs although
the seizures lasted only #20 min. The number of dead
neurons could be equal between ARs and BRs only when
the seizures of BRs was lasting .60 min. Conclusion: (1)
Premature brain was more likely to have prolonged seizures
or SE than adults. (2) There was a big difference of the resistance
to brain damage caused by severe seizures between the
premature and mature brain, the more premature the more
resistant. (3) Brain damage induced by severe seizures was
characteristically observed as selected neuronal loss predominantly
at the hippocampus and dentate gyrus. (4)
Prolonged SE, particularly more than 60 min, could also
induce brain injury in BRs in spite of their much stronger
resistance to the injury.
FP-A-035
Serial observation on the patterns of selective neuron
injury after prolonged seizures in premature and
mature brain
L. Jiang, F.C. Cai, X.P. Zhang, X. Li
Children’s Hospital, Chongqing University of Medical
Sciences, Chongqing, China
Objective: To explore the difference of the patterns on
selective neuron injury after prolonged seizures or convulsive
SE in the premature and mature brain. Methods: ARs
experienced 15–20 min seizures, and BRs experienced 21–
75 min seizures were sacrificed at six time points during the
period of 4–168 h after the seizure stopped. The necrotic and
apoptotic neurons were counted microscopically. The incidence
of apoptosis was also comparatively studied by FACS,
TUNEAL and electron microscopy. Results: (1) The process
of neuronal death was shown even at 4 h after severe seizures.
The peak of necrotic process reached at 24–48 h after
seizures in ARs (180 ^ 38 cells). It was two times higher
than BRs (90 ^ 5.9 cells). (2) In ARs, apoptotic neurons
had been shown from the early stage after seizures, and the
peak level of apoptosis (28 ^ 3.3%) was reached at 24–72 h
after prolonged seizures, which was 5–8or5–6 times higher
than controls, and 4–7 or 3–4 times higher than BRs
measured by FACS or TUNEAL. (3) However, there was
only a mild increase of apoptotic neurons in BRs at early
stage, but lower than that of control in 24 h. Conclusion:
(1) Selective neuronal death induced by prolonged seizures
and SE resulted from necrosis and apoptosis. (2) Significant
processes of necrosis and apoptosis of neurons could be well
presented from the early stage, and apoptosis became dominant
with time 12 h after seizures in ARs. (3) The severity of
neuronal death in BRs was much lower than ARs, especially
in the process of apoptosis after severe SE. It was indicated
that there could be an internal response physiologically in the
premature brain after SE to protect neuronal death, or even to
inhibit the process of apoptosis.
FP-A-036
A molecular biological study on the resistance to brain
damage induced by prolonged seizures in the premature
brain
L. Jiang, F.C. Cai, X.P. Zhang
Children’s Hospital, Chongqing University of Medical
Sciences, Chongqing, China
Objective: In a previous study we found that the premature
brain has a stronger resistance to brain damage induced
by prolonged seizures or SE. In this study we tried to
explore the molecular biological basis of internal protective
response in the premature brain which reduces the process
of apoptosis and necrosis of neurons. Methods: Megimide
was injected in healthy ARs and BRs to evoke prolonged
seizures and SE. ARs and BRs rats were sacrificed at 1/2, 2,
8, 24, 48, and 72 h after the prolonged seizure stopped.
Hippocampus, dentate gyrus and parietal cortex were
taken for immunocytochemistry and Western blot studies
for expression of apoptosis associated genes bcl-2 and
p53. Results: (1) There was a much stronger and higher
rate (90%) of p53 expression in ARs’ brain, and such a
state was continually kept even at 72 h after the seizure
stopped; however, bcl-2 expression was weak and low

Abstracts 423
(57%), and no difference was found in bcl-2 expression at
several hours as compared with normal controls. (2) In BRs’
brain there were more than 85% neurons with a strongest
expression of bcl-2; and the strongest bcl-2 expression was
sustained 72 h after SE. However p53 expression lasted less
than 24 h. Conclusion: Significant difference between the
premature and mature brain was shown in genes expression
associated apoptosis after seizures. Anti-apoptosis gene
(bcl-2) expression was much higher and longer than apoptosis
gene (p53) in BRs’ brain, and it was reversed in the
ARs group. The strongest expression of bcl-2 after SE could
be the important molecular biological basis for the special
resistance to neuronal injury in the premature brain.
FP-A-037
A dynamic study of neuronal death after prolonged
seizures in rats
L. Jiang, F.C. Cai, X.P. Zhang
Children’s Hospital, Chongqing University of Medical
Sciences, Chongqing, China
Objective: To explore the feature of selective neuronal
injury after prolonged seizures in the mature brain, and to
explore the relationship of serum neuron specific enolase
(NSE) with brain damage. Method: Prolonged seizures
were evoked by Megimide in 30 adult Wistar rats. All rats
experienced prolonged seizures with duration of 15–20 min.
Five rats were sacrificed at 4, 12, 24, 48 and 72 h and 7 days
after seizures stopped. The dead neurons were observed
light-microscopically (HE & Thionine staining) and electron-microscopically
at the areas of hippocampus and the
cortices. At the same time, the serum level of NSE was tested
by ELISA. Results: (1) There was a obvious selective neuronal
death after prolonged seizures. The process of neuronal
death was remarkably shown even at the early stage of 4 h
after severe seizures. A peak of necrotic process was counted
at 24 , 48 h after seizures (180 ^ 38 cells); it was five times
higher than that for controls (38 ^ 6 cells). (2) By the ELISA
study, the NSE level was also increased 4 h after seizures, and
reached its peak concentration at 48 h (5.13 ^ 0.77 nmol/l),
much higher than that of controls (3.53 ^ 0.87 nmol/l).
Conclusion: Prolonged seizures in the mature brain will
lead to selective neuronal death mainly in the hippocampus,
which was related with changes of serum NSE. Serum NSE
could be a useful marker for detecting neuronal death.
FP-A-038
The role of heat shock reaction for protection of brain
damage in the premature brain after prolonged seizures
L. Jiang, F.C. Cai, X.P. Zhang
Children’s Hospital, Chongqing University of Medical
Sciences, Chongqing, China
Objective: In our previous study we found that the premature
brain has a stronger resistance to brain damage induced
by prolonged seizure. In this study we tried, to explore the
role of heat shock reaction (HSR) and its molecular mechanism
for protection of brain damage in the premature brain
after prolonged seizures. Method: Prolonged seizures were
evoked by Megimide in 30 each of BRs and ARs, respectively.
At 1/2, 2, 8, 24, 48 and 72 h after the seizures stopped,
five rats were sacrificed in each group, and the hippocampus,
dentate gyrus and temporal cortex of their brains were taken
for the following studies: (1) expression of HSR associated
protein, heat shock protein70 (HSP70) was tested by immunocytochemistry;
and in thick liquid of brain tissue by
Western blot; and (2) measurement of the NO level in thick
liquid of brain tissue by fluorospectrometry. Results: (1)
There was a big difference in expression of HSP70 between
the premature and mature brain after severe seizures. Positive
rate of the neurons with HSP70 expression was as high as
85% in the premature brain, and 52.5%of them with strongest
or stronger expression. However, in the adults’ brain, the
positive rate was 65% only, particularly with weak expression
in most (77.5%). (2) A similar result was obtained by
Western blotting. The strongest or stronger HSP70 expression
was well shown continually in the premature brain even
3 days after severe seizures. In contrast, the strongest or
stronger HSP70 expression in the adults’ brain was sustained
less than 24 h. (3) The NO level in the brain quickly increased
in both age groups after severe seizures. A peak level
(9.0 ^ 1.2 nmol/l) in the baby brain was reached within 2 h
after SE but decreased rapidly in 8 h and was lower than
control (5.0 ^ 1.4 nmol/l) 24 h later. In contrast, there was
a peak level (6.6 ^ 1.2 nmol/l) in 24 h after seizures but with
the high level continually in the adult brain. Conclusion: (1)
There was also a significant difference between the premature
and mature brain in heat shock response after prolonged
seizures. There was quick and continual HSP70 expression at
a high level to protect the neurons in the premature brain, but
only weak expression temporally in the adult brain. (3) The
strongest expression of HSP70 after SE could be the important
molecular biological basis of special resistance to neuronal
injury in the premature brain. They are not only to inhibit
apoptosis and protect neurons but also to reduce NO in brain.
FP-A-039
An experimental study on rectal administration of a
mixed solution of clonazepam with acetaminophen
L. Jiang, F.C. Cai, P. Qu
Children’s Hospital, Chongqing University of Medical
Sciences, Chongqing, China
Objective: To study the efficacy of clonazeparm (CNP)
after rectal administration of CNP with acetaminophen
(ACE). Methods: (1) The mixed solution of CNP-ACE (1
mg CNP with 100 mg ACE) and CNP were administered
rectally to Wistar rats in a dose of 1.5 mg/kg, respectively.
(2) The concentration of CNP was measured by HPLC in the

424
Abstracts
blood at different time points, and the parameters of pharmacokinetics
were calculated by a computer program of
3P87. (3) The pharmacology of CNP was detected by the
change of EEG patterns and the efficacy of seizure control.
(4) At the same time the rectal mucosa was studied pathologically.
Results: (1) The pharmacokinetic study demonstrated
that CNP was rapidly absorbed from rectum in both
groups with a peak blood level at 15.3 ^ 6.1 and 15.8 ^ 1.6
min, respectively. (2) Within 5 min after CNP or CNP-ACE
administration, EEG patterns were significantly changed
and the frequency of b band was increased by 7.8 and
7.3%. (3) Compared with the control group, the incidence
of seizures, duration of seizures, and death induced by
seizures were decreased from 100 to 50, 20%; 9.8 ^ 3.2–
0.7 ^ 0.6, 0.3 ^ 0.1 min; 70% to 0, respectively. (4) There
were no obviously pathological changes of rectal mucosa or
any side effect in both groups. Conclusion: ACE had no
obvious interfering effect with CNP when such a mixed
solution was administered rectally in Wistar rats. CNP-
ACE may be a quick and effective way to control febrile
seizures in clinical work, especially for outpatients.
FP-A-040
The expression of caspase-3 mRNA in the hippocampus
of 7-day-old hypoxic-ischemic rats and the mechanism of
neural protection by magnesium sulfate
Y. Tang, F.-L. Zhao
Department of Pediatrics, The Third Hospital of Beijing
University, Beijing, China
Objective: There was consanguineous relationship
between caspase-3 and early damage after hypoxia and ischemia.
Caspase-3 played a key role in the process of apoptosis
in the neuron. Magnesium sulfate could protect the neuron
from injury but the mechanism was not clear yet. The
research was to investigate the expression of caspase-3
mRNA in the hippocampus of 7-day-old hypoxic-ischemic
rats and the possible mechanism of neural protection by
magnesium sulfate. Methods: The model of 7-day-old
hypoxia-ischemia rats was performed. The rats were divided
randomly into six groups as follows: (1) normal control
(n ¼ 4); (2) sham surgery control (n ¼ 4); (3) hypoxia-ischemia
(n ¼ 4); (4) sodium chloride injection with hypoxiaischemia
(n ¼ 4); (5) magnesium sulfate pro-injection with
hypoxia-ischemia (n ¼ 4); and (6) magnesium sulfate postinjection
with hypoxia-ischemia (n ¼ 4). The therapy groups
received a bolus injection of 500 mg/kg magnesium sulfate
intraperitoneally 0.5 h before or after hypoxia-ischemia.
Semi-quantitative RT-PCR was used to measure caspase-3
mRNA expression in the hippocampus 24 h after hypoxiaischemia.
The differences were compared among the groups.
Results: The expression of caspase-3 mRNA was significantly
increased in the hippocampus of the hypoxia-ischemia
pups (P , 0:01). Both magnesium sulfate pro-injection and
post-injection significantly decreased the expression of
caspase-3 mRNA (P , 0:05). Conclusion: Caspase-3 is activated
in the hippocampus of the 7-day-old rats 24 h after
hypoxia-ischemia. The suppression of the expression of
caspase-3 mRNA in the hippocampus is probably related to
the protective effect of magnesium sulfate to brain injury
with hypoxia-ischemia.
FP-B
Epidemiology
FP-B-001
A neuroepidemiological study on severe motor and
intellectual disabilities syndrome in Okayama
prefecture
Y. Yamatogi, M. Morooka, M. Murao
Okayama Prefectural University, Kuboki Soja-City, Japan
Objective:Toclarifytheinfluencesofmedicalprogressand
social support systems, a neuroepidemiological study was
carriedoutonseverelymentalandmotorhandicappedpersons
with onset before 18 years of age, on the prevalence day of
January 31, 2001, in Okayama Prefecture in Japan. Results:
(1) 681 severely handicapped persons were found in a population
of1 958 403; prevalence rate was 0.348/1000 population
(0.414 for males and 0.287 for females). Prevalence rates in
the age groups were: 0.33 (0–4 years); 0.87 (5–9 years); 0.97
(10–14 years); 0.73 (15–19 years); 0.57 (20–24 years); 0.52
(25–29 years); 0.55 (30–39 years); 0.32 (40–49 years); 0.04
(50–69 years); and (2) cerebral palsy was most frequently
observed in 57.7% of patients, followed by post-encephalitis
orencephalopathicstatesin13.5%.Etiologieswerepresumed
prenatal in 28.8%, perinatal in 42.6%, postnatal in 22.6%, but
undetectable in 6.0%. A total of 72.8% had epilepsy, which
was more prevalent in those with more severe mental retardation.
(3) According to the severity of handicap, 52.3%
belonged in the severest group and 38.2% in the group with
some, but useless, movement and severe mental defect. The
rate of those institutionalized was 43.5%; lowest in the severest
group, but with a rapid increase after the age of 20 years.
Conclusion: No change was observed in the prevalence rate in
school-age during the last 35 years, but was etiologically
different; very-low-weight births, brain malformations, chromosomal
abnormalities and postnatal factors were increased
and hyperbilirubinemia was markedly decreased. Extremely
severe cases are increasing.
FP-B-002
A study on the epidemiology of childhood cerebral palsy
in Zhejiang Province, China
Z.-Y. Zhao, X.-J. Zhou, Q.-X. Shui
The Affiliated Children’s Hospital of Zhejiang University
School of Medicine, China
Objective: To understand the prevalence and status of CP

Abstracts 425
in children, in order to provide basic data for future prevention
and therapy of CP. Methods: During October and
November 1998, we investigated the status of CP among
children aged 1 , 6 years from 66 townships of 15 cities or
counties in Zhejiang province. The investigators were given
professional training before the study. Physical examinations
were performed on children selected by screening
standard. According to the diagnostic standard, some of
these children were diagnosed with CP. Results: The register
comprised 62–949 children aged 1 , 6 years, 92 of
whom were diagnosed with CP (1.46‰). No statistical
significance was found between sex or age (P . 0:05).
Furthermore, no significant difference was found in prevalence
between children in cities and rural areas. Conclusions:
The prevalence of CP in Zhejiang province was
lower than that of some developing countries, and also
lower than that of Jiangsu province.
FP-B-003
Epidemiology of childhood brain tumor in Croatia – 30
years analysis of a series of hospital patients
I. Prpić, E. Paučić-Kirinčić, M. Smokvina, A. Sasso, Z.
Modrušan-Mozetič, D. Fiket
University Children Hospital ‘Kantrida’ and Medical
Faculty of University of Rijeka, Rijeka, Croatia
From 01.01.1971. to 31.12.2001 in the Pediatric Clinic
‘Kantrida’, 87 children with primary brain tumor were
analysed retrospectively and prospectively. The mean incidence
of brain tumor was three children annually. There
were 48 boys and 39 girls, and the average age was 6.7
years (^4.1 years). In 48 children (55.1%) the tumor was
located infratentorial (IT), and in 39 (44.8%) supratentorial
(ST). The most common pathohistological type was astrocytoma
and medulloblastoma. Before the brain tumor was
confirmed, symptoms lasted an average of 55.9 days
(median 30 days); the IT tumors 73.1 days, and the ST
tumors 31.5 days, which is a statistically significant difference.
In making a diagnosis, based on neuroimaging techniques,
brain CT was used from 1978, and MR
systematically since 1989. With the purpose of early detection
of brain tumor in children, our data indicates the need
for systematic follow-ups and broader medical attention to
children who besides specific tumor symptoms, show nonspecific
EEG changes. During the last 10 years (1991–2001)
from a total of 36 children diagnosed with brain tumor, eight
have died (22%), 16 are still living (44%), while the
outcome for the other 12 children is unknown. This is in
line with a general survival rate of children diagnosed with
brain tumor (40–50%). Results of our research are comparable
to the results of research conducted on a larger number
of patients which point to the fact that analysis of a hospital
series of patients provides reliable data. Moreover, such
research can be used to implement a registry of children
with history of brain tumor with the purpose of following
the progress of the treatment. and analysing the immediate
and long term effects on the children’s quality of life.
FP-B-004
The epidemiological data of febrile seizures in the
northern coastal region of Croatia
E. Paučić-Kirinčić, S. Surijan, I. Prpić, Z. Modrusan-
Mozetič, A. Sasso, D. Fiket
University Children Hospital ‘Kantrida’ and Medical
Faculty of University of Rijeka, Rijeka, Croatia
Clinical and epidemiological investigations of FS have
appeared in the paediatric literature for many years but
accurate data from Croatia are deficient. From 1.1.1996.
till 31.12.2000. at the University Children Hospital
‘Kantrida’ 399 children with FS were retrospectively and
prospectively analysed. Children Hospital ‘Kantrida’ is the
only referral specialist child health centre in this region of
Croatia and all children are referred to the Hospital to ensure
accuracy of the presented data. There were 208 boys and
191 girls. The peak age incidence was between 12 and 24
months and 87.9% of the children were between 7 months
and 3 years of age. The FS were frequently connected with
upper respiratory tract infections and most FS occurred in
the month of February. A total of 271 (67.9%) children had
simple FS, 37 (9.3%) children had complex FS and 91
(22.8%) children had repeated FS. The mean days of hospitalization
were 8.9 days, but longer for complex FS (10.8
days). FS represents 18.2%, of total number of hospitalized
children in the Department of Child Neurology, which is
time and money-consuming for conditions with minor
long term consequences. The results of our study are similar
to the results of researches conducted on larger numbers of
patients, which demonstrates that analyses of a hospital
series gives reliable data.
FP-B-005
A clinical-epidemiological study of epilepsy in the first 3
years of life among children who attended the
Alexandria university children hospital for the period of
1 year
F.M. Kamel, M.I. Kamel, T. El-Omar, A.M.M. Maged
Department of Pediatrics, Faculty of Medicine, Alexandria
University, El-Shatby, Alexandria, Egypt
There are many methodological difficulties in studying
epidemiological aspects of epilepsy are developing countries.
These include: incomplete and inaccurate medical
records; questionnaires may miss mild, partial and inactive
epilepsy; traditional views of illness; symptoms and etiology
of epilepsy are different in rural areas; and lastly, epileptic
seizures may be misdiagnosed. This study comprised a hospital-based
cross-sectional cohort. The aim was to evaluate the
pattern of epilepsy in children aged 3 years or less at the time

426
Abstracts
of the study, among those children who attended the epilepsy
clinic at the Alexandria University Children Hospital for a
period of 1 year (April 1999–April 2000). A total number of
182 children with epilepsy were selected, and an equal
number of age and sex-matched healthy children served as
controls. Records were revised and filed in a special computer
software package. The data included: demographic characteristics,
medical history, family history, seizure
description, neurological signs, investigations, treatments,
compliance and epilepsy classification. High risk factors
found were: parental illiteracy, epilepsy in the family,
parents who were first cousins, lack of antenatal care, obstetric
problems, congenital malformations, home delivery,
admission to neonatal intensive care units, birth asphyxia,
postnatal head trauma, meningitis or encephalitis, non-traumatic
intra-cranial hemorrhage, status epileptics, post-DPT
problems, and febrile convulsions. Partial seizures occurred
in 27.4% of cases, while the generalized were found in
80.2%, and neurological handicaps were found in 52.7%.
Symptomatic epilepsy was diagnosed in 58.2% of cases.
Recognized epileptic syndromes were found in 15.9%.
Monotherapy was the rule in 86.6% of cases. In conclusion:
major determinants of epilepsy in developing countries,
though similar to developed countries, still constitute controversy.
Epilepsy care in developing countries may be in accordance
with international guidelines; however, precise
delineation of the needs should be based on actual characteristics
in each country.
FP-B-006
Clinical epidemiological analysis of seizures in children
for last 5 years
C.-Y. Zhang
Paediatrics of Xi’an No. 1 Hospital, Xi’an, China
Objective: To analyze the characteristics of clinical
epidemiology of seizures in children during the last 5
years. Methods: Collection of data of 169 cases of seizures
in children treated in the Paediatrics Department, Xi’an No.
1 Hospital from January 1997 to December 2001, and analysis
of types of seizure, the illness-age at onset, seasonal and
annual distribution of cases, and related factors. Results:We
treated seizures in 169 children (107 males and 62 females)
over the last 5 years. The annual distribution of cases was
23, 18, 26, 45, 57. With the annual increasing tendency,
60.3% of the cases manifested in the last 2 years. Generalized
tonic clonic seizures occurred in 158 cases (93.5%).
Ninety four patients (55.6%) had a seizure for the first time,
75 patients (44.4%) had a history of previous seizures.
Febrile convulsions occurred in 140 cases (82.8%), the
body temperature was 38 , 398C in 68 cases, 39 , 408C
in 59 cases, and above 408C in 13 cases. The simple type
of febrile convulsion occurred in 105 cases, and the complex
type in 35 cases. The patients with respiratory tract infection
numbered 109 (77.9%). A FS occurred in 29 cases (17.2%),
14 of these patients had a history of complex febrile seizures
(more than three episodes), and 11 of these patients had
partial seizures with abnormal EEG. The peak age at
onset was from 8 months to 3 years; 71.6% (121/169) of
patients belong to this age group. The seizures occurred
most frequently in January (34 cases), December (31
cases), and February (25 cases), a total of 53.3% patients
had seizures in these months. Patients (28.4%) had seizures
in August, September, and October. It seems that the
seizures occurred more frequently in winter and autumn.
Conclusions: Infant seizures are one of the most common
serious and emergent illnesses in the Pediatrics Department,
with an increasing incidence in recent years. There were
obvious features regarding age and season. Respiratory
tract infections were the most common diseases in these
patients. Febrile convulsions, especially the complex febrile
convulsions, are an important danger factor for future development
of epilepsy.
FP-B-007
Incidence patterns of cerebral palsy in Shiga Prefecture,
Japan, 1977–1991
J. Suzuki a , M. Ito b
a Department of Preschool Education, Shiga Women’s
Junior College; b Department of Pediatrics, Shiga Medical
Center for Children, Shiga, Japan
The prevalence of CP in 6-year-old children in Shiga
Prefecture, Japan, born between 1977 and 1991, was
compared in three successive 5-year periods: period I
(1977–1981), period II (1982–1986) and period III
(1987–1991). Data on accumulated age-specific prevalence
of CP was collected and calculated. During the study
period, 242 293 children entered elementary school, and
326 cases (194 boys, 132 girls) of CP were ascertained
in Shiga Prefecture. The overall prevalence of CP per
1000 6-year-old children was 1.35. The prevalence of CP
for 6-year-old children increased from periods II to III,
although it did not vary from periods I to II. The proportion
of low birth weight (LBW) infants and pre-term infants
among those with CP increased during periods I–III. The
prevalence of CP for term and birth weight .2500 g
infants did not vary over the study period. The prevalence
of CP for pre-term and LBW infants, especially gestational
age ,32 weeks and birth weight ,1500 g, increased from
periods II to III, although the prevalence did not increase
from periods I to II. Multiple births and use of mechanical
ventilation increased from periods II to III. The changes in
the prevalence of CP in Shiga Prefecture suggest that the
intact survival among pre-term and LBW infants increased
in period II owing to better obstetric and neonatal care, and
further improvement in survival of very small babies
receiving intensive care increased CP prevalence in period
III.

Abstracts 427
FP-B-008
The prevalence of epilepsy in Turkish children aged 0–
16
A. Serdaroglu, S. Özkan, K. Gücüyener, K. Aydin, S.
Tezcan, S. Aycan
Sebahat Tezcan, Sefer Aycan, Ankara, Turkey
The aim of this cross-sectional study was to determine the
prevalence of epilepsy in Turkey among children aged 0–16.
The study population comprised 24 773 569 children aged 0–
16 living in Turkey. Since the prevalence of epilepsy in childhood
was 1–0.001% in the world literature, the sample size
was determined as 48 260 with 0.05 error type I; 0.10 error
type 2 (power 0.90) and effect size was 2. By cluster sampling
methods, children from city districts, sub-districts and
villages were selected according to their weight. A total of
46 813 (97 %) children were reached. In the study questionnaire,
there were sections regarding personal information,
questions about related factors, and questions about the diagnosis
of epilepsy and physical examination. Epilepsy classification
was made according to the International League
Against Epilepsy (ILAE). The prevalence of epilepsy was
determined as 0.8%. A total of 55.2% of the subjects with
diagnosed epilepsy were grouped as general; 39% partial
and 5.8% were identified. Age, place of residence, mode of
delivery, place of delivery socioeconomic status had no statistically
significant effect on development of epilepsy. The risk
was increased by male sex, and preterm and post term deliveries.
FP-B-009
The prevalence status of fetal alcohol syndrome among
children in the special classes of east Taiwan
H.-T. Kuo a , P.-Z. Tsai b , M.-L. Lee a
a Research Institute and b Faculty of Early Education and
Care, Chaoyang University of Technology, Chinese Taipei
Fetal alcohol syndrome (FAS) is a clinical diagnosis characterized
by fetal pre- and postnatal growth delay, dysfunction
of the central nervous system, and facial dysmorphism,
due to maternal alcohol consumption during pregnancy. In
our study, we focused on the prevalence rate of FAS and fetal
alcohol effect (FAE) on wsux RION special classes in the
area of east Taiwan. The results revealed that of the 847
children (from over ten villages and towns), aged between
6–15 years, who were examined, three were diagnosed with
FAS and two with FAE. This result equals a prevalence rate
of 0.35 (FAS) and 0.24% (FAE). Hualien City has the highest
prevalence rate: 0.63 (FAS) and 0.21% (FAE). The prevalence
rate according to sex is 0.70 (male) and 0.48% (female).
All these results are almost the same, but higher than the
average international rate of 0.19%. In this study, we have
proved: (1) alcohol has the same teratogenicity in Taiwan as
in other parts of the world; and (2) in east Taiwan, there is an
even higher prevalence rate than in other parts of the world.
We think this is a significant phenomenon in Taiwan, because
the children will need additional help that will place a high
financial burden on the government in the future. Of special
importance is the fact that these conditions can be prevented,
if the mother does not drink alcohol during pregnancy.
FP-B-010
The epidemiology of subacute sclerosing
panencephalities at the Philippine General Hospital,
1999–2001
A.M. Salonga, M.B. Lukban, B.C. Sanchez, J.R. Pipo-
Deveza, B.C. Chua
Section of Pediatric Neurology, Institute of Neurosciences,
University of the Philippines-Philippine General Hospital
and Medical Center, Manila, Philippines
The demographic characteristics and incidence of SSPE
among pediatric patients at the Philippine General Hospital
were determined by review of data from the hospital registry
of confirmed cases of SSPE patients from January 1999 to
December 2001. Sixty-three cases were identified. All
patients are of Filipino descent, with 55% coming from the
national capital region (Metropolitan Manila). Age at presentation
ranged from 1.5 to 17 years, with a mean of 9.8 years.
There was a male predominance, with a male: female ratio of
1.2:0.8. Eighty-nine percent of the patients had measles
infection prior to the development of SSPE. Mean age at
measles infection was 1.4 years. Only 37.7% of the cases,
however, had measles vaccination. Of the 89% of patients
who had measles infection, 30% had previous measles vaccination.
Mean average interval from measles infection to onset
of SSPE was 8.6 years. The majority were in the late stage at
diagnosis; 44.4% were in Stage II, and 42.9% were in Stage
III (Dyken Clinical Staging). Only six patients (9.5%) were in
Stage I. Our patients received the following treatment modalities:
intraventricular interferon (six cases), IVIG (15 cases),
isoprinosine or inosiplex alone (18 cases), interferon 1 IVIG
(one case). Isoprinosine or inosiplex was given together with
IVIG or interferon in 33 cases. Anticonvulsants were used as
needed. Fifty-six patients (88.9 %) are alive and seven
patients (11.1%) succumbed to infection. Three patients are
in remission, and attending school at present.
FP-B-011
Multiple sclerosis in children: the data of a prospective
study in Russia
O.V. Bylova, A.N. Boilo, V.M. Studenikin, O.I. Maslova,
E.I. Gusev
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific Center of Child Health (Russian Academy of
Medical Sciences), Moscow, Russia
In Moscow since 1996, we have performed a prospective

428
Abstracts
study of 67 patients with multiple sclerosis (MS), with onset
before 16 years of age (mean duration of observation
4.91 ^ 0.58 years). Age and symptoms of MS onset, duration
of the first and the second remissions, relapse frequency, time
from onset to secondary progression and to permanent
disability with expanded disability status scale (EDSS) ¼ 3
and EDSS ¼ 6 were analyzed. Mean age at onset of MS in 67
children (38 girls and 29 boys) was 11.72 ^ 0.34 years. There
were more boys with MS onset before age 8, and significantly
more girls with MS onset at ages 12–13. The analyses of
initial symptoms of MS showed that optic neuritis was the
most frequent (32.8%), the second was brainstem symptoms
(25.4%), and third was sensory disturbances (20.9%).
Female sex was associated with optic neuritis and sensory
disorders at MS onset, while male sex was associated with
brainstem lesions, both clinically and on MRI. No cases with
primary progressive MS were found. Annual relapse rate was
1.00 ^ 0.06 per year; 49 patients (73.1%) had two or more
relapses during the first 2 years of MS. At the final observation,
seven patients (11.4%) had secondary progressive MS
course, 17 (25.4%) had confirmed disability with EDSS ¼ 3,
and three (4.5%) EDSS ¼ 6. Annual relapse rate, relapse
frequency during first 2 years of the disease, duration of the
first and the second remission were significantly associated
with time to EDSS ¼ 3. The high percentage of young
disabled patients with active MS course, raises the question
of early disease modifying treatment.
FP-B-012
The prevalence of headache in Swedish schoolchildren
K. Laurell, B. Larsson, O. Eeg-Olofsson
Departments of Neuroscience and Women’s and Children’s
Health, Uppsala University, Uppsala, Sweden; Department
of Child and Adolescent Psychiatry, NTNU, Trondheim,
Norway
Headache is one of the most common health problems, the
first symptoms appearing already at school age. During the
last decades the frequency of unspecific headache as well as
migraine has apparently increased in children and adolescents.
The aim of this study, which was performed during
1997/1998, was to report the prevalence of headache in
schoolchildren from the city of Uppsala, and to compare it
with a similar study performed by Bille 1955. The study
group consisted of 1850 schoolchildren, aged 6–17 years
(median:11). In order to limit the sociodemographic differences
and to get a representative population, the material was
stratified with one class in each grade from different schools
in Uppsala. A questionnaire including questions to receive
information about different types of headache was distributed.
The response rate was 74%, equivalent to 1371 children,
686 girls and 685 boys. The IHS criteria were used for classification.
No headache at all during the last year was
reported by 16% compared to 41% in the study by Bille.
Headache of primary type was found in 45%. For both girls
and boys an increased incidence occurred up to 11 years,
whereupon the incidence further increased in girls but not
for boys. The prevalence of migraine was 15.8% and for
tension type headache 13.6%. Besides comparison with
other prevalence studies, the applicability of the IHS criteria
for classifying headache in children will be discussed and an
exclusion of the duration criterion proposed.
FP-B-013
Survey reports of epidemiological studies of Japanese
encephalitis (JE) in Andhra Pradesh, India
P. Nagabhushana Rao a , Z.-Y. Xu b , J. Jacobson c , W. Liu b
a Niloufer Hospital and Department of Neurology, Osmania
Medical College, Hyderabad, Andhra Pradesh, India;
b International Vaccine Institute, Seoul, Korea; c Program
Officer, Bill and Melinda Gates CVP, Seattle, USA
Survey reports of epidemiological studies of Japanese
encephalitis (JE) in Andhra Pradesh, India, from 1980 to
1997 were analyzed retrospectively and cases from the referral
hospital in the state capital were compared to cases from
rural sites to assess the case fatality rate (CFR) and causes of
death. Subsequently, these results were used to outline appropriate
preventative measures for implementation. The CFR
was used to assess the success of the interventions. From 1980
to 1997, there were 9525 suspected cases of JE reported from
rural sites in Andhra Pradesh out of which 3528 died for a
cumulative CFR of 37.04 %. These results were compared
with 189 morbid cases of suspected JE admitted to Niloufer
Hospital (NH) where ventilator facilities were not available
and patients received only symptomatic treatment. Out of
them 23 cases died for a CFR of 12.17%. Causes of mortality
in NH were analyzed and compared with those of rural areas.
The causes of death, mostly preventable in cases treated in the
rural area, significantly differed from those in NH. Case
management differed between the two groups. The care
given in NH could have been given in the rural setting with
the available medical and nursing staff and budget. Therefore,
detailed step-by-step preventive, diagnostic and clinical
management guidelines were prepared and 65 000 copies
were distributed freely and training was provided for implementation.
A dramatic impact was found within 3 years with a
fall in the cumulative case fatality rate to 12.12% in the year
2001.
FP-C
Perinatal/Neonatal Neurology
FP-C-001
Assessment of damage and efficacy of rehabilitation
therapy of posthypoxic encephalopathy
A. Nigin, D.I. Akhmedova, B.G. Gafurov, K.Sh. Salikhova,
Z.K. Mirsaeva
Scientific Research Institute of Pediatrics, Proesd Talant 3,
Tashkent, Uzbekistan

Abstracts 429
Investigations on changes of cerebral circulation in the
circle of Willis performed by transcranial Dopplerography
in young children, showed that in the diagnosis of posthypoxic
states and their complications, in the evaluation of the
efficacy of criteria of the therapeutic effect of neurotropic
drugs such as in acute periods, or stages of dynamic monitoring,
compared to other functional methods, the leading
role may belong to the non-invasive method of ultrasound
investigation, i.e. transcranial Dopplerography. Thus, in our
investigations we determined Dopplerography criteria of
treatment efficacy. Taking into account that hypoxicischemic
and other damages to the CNS have wave character
and pathologic processes not limited by primary focus of
damage, we consider that observation of children should be
carried out gradually during the whole period of the first
year of life, not only in the contingent of children with
severe damage to the CNS, but also in children with clinically
invisible delayed forms. Dynamic Dopplerography of
cerebral hemodynamics enabled us to reliably determine
perinatal damage to the CNS in children with light injuries
of the CNS. This method allowed us to evaluate: (1) efficacy
of therapy administered, and to increase this therapy if
necessary; (2) compensatory capacities of cerebral autoregulatory
mechanisms in selected cases; and (3) to avoid
administration of pathogenically unfounded therapy.
FP-C-002
Neonatal EEG tracing of burst-suppression: etiological
and evolutionary aspects
V. López-Martín, A. Martínez-Bermejo, J. Arcas, C. Roche,
A. Tendero
Service of Neuropediatrics, Hospital La Paz, Madrid, Spain
Introduction: The EEG tracing seen during the neonatal
period which shows so-called discharges of burst-suppression,
is caused by a severe disorder of cerebral electrogenesis
occurring at this time. Objective: To determine the
aetiology, clinical significance and evolution of a group of
newborns with this type of EEG tracing. Patients and methods:
We performed a retrospective study of full term babies
in whom burst-suppression EEG recordings had been
obtained during the neonatal period. Results: We studied
34 patients. In 14 cases the tracing was associated with
hypoxic-ischemic encephalopathy; four with meningitis;
another four with early infantile epileptic encephalopathy
(Ohtahara syndrome); four cases were attributed to drugs
(four with fentanyl associated in one case with phenobarbitone
and in another with midazolam); two cases were due to
early myoclonic epilepsy; three to multiorganic failure; one
to non-ketonic hyperglycinemia and another to leucinosis.
In one patient the aetiology could not be determined. Seven
patients died before the age of 6 months. Severe neurological
sequelae were seen in all the others except for four cases
(three treated with fentanyl and 1 case with hypoxicischemic
encephalopathy). Conclusions: The presence of a
burst-suppression EEG tracing in a term neonate makes it
necessary to perform extensive studies to determine the
aetiology. Although associated with a worse prognosis,
those not treated with piperidine derivatives should be classified
separately. Those treated with piperidine derivatives
have a good prognosis.
FP-C-003
The early intervention treatment of hypoxic-ischemic
brain damage
L. Liu, S.-T. Fu, Z. Liao
Department of pediatrics, The Second People’s Hospital of
Chengdu, China
Objective: For more effective treatment and reduction of
permanent disability of hypoxic-ischemic brain damage
(HIBD) caused of newborn asphyxia. Methods: 60 cases
of mild and serious newborns with HIBD were divided
into the early intervention treatment group and control
group. After HIBD early treatment, for the intervention
group, the training of motion, cognition, language and social
intercourse were given for 2 years. The development quotient
(DQ) and clinical data were used to evaluate treatment
effect. Results: Compared with the score of DQ that was
followed up for 3, 6, 12, 18 and 24 months, the DQ of the
intervention group was obviously higher than that of the
control group (P , 0:05, P , 0:01). The percentage of
unfavorable prognosis of the intervention group was only
5%, obviously lower than that of the control group
(P , 0:05). Conclusion: The ability of compensation and
plasticity of the brain is best in the early infant stage.
Early intervention can promote brain development and the
connective passageways of nerve cells. It is valuable for the
improvement of the patient’s prognosis.
FP-C-004
Correlation between cerebral palsy (CP) and
periventricular leukomalacia (PVL) in premature
infants
K.-Z. Liu, J.-H. Yao, C.-H. Wang
ICU, Shanxi Children’s Hospital, Taiyuan, Shanxi, China
Objective: To study the prevalence of CP in premature
infants with PVL. Methods: 23 premature infants with PVL
comprised the study group; they were divided into two
subgroups according to cranial CT presentations of
whether lesions were symmetrical, 30 full-term infants
with HIE served as the control group. Results: The prevalence
of CP in the PVL group was significantly higher than
that of the control group (P , 0:01). The prevalence of CP
in the PVL group with symmetrical lesion was remarkably
higher than that with unilateral lesion (P , 0:01). Conclusion:
The premature infants with PVL had a higher probability
of developing CP. The severity of the lesions and

430
Abstracts
the symmetry of lesions in cranial CT were good predicting
factors for the neurological outcomes of the patients.
FP-C-005
The changes and clinical significance of determination of
the neuro-specific enolase in serum of neonates with
hypoxic ischemic encephalopathy
Y. Hong a , Y.-M. Liu b , D.-Z. Chen a
Department of Pediatrics, Zhongnan Hospital of Wuhan
University, Hubei Province, China; Department of Neurology,
Zhongnan Hospital of Wuhan University, Hubei
Province, China
Objective: To observe the changes of the neuro-specific
enolase (NSE) concentration in the serum of a neonate with
HIE and evaluate its clinical significance. Methods: Fortyeight
neonates with HIE were examined. The concentration
of serum NSE was measured at 3 and 7 days of life. The
cranial CT scan was examined in the 1st week of life.
Results: The concentrations of serum NSE of neonates
with HIE (diagnosed clinically or by CT) at 3 days of
life increased, especially in moderate and heavy cases,
and were identical to the clinical and CT manifestations.
Conclusions: NSE is a specific parameter for early diagnosis
of HIE and estimate of hypoxic-ischemic brain
damage and prognosis.
FP-C-006
Clinical analysis on late diagnosis and late or irregular
treatment of neonatal hypoxic-ischemic encephalopathy
(HIE)
H. Yang, L. Zhang
Paediatric Department, First Teaching Hospital Medical
College of ShiHeZi University, Xinjian, China
Objective: To emphasize early diagnosis and treatment of
neonatal HIE in order to decrease sequelae. Methods: Retrospective
analysis of perinatal period factors combined with
clinical manifestation and EEG, skull CT data, to sum up the
clinical data and clinical treatment effect of 36 infants with
late diagnosis, and treatment of HIE. Results: A study of the
curative effect and prognosis demonstrated that the early HIE
diagnosed and treated group of infants had better clinical and
EEG results than the later diagnosed group. For infants who
were diagnosed with HIE at age 6–7 months and 1-year-old,
the clinical symptoms were restored more slowly, and no
clear improvement was shown by EEG. In comparison,
those neonates who were admitted to our pediatric department
at the same time and were given the early, regular and
combined treatment, achieved a quite different curative
effect and prognosis. A certain degree sequel of nervous
system have been left, patient’s limb had dyscinesia and
hypophvenia. Conclusion: The curative effect and prognosis
of late diagnosis, and late or irregular treatment given to HIE
patients, are worse than that of early diagnosis and regular
treatment of neonatal HIE.
FP-C-007
The effect of oxygen chamber in the treatment of infant
hypoxic-ischemic encephalopathy (HIE)
Z.-Z. Wei, W.-Q. Geng, M. He
Shenyang Children’s Hospital, Shenyang, Liaoning, China
Objective: To study the clinical response to the oxygen
chamber of 100 infants with HIE. Methods: Control group:
50 cases of infant HIE treated with cerebroprotein hydrolysate
injection. Study group: 50 cases of infant HIE treated
with cerebroprotein hydrolysate injection in the oxygen
chamber. Infant age: 0–5 days after birth. No statistically
significant difference was found between age and sex in the
two groups (P . 0:05). The transparent infant oxygen
chamber was manufactured by 701 Research Institute of
China Boat Industry Company. Observation Index:
NBNA, DQ, response to the treatment and the evaluation
of prognosis. Results: In the control group: 38 cases (76%)
NBNA score $35, 12 cases (24%) NBNA ,35; 36 cases
(72%) DQ $80, 14 cases (28%) DQ ,80. In the treatment
group: 45 cases (90%) NBNA $35, five cases (10%) NBNA
,35 (10%); 46 cases (92%) DQ $80, four cases (8%) DQ
,80. A statistically significant difference was found
between the two groups (P , 0:05). Conclusions: (1) Treatment
of HIE with the aid of an oxygen chamber is satisfactory.
(2) Treatment of HIE with the aid of an oxygen
chamber decreases the treatment-abandoning rate, disability
rate and mortality.
FP-C-008
Early intervention in hyperbilirubinemia and the
abnormalities of brainstem auditory evoked potential
(BAEP)
H.-Q. Li
Woman’s and Children’s Hospital, Baoji, Shanxi Province,
China
Objective: To observe the hyperbilirubinemic damage to
the conducting route of the hearing nerves, and to study the
diagnosis and curative effect of brainstem auditory evoked
potential (BAEP). Methods: Of 62 hyperbilirubinemic cases,
the ratio of abnormal BAEP account for 61%, as the following
increased interval period (PL) of BAEP’s I, III, V waves,
extended and disappeared interval peak length (IPL). Fifty
seven of the 62 cases had abnormal hearing. We divided them
into three groups A–C with different treatment regimens.
Group A: control group; group B: cerebrolysin 1
hyperbaric; and group C: citicoline sodium injection 1
taurine granules. Results: The disappearance ratios of abnormal
hearings of groups A–C after treatment were 40, 70 and
65%, respectively. No notable difference was found in cura-

Abstracts 431
tive effects between the treated groups (t ¼ 5, P . 0:05), but
the control group (group A) was notably different from the
other two groups (t ¼ 15, P , 0:05). Conclusion: As the
ratio of hyperbilirubinemic hearing injuries is 61%, we
should pay attention to it; BAEP has diagnostic value on
hearing nerve conducting route damage caused by hyperbilirubinemia.
It is also a marker for judging the curative effect.
FP-C-009
The therapeutic effect of naloxone plus cerebrolysin in
the treatment of neonatal hypoxic ischemic
encephalopathy
G.-H. Sun
The Second People’s Hospital of Yichang City, Yichang,
Hubei, China
Objective: To observe the therapeutic effect of naloxone
plus cerebrolysin in the treatment of neonatal HIE. Methods:
Sixty one neonates with HIE were randomly divided into
two groups: 31 as the treatment group who were treated by
naloxone plus cerebrolysin, 30 as the control group who
were treated by cerebrolysin only. Results: The clinical therapeutic
effect in the treatment group was 93%, in the control
group it was 83%. There are significant differences between
the clinical therapeutic effects of the two groups, x 2 ¼ 4:69,
P , 0:05. Conclusion: Naloxone plus cerebrolysin can raise
the clinical therapeutic effect of neonatal HIE.
FP-C-010
High-risk infants in Shenyang: netweb screening report
and methods
Q.-F. Yuan, C.-N. Wang, S.-Y. Shang, Y. Jin
Woman and Child Health Center, Shenyang, China
Objective: To conduct overall early screening of high-risk
neonates and initiate early intervention. Methods: On the
basis of second degree netweb, the third degree netweb-
Woman and Child Health Center determined early diagnosis
and treatment according to the following methods: the Vojta
posture reflection, child neurological development method,
muscle tension, joint activity degree, among others and then
made the assessment. Results: 1235 high-risk neonates were
screened, including normal neonates (n ¼ 637), and abnormal
neonates (n ¼ 598); 306 (24.8%) of the abnormal
neonates suffered from breastfeeding jaundice. Among
breastfed babies with jaundice, 65 (21.2%) had hyperbilirubinemia,
183 (14.8%) had central in coordination, 86
(6.9%) had CP, ten (0.8%) suffered from mental retardation,
and 13 (1.1%) from other conditions. All 306 cases of
breastfed babies with jaundice were cured after 1 year of
treatment according to the study protocol. The number of
central in coordination and CP is 115, among which positive
rate is 98%, normalization is 50%. The perinatal period is
one of the main high-risk factors. The objective of early
screening is to identify various adverse factors in the perinatal
period, and initiate early intervention to prevent the
occurrence of CP.
FP-C-011
Effects of tetramethyl pyrazine (TMP), and
prostaglandin E1 on newborn rats with hypoxicischemic
brain damage (HIBD)
Z.-Y. Jin, C.-H. Xu, H.-Z. Li, Z. Jin, C.-J. Jin
Paediatric Department, Yanbian Hospital, Yanji City,
China
Objective: To study the effects of exogenous tetramethylpyrazine
(TMP) and prostaglandin E1 (PGE1) on brain
neuronal apoptosis of hypoxic-ischemic brain damage
(HIBD) in neonatal rats. Methods: We selected 52, 7-dayold
Wistar rats and randomly divided them into six groups:
normal control group (n ¼ 8), HIBD group (n ¼ 7), normal
saline (NS) group (n ¼ 7), TMP treated group (10). After
established HIBD model, intraperitoneally and subcutaneous
injected TMP and PGE1 then the rats were killed
after hypoxia and ischemia for another 48 h alive. The
number of neuronal apoptosis, the apoptotic rate and apoptotic
density of rats was detected after the brain tissue was
stained by HE and TUNEL. Results: The number of neuronal
apoptosis, apoptotic rate and apoptotic density in the
HIBD group was much higher than in the normal controls
(P , 0:01); the value in the TMP, PGE1 and TMP 1 PGE1
groups was much lower (P , 0:01, P , 0:01, P , 0:01,
respectively), and the value in the TMP 1 PGE1 group
was much lower than that in the TMP and PGE1 groups
(P , 0:05). Conclusion: TMP and PGE1 can pass through
the blood–brain barrier and inhibit cerebrocellular apoptosis;
Moreover, a combination treatment of TMP and PGE1
is more effective than if administered separately; they coordinate
and complement each other, and can enhance the
curative effect.
FP-C-012
Experimental research on the hyperbaric oxygen (HBO)
treating mechanism on SD neonate rat model with
hypoxic-ischemic brain damage (HIBD)
L. Liu, Y.-J. Yang, Y.-J. Jia, J.-H. Song
Xiangya Hospital, Central South University, Changsha,
China
Objective: To clarify the mechanism of hyperbaric
oxygen (HBO) in the treatment of the SD neonate rat
model with HIBD. Methods: Forty rats were randomly
divided into four groups: normal, sham, HIBD and HBO
groups. The rats in the HBO group were treated with 2.0
atmosphere absolute HBO every day for 7 days. At the end
of the HBO treatment, we investigated the Bcl-2 and Bax,
apoptosis-regulating protein in CA1 sector of hippocampus

432
Abstracts
and cortex of the brain, and IkBa expression by Western
blot. Results: HBO can increase the expression of Bcl-2 and
reduce the expression of IkBa with no obvious change in
Bax expression compared to the other three groups. Conclusion:
HBO can induce NF-kB activation and the expression
of Bcl-2 to protect the neuron from apoptosis.
FP-C-013
Research on improving the mental development of
hypoxic-ischemic encephalopathy (HIE) children by
early intervention
J.-H. Song, R. Huang, Y.-J. Yang, J. Liu, Z. Luo
Xiangya hospital, Central South University, Changsha,
Hunan, China
Objective: To understand the influence of early intervention
on the mental development of children with HIE, and to
ascertain the best time for this measure. Methods: 36 children
with HIE, diagnosed by doctors at the outpatient
department, who did not receive early intervention, served
as the control group. Thirty two children who accepted early
intervention comprised the intervention group. Mental
development of the children in both groups was tested by
the CDCC mental developmental test. Results: The Mental
Development Index (MDI) score of one child and the
Psychomotor Development Index (PDI) score of two other
children were in the marginal level. The MDI and PDI
scores of all the other children were all above medium.
All the children in the control group suffered from mental
retardation. The mental development in the two groups was
significantly different (P , 0:01). Conclusion: Early intervention
can improve the mental development of children
with HIE.
FP-C-014
Study of hypoxia inducible factor 1a (HIF-1a) gene in
neonatal rat models with hypoxic ischemia
X.-R. Zheng, Y.-J. Yang, Y.-J. Jia
Xiang Ya Hospital, Central South University, Changsha,
China
Objective: To explore the expression of hypoxia inducible
factor 1a (HIF-1a) gene in a neonatal rat model of cerebral
hypoxic ischemia (HI), and to evaluate whether the
expression of the HIF-1a gene could be available as a
marker of hypoxic ischemia. Methods: Using a neonatal
rat model of cerebral hypoxic ischemia, the expression of
HIF-1a was detected by employing a method of quantitative
RT-PCR in cerebral tissue. Results: In the normoxic condition
the expression of HIF-1a mRNA in cerebral tissue of
the neonatal rat was at a very low level. Expression of HIF-
1a mRNA occurred at once after HI in the HI group, reached
its peak at 24 h, and maintained a higher level within 72 h,
decreased at 7 days after HI, but was still at a mild higher
level compared with the normal group. Conclusion: These
results demonstrate the expression of HIF-1a increased
rapidly under hypoxia-ischemia in neonatal rats. It was
confirmed that expression of the HIF-1a gene could be
sensitively induced by HI, and it may be available as a
marker of hypoxic ischemia.
FP-C-015
Twenty-four hour non-invasive monitoring of anterior
fontanel pressure and blood pressure in sick neonates
Y.J. Yang, Q.H. Wang, L. Liu, X. Wang
Division of Neonatology, Xiangya Hospital of Central South
University, Changsha, China
Objective: The present study was undertaken to investigate
the method of anterior fontanel pressure (AFP)
measurement to establish the rhythm of AFP and mean
arterial pressure (MAP) over 24 h in sick neonates. Method:
Sixty-seven neonates were divided into four groups: nonintracranial
disease, slight asphyxia (ASP), hyperbilirubinemia
and premature infant. None of the patients received
dehydrated therapy. AFP (intracranial pressure monitor
SP2000) and MAP were monitored continuously over 24
h. Results: (1) The AFP levels in all patients ranged from
1 to 10 mmHg. (2) The AFP was variable over 24 h, the
highest level of AFP was at 09:00–11:00 h, whereas the
lowest was at 01:00–02:00 h. (3) AFP was lower in premature
infants than term infants. (4) There were no correlations
among AFP and weight, size of fontanel, gestational age,
head circumference and MAP. Conclusions: (1) The appreciated
time was at 09:00–11:00 h in 1 day for AFP measurement.
(2) AFP was lower in the premature infants than term
infants. (3) The AFP has increase obviously in neonates
with slight asphyxia, non-intracranial disease or hyperbilirubinema.
FP-C-016
Relationship between hyperbaric oxygen therapy and
apoptosis, caspase-3 in neonatal hypoxic-ischemic brain
damage
Y.-J. Yang, L.-X. Liu, V. Tukei, Y.-J. Jia
Department of pediatrics, XiangYa Hospital, Central South
University, Changsha, China
The objective of this experiment was to investigate the
probable mechanisms of HBO therapy by assessing the
expression of apoptosis and caspase-3 mRNA in a neonatal
HIBD model. Healthy 7-day old SD rats were randomly
divided into three groups: control group (n ¼ 8), HIBD
group (n ¼ 8), and HBO group (n ¼ 8). Detection of the
expression of apoptosis and caspase-3 mRNA was
performed by the TUNEL and hybridization in situ techniques.
Using these techniques the results showed that there
was minimal or no expression of apoptosis and caspase-3

Abstracts 433
mRNA in the control group, but there was massive expression
of apoptosis and caspase-3 mRNA in the HIBD group.
HBO therapy (HBO group) significantly decreased apoptosis
in neuronal cells [(252.96 ^ 68.59) versus
(342.24 ^ 74.16), P , 0:05] and weakened the expression
of caspase-3 mRNA in HIBD rats. The expression of apoptosis
and caspase-3 mRNA was obviously increased in the
HIBD group. We conclude that HBO therapy can weaken
the expression of caspase-3 mRNA and decreases apoptosis
in cells, and this is the probable mechanism of neuronal
protective effect of HBO therapy.
FP-C-017
Change of serum sodium in 78 newborn infants with
hypoxic-ischemic encephalopathy
B. Liu
Department of Infant Medicine Jinzhou Children’s Hospital,
Jinzhou, China
Objective: To study the change of serum Na level in
neonate HIE, and explore the relationship between serum
Na level and severity of HIE. Methods: Serum Na levels
were measured by MI-921 K-Na-Cl Analyzer in 78 neonates
with HIE. Results: Serum Na levels of 70 cases were lower
than the normal value. Serum Na levels of moderate and
severe groups were much lower than that of the mild group.
There were significant differences between the moderate
and severe groups. Conclusion: Serum Na reduced in
neonates with HIE, and the changes of serum Na levels
was related to the severity of HIE.
FP-C-018
Changes of local fibrinolysis in premature newborns
with periventricular leucomalacia
K.S. Ormantaev, L. Saulebekova, D. Kachurina, E.
Gromenko
Scientific Center Pediatrics and Children’s Surgery,
Almaty, Kazakhstan
Severe cerebral ischemia is often accompanied by disturbances
of hemostasis. The aim of our investigation was to
study the tromboplastic and fibrinolitic properties of liquor
in 36 premature newborns with PVL. The mean weight of
the premature newborns was 1400–2000 g. The level of 5-
nucleotidase in liquor was high 2486.0 1 35.0 n/cat, which
indicated the presence of tissue and cerebral thromboplastin.
Production of degradation of fibrinogen (PDF) in liquor was
determined in concentrations from 3.1 to 3.75 mg.%, Severe
cerebral ischemia in premature newborns is accompanied by
withdrawal activators of plasminogen, high fibrinolytic
activity, and appearance of PDF in CSF. This indicates
the development of local fibrinolysis and more severe disorders
of cerebral circulation
FP-C-019
The neurobiology of the normal and brain damaged
infant cry: a comparison of animal, statistical and
neurobiological models
C. Lenti a,b , B.D. Lenti b,c , D. Galbusera a,c , F. Rocca a,c
a Chair of Child Neuropsychiatry, University of Milan,
Milano, Italy; b Laboratory of Bioacoustics and Comparative
Analysis of Behaviour and Cognitive Functions,
University of Milan, Italy;
c Faculty of Paedagogical
Sciences, University of Urbino, Urbino, Italy
The cry of the human infant primate is a differentiated
signal that can be voiceless, partially voiced and voiced
with many different melodic contours. The physioacoustics
of the infant cry, however, is poorly known and we must rely
on animal models, or on statistical elaboration on measured
parameters of recorded and measured infant cries. In this
preliminary work we compare the qualitative and quantitative
parameters of measured on cries produced by six asphyxic
brain-damaged infants with the same parameters obtained
from normal infants, and examine the differences that can
help in understanding the neurobiology of the human cry.
Our preliminary data show alterations of voicing and abnormal
melodic contours: The frequency jump, that is a discontinuity
in the melodic contour is present only in the
asphyxiated subjects and the vibrato is significantly more
frequent in the pathological sample. This indicates a role
for the higher nervous centers in controlling the shape and
continuity of the contours. A surprising fact was that even
with massive lesions, many single cry units appeared similar
to those emitted by normal infants: This could suggest that the
programming of the cry unit’s main characteristics is
controlled by lower centres, possibly related to the respiratory
function, and whose lesions could not be compatible with life.
Results will be compared with animal and statistical models.
FP-C-020
Study of the electroencephalogram in neonatal infants
with fetal distress
C.-J. Yan a , A.-S. Piao a , G.-H. Li b
a Yanbian women-children hospital, Yanji City, Jilin, China;
b New ear children hospital, Yanji city, Jilin, China
Background: Fetal distress is fetal compromise caused by
hypoxia. Fetal distress can cause brain damage, so early
diagnosis and treatment for fetal distress is very important.
The most commonly used tests for fetal distress are the nonstress
test (NST), and the contraction test (CST). Although
the NST and CST have low false-negative rates, both have
high false-positive rates. In this study, we investigated the
value of the electroencephalagraph in fetal distress. Methods:
Twenty-one cases of fetal distress in newborn were
included in this study and 21 normal newborns were
selected for controls. In the study group, the range of gesta-

434
Abstracts
tional age, Apgar score and weight were. A total of 37–42
weeks, 8–10 and 2500–3999 g, respectively. We used the
international 10–20 electrode placement newborn system
and recorded the sleeping EEG by monopolar and bipolar
montage. Results: Index of EEG in fetal distress of
newborns was significantly lower than that of non-fetal
distress newborns (P , 0:05). The results suggested that
pathophysiological changes by hypoxia in fetal distress
could cause brain dysfunction and EEG could detect this
type of disorder. Conclusion: EEG in the newborn is useful
diagnostic method for fetal distress and it is necessary to
check EEGs in newborns at risk to prevent prolonged brain
damage by fetal distress.
FP-C-021
Intracranial hemorrhage in infants after massaging by a
traditional birth attendant
E.S. Herini, Sunartini, S.W. Daniel
Department of Child Health, Medical School, Gadjah Mada
University, Sardjito Hospital, Yogyakarta, Indonesia
The overall incidences of birth injuries appear to be
declining as a result of improved obstetrical practices.
However, it is unknown whether or not incidences of head
trauma were caused by massage of traditional birth attendants.
As a culture in Indonesia especially in Yogyakarta
and Central Java, the infants were massaged after delivery in
order that the baby would be. Despite good intentions,
massaging resulted in intracranial bleeding as if it was
caused by abuse. We report four infants with intracranial
hemorrhage after massaging by traditional birth attendants.
All the infants referred to the Sardjito Hospital, Yogyakarta
were in a bad condition and had severe anemia (hemoglobin
was 6.6, 4.4, 8.6 and 3.7 mg/dl), while the clotting bleeding
times were normal in three infants and the rest had
prolonged clotting time. Head CT scan showed a right hemisphere
cerebral infarct due to intracranial hemorrhage in
case 1, epidural hemorrhage and subdural hemorrhage in
case 2, intracranial hemorrhage in the right temporal parietal
lobe in case 3 and the last case had a subdural hematoma in
the parieto-occipital bilateral region. Three underwent
craniotomy and one was under observation only.
FP-C-022
The effect of hyperbaric oxygen treatment on neonatal
hypoxic-ischemic encephalopathy (HIE)
Q.-H. Yu
Department of Pediatrics, Center Hospital, Qilu Petroleum
Chemical Company, Shandong, China
Objective: To analyze the effect of hyperbaric oxygen
treatment on neonatal hypoxic-ischemic encephalopathy.
Method: The 40 patients with HIE were treated with hyperbaric
oxygen combined with other regular therapy, 30
patients with HIE had only regular therapy. The clinical
features, NBNA scores and cranial CT findings were
recorded during following 3 , 6 months. Result: The
patients treated by hyperbaric oxygen combined with regular
therapy had significant better prognosis than those with
only regular therapy. Conclusion: The hyperbaric oxygen
treatment should be applied to neonatal HIE as soon as
possible for its remarkable effect on improving prognosis.
FP-C-023
Brain stem involvement in neonatal neurological
disorders: ultrasonographic findings and clinical
correlates
Y.-F. Tu, C.-C. Huang
Department of Pediatrics, National Cheng Kung University
Hospital, Tainan City, Taipei, Chinese Taipei
Neurologic disorders with brain stem involvement often
indicate poor prognosis. Conventional cerebral ultrasound
examination through anterior fontanelle is limited in evaluating
the brain stem lesion. There are few reports on other
trans-cranial views that demonstrate the extent of brain stem
lesions. From the year 2000 through 2002, we found 11
newborns with brain stem lesions detected by ultrasound
examinations obtained via squamous portion of temporal
bone and foramen magnum. The etiology included metabolic
encephalopathy (n ¼ 5), hypoxia-ischemia encephalopathy
(n ¼ 4), birth trauma (n ¼ 1) and group B
Streptococcus meningoencephalitis (n ¼ 1). All the
newborns presented with consciousness disturbance, hypotonia,
and respiratory insufficiency that require artificial
ventilation. Trans-axial ultrasound findings showed hyperechogenic
midbrain and/or pons, and the degree of hyperechogenecity
correlated to disease severity. Trans-foramen
magnum examination demonstrated the medulla and cervical
cord lesions, and especially signs of tonsil herniation
(absence of cisterna magna). All patients had a poor
outcome: six died, and the other five have severe neurologic
deficits, including ventilator dependency. We concluded
that cerebral ultrasound examination through temporal
bone and foramen magnum is valuable in defining the
brain stem involvement, which has a prognostic implication
in neonatal neurological disorders.
FP-C-024
The early application of brain-cell metabolism activator
in the newborns with hypoxic-ischemic encephalopathy
(HIE)
B.-J. Liu, P. Zhang, Z.-H. Zhang
Pediatrics department of Laiwu Iron and Steel Group Ltd.
Hospital, Shandong, China
Objective: To explore the effect of the brain-cell metabolism
activator, by the early application of citicoline or

Abstracts 435
cerebrolysin, on the prognosis of the newborns with HIE.
Methods: We selected 108 newborns with HIE who had
been admitted to hospital during the period 1999 , 2001.
We randomly divided them into two groups: the first group
included 51 cases that began to take citicoline or cerebrolysin
from the 7th day after birth. The second group
comprised 57 cases that began to take these drugs before
24 h after birth. These brain-cell metabolism activators
were combined with other routine treatments. There were
no significant differences in the severity of diseases
between the two groups (P . 0:05). The period of treatments
were both 14 , 21 days. The NBNA evaluations and
electroencephalogram examinations were carried out on
the 7th day, 14th day, the 1st month, the 2nd month, the
6th month and the 1st year after birth, respectively. Results:
The rates of unfavorable prognosis were 19.61%, and
5.26% in the first and second groups, respectively. The
differences were significant between the two groups
(P , 0:05). Conclusion: Early application of brain-cell
metabolism activator may decrease the rate of unfavorable
prognosis of newborns with HIE.
FP-C-025
Characteristics of immune status of newborns with
hypoxemic – ischemic encephalopathia
Kh.T. Mukhamedova, F.A. Nazarova
Doctors Advance Training Institute, Tashkent, Uzbekistan
Chronic fetal hypoxia results in thymus involution. At
neonatal asphyxia there accrues blocking of ferments,
responsible for thymosin synthesis, which results in reduction
of activity both of cellular and humoral immunity.
Studies of immune status revealed that specific weight of
lymphocytes in peripheral blood of patients with perinatal
brain injuries ranges within the normal limits with a rising
tendency in children with severe injuries of CNS. In children
with severe brain injures at birth the absolute numbers of
lymphocytes have been increased as well. Conducted studies
of main sub-populations of T- and B-lymphocytes in children
with perinatal brain injures showed redivision of main immunity
cellular factors depending on severity and duration of
hypoxia. Studies of T-lymphocyte content in children who
had had asphyxia revealed much lower readings (indications)
at birth, irrespective of severity and duration of anoxaemia.
Decrease of cellular immunity in newborns who had had
asphyxia indicates an impairment of the main regulating
member of immune reactions and is combined with decrease
of anti-infective resistance of the organism.
FP-C-026
Characteristics of cellular immunity in newborns with
perinatal brain injures
Kh.T. Mukhamedova, F.A. Nazarova
Doctors Advance Training Institute, Tashkent, Uzbekistan
We conducted an immunologic examination of 40
newborns who had had asphyxia and perinatal hypoxia.
The newborns were been divided into two groups: The
first group was composed of newborns whose condition
was mild to severe, who had had chronic hypoxia due to
the mother’s chronic diseases, and complicated pregnancy.
The second group was composed of newborns who had had
acute asphyxia with combined hypoxia. Studies of subpopulations
of T-lymphocytes/T-helpers, T-suppressors/in
children with perinatal brain injures revealed a decrease of
relative and absolute number of T-lymphocytes, suppressors
and helpers. The findings indicate that perinatal brain injury
leads to substantial impairment of the newborns’ organism
immunologic reaction to imbalance of the immunity cellular
basis, which depends on the severity of the patient’s condition,
age, duration of anoxaemia, state of health and course
of mother’s pregnancy. A severe form of combined hypoxia
results in more acute impairment of cellular immunity,
which makes this group potentially threatening regarding
sepsis and development of acute neurology symptoms.
FP-C-027
Hypoxia-ischemia induced upregulation of brain iron
histochemistry in neonatal SD rats
M.-Y. Hei, S.-J. Kuang
Department of Pediatrics, the 3rd Affiliated Xiangya Hospital
of Central South University, Changsha, Hunan, China
The developing characters of the neonatal brain make
them vulnerable to multiple types of injury, including
hypoxic/ischemic injury. The over-accumulation of iron in
the brain may lead to free radical-caused cell damage after
HI insult. Objective: To understand the histochemical
change of iron in the brain tissue in both normal and HI
insulted neonatal SD rats. Method: 30 neonatal SD rats
were randomly divided into normal group (n ¼ 15) (including
postnatal days 7, 8, 10, 14 and 21 subgroups) and HIE
group (n ¼ 15) (postnatal day 7 rats treated with right carotid
artery ligation combined with 2.5 h 8% O 2 /92% N 2 exposure).
After 12, 24 h, 3, 7 and 14 days of recovery, the HIinsulted
animals were perfusion fixed. A total of 45 mm
thick free-floating sections were stained in Perl’s solution
and intensified by DAB/H 2 O 2 . Results: In normal P7–P14
SD rats, there were iron-positive cell foci mainly distributed
in cingulum and corpus callosum, periventricular zone,
internal capsule and the tip of the external capsule. The
iron positive cells were morphologically similar to oligodendrocytes
and microglia. The iron staining intensity of
ipsilateral hemisphere in the HI insulted brain was increased
as early as 12 h after HI and progressed in a time-dependent
pattern with the peak at 7 days after HI insult, especially in
the damaged cortex, internal capsule and hippocampus. The
iron-positive material was mainly glia. An intensified
phenomenon of perivascular iron positive foci was particularly
noticeable. Conclusions: Brain iron histochemistry

436
Abstracts
was upregulated after HI-insult and can be used to assess
cerebral damage in moderate to late stages after hypoxic
ischemia insult.
FP-C-028
Effect of early intervention on the brain and
development quotient of newborns with hypoxic
ischemic encephalopathy
D.-Y. Zhou, M. Peng, S.-Y. Jiang
Harbin Children’s Hospital, Daoli District Harbin, Harbin,
China
Objective: To explore the efficacy of early intervention on
newborns suffering from hypoxic ischemic encephalopathy
(HIE), and evaluate their prognosis. Method: One hundred
newborns suffering from neonatal HIE (states of II, II–III,
III) who received early intervention were followed-up for 2
years. CT and DQ were performed in these cases. Results:
(1) An early intervention was carried out for improving the
prognosis of the newborns with HIE (II, II–III) The normal
rate of their brain CT and DQ in the early intervention group
was higher than that of the non-intervention group. This
difference was statistically significant, P , 0:01. There
were significant differences in neonatal HIE (III),
P , 0:05. (2) The follow-up examination showed a high
correlation between the brain CT and DQ in the early intervention
group, r ¼ 0:88. The brain CT in the early intervention
group was improved, accompanied by the improvement
of clinical signs (DQ level). Meanwhile, the low correlation
between the brain CT and DQ in the non-intervention group,
r ¼ 0:55. Conclusion: Clearly early intervention can
improve the prognosis of neonatal HIE. CT integrated
with DQ can improve accuracy and early diagnosis.
FP-C-029
P-selectin expression and neuroprotective effect of
fucoidin on hypoxic-ischemic brain injury in neonatal
rats
B.-L. Eun a , K.-B. Kim b , D.-H. Pee a , Y.-C. Tockgo a
Department of Pediatrics, College of Medicine,
a Korea
University, b Kwandong University, Seoul, Korea
Objective: Leukocyte-endothelial adhesion is a key step
in the initiation of post-ischemic reperfusion injury in many
organ systems. We hypothesized that P-selectin might
mediate post-HI injury in the immature rat brain. Methods:
To elicit focal hypoxic-ischemic brain injury, 7-day-old
(P7) rats (n ¼ 160) underwent right carotid coagulation,
followed by 2 h hypoxia (F i O 2 ¼ 0.08). We performed
RT-PCR for mRNA (n ¼ 28) and Western blot for protein
of P-selectin (n ¼ 37). To test the efficacy of a pretreatment
regimen, P7 rats (n ¼ 59) received fucoidin immediately
before and again after hypoxia intra-peritoneally with a 26
gauge needle. Results: P-selectin mRNA expression in the
ipsilateral (right) hemisphere reached a peak at 8 h after HI
and then was barely detected after 24 h. P-selectin protein
was detected as early as 15 and 30 min at both hemispheres
in experimental rats and decreased at 1 h, and increased in
the right hemisphere at 4 h post-HI, peaked at 8 h and was no
longer detectable at 24 h. We found a substantial neuroprotective
effect after pretreatment with fucoidin as a dose
dependency. Conclusion: The temporal profiles of post-HI
P-selectin mRNA and protein expression are consistent with
a role in the evolution of subsequent brain injury. Potential
of therapeutic application of P-selectin blockade or similar
anti-adhesion strategies may reduce the brain damage after
perinatal HI.
FP-C-030
Clinical analysis of 86 neonatal hypoxic ischemia
encephalopathy
A.-Q. Li
The department of Pediatrics, Weihai Municipal hospital,
Huancui district Weihai, Shandong, China
Objective: To investigate the relationship between the
clinical degree of severity of HIE with asphyxia and the
cranial image changes. Method: The data of 86 neonates
diagnosed with HIE were analysed retrospectively. Seventy
five cases had at least once undergone cranial CT examinations
between the age of 2 and 19 days. Result: Among 86
subjects, including 54 male cases and 32 females, 58 cases
diagnosed with mild HIE included 20 cases of mild
asphyxia, and 38 cases of severe asphyxia. Among 17
cases of moderate HIE, one case had mild asphyxia and
16 cases were severe. In 11 cases of severe HIE all had
severe asphyxia. In 75 cases with cranial CT, 58 cases
showed abnormal, including 31 cases of mild pathological
changes, 16 had moderate change, and 11 cases of severe
changes. Among the cases with severe changes in CT scan,
there were three with parenchymal hemorrhage and three
with subarachnoid hemorrhage. The positive rate of CT in
mild and moderate HIE were 64.7 and 84%, respectively. In
severe HIE, all patients had apparently abnormal changes.
The patients diagnosed as mild HIE with mild asphyxia
were usually presented 36 h after birth. The clinical symptoms
of patients with the brain lesion mainly in the occipital
lobe were staring, lethargy, etc., usually not affecting eating.
If the lesion in anterior frontal or temporal lobes, the
patients presented irritability, bulge of fontanel, and many
of them had convulsions. Some had apnea or stubborn
hiccups. The common drugs used in HIE, included vitamin
K, sodium bicarbonate, luminal, antibiotics, ATP, citicoline,
and dehydrating agents. The effect of using dehydrating
agents regularly was better than using them provisionally.
Conclusion: This observation showed the more severe the
asphyxia the more the severity of the state of the patients. It
was important to start treatment before the hypoxemia and
ischemia occurred, and after resuscitation of asphyxia.

Abstracts 437
Additionally, the cooperation of pediatricians and obstetricians
in treatment was imperative to improve the prognosis
of the patients with asphyxia or HIE.
FP-C-031
Neonatal choroid plexus cysts (CPC) and early
childhood developmental outcome
K.-L. Hung, H.-T. Liao
Department of Pediatrics, Cathay General Hospital, Taipei,
China
Choroid plexus cysts (CPCs) are incidental findings on
sonograms of the neonatal head. The true incidence is not
yet known. Childhood neurodevelopmental outcome associated
with neonatal CPCs has been rarely reported. The aim
of this study was to determine the incidence of neonatal
CPCs and early childhood developmental outcome. During
the period July 1997 to June 1998, 2111 normal newborns
underwent brain sonographic examinations as a routine
screening. All newborns detected as having CPCs underwent
serial sonograms at 1, 2, 4 and 6 months of age. Developmental
milestones were evaluated subsequently at followup
visits. Developmental assessment was performed with
the Denver II Developmental Screening Test. The results
showed CPCs were identified in 186 neonates (8.8%). Of
these, 14 (7.5%) were bilateral. The mean size of the cysts
was 2.6 mm (range 1.1 , 8.6). Five cysts were larger than 5
mm in size. Physical check-up was normal in all the 186
neonates with CPCs follow-up ultrasonic studies were available
for 155 children. The cysts regressed spontaneously in
the majority of the infants and, by 6 months of age, residual
cysts were visible in 18 infants (11.6%) only. During the
clinical follow-up period from 30 to 42 months, developmental
outcome was normal in all available 179 children.
The existence of isolated CPCs in the newborn is neither
associated with abnormal physical findings nor with any
delay of early childhood development.
FP-C-032
Protective and therapeutic effects of valproic acid
against hypoxic-ischemic brain injury in the newborn
N. Kabakus, I. Ay, S. Aysun, F. Soylemezoglu, A. Ozcan, B.
Celasun
Pharmacology and Neuropathology, Departments of Pediatric
Neurology, Hacettepe University Faculty of Medicine,
Ankara, Turkey
HIBD is still an important cause for morbidity and
mortality in the newborn, and new approaches need to be
developed for the treatment of HIBD. The present study
aims to investigate neuroprotective/neurotherapeutic
effects of valproic acid (VPA) which is reported to be a
protective and therapeutic agent in cellular damage. VPA
was administered acutely (first 24 h) and chronically (after
24 h) at both low (200 mg/kg per day) and high (400 mg/kg
per day) therapeutic doses. All experiments were
performed on 7-day-old rat pups which is an immature
rat model. The findings have shown that VPA had no
significant therapeutic effect on cerebral infarcts
(P . 0:05) following both acute and chronic administration.
However, when administered for five consecutive
days, VPA had significant protective and therapeutic
effects on the area of cerebral infarct in a dose-dependent
manner (control: 17.9%; low dose: 11.2%; high dose:
7.9%). High doses of VPA within the first 24 h showed
therapeutic effects on the patchy lesions in the thalamus,
caudate nucleus and putamen (P , 0:05). VPA protected
the brain from possible deterioration of patchy and cortical
(cerebral and hippocampal) damages into more severe
forms (grades 3–4/4). VPA also prevented progression of
pronounced atrophy in the ipsilateral hemisphere (,75%).
In view of the present results it is concluded that therapeutic
properties of VPA in HIBD need to be elucidated in
more comprehensive studies.
FP-C-033
The effect of intravenous immunoglobulin in hypoxicischemic
brain damage in an experimental immature rat
model
G. Haliloglu, P. Atilla, M. Berker, B. Anlar, A.N. Cakar, S.
Aysun
Histology and Embryology and Neurossurgery, Departments
of Pediatric Neurology, Hacettepe University Faculty
of Medicine, Ankara, Turkey
Immunoinflammatory system and cytokines activate a
biochemical cascade of events after hypoxia-ischemia.
Effects of intravenous immunoglobulin as cytokine suppression
and remyelination have been reported in human tissue
cultures and demyelinating diseases. The aim was to study
these effects in histopathological findings in hypoxia-ischemia
immature rat model. Hypoxic-ischemia was induced in
7-day-old rats (right common carotid artery occlusion 18%
O 2 for 2.5 h). The animals were then divided into two groups:
first group received 1 g/kg IVIg intraperitoneally, and the
second group received the same amount of serum physiologic
within 1 h after the insult. The animals were decapitated
after 24 h, and a morphologic classification was done using
vacuolization, clumping of nuclear chromatin, disappearance
of nuclear depression and nucleus pyknosis from both
hemispheres and frontal, temporal and occipital regions
separately. It was demonstrated that vacuolization and
nuclear pyknosis were decreased in the inner cortex in the
first group. Pyknotic changes seen in the outer cortex in both
groups represented cells in the process of apoptosis,
supported by TUNEL method. This study demonstrated the
morphologic effects of IVIg on hypoxic-ischemic encephalopathy
in an immature rat model, and forms a base for
further studies.

438
Abstracts
FP-C-034
Brainstem evoked response in term neonates
L.M.F.F. Guilhoto, E.A. Abreu, E. Ornelas, V.S. Quintal,
M.T.Z. da Costa
Hospital Universitário da Universidade de São Paulo, São
Paulo, Brazil
Brainstem evoked response (BSER) in neonates may
evaluate objectively the integrity of the auditory system
up to the brainstem level. It seems to be a reliable test
for auditory and neurological dysfunction at this age and
it permits early diagnosis and rehabilitation. The purpose
of this study is to demonstrate the latencies of BSER in
normal term neonates in order to obtain normative data in a
university hospital. BSER was performed on the 2nd day of
life of 47 normal newborns (25 males, 22 females) ranging
in gestational ages between 37 and 40 weeks that did not
present with familial history of deafness. The examination
was performed during sleep, after feedings, with 80 dBHL
clicks of alternating polarity presented monaurally at a rate
of 10/s. A total of 2.000 stimulus trials was averaged and
duplicated for each ear. The mean latency time of waves I–
V, as well as the inter-peak latencies were measured. The
mean of the latencies in milliseconds was the following:
wave I, 1.79 (SD 0.20); wave II, 2.88 (SD 0.28); wave III,
4.54 (SD 0.31); wave IV, 5.86 (SD 0.36); wave V, 6.75
(SD 0.38); IPL I–III, 2.75 (SD 0.36); IPL III–V, 2.22 (SD
0.22); and finally IPL I–V, 4.97 (SD 0.43). Normative
BSER studies in term neonates undertaken in university
hospitals are necessary to establish reference values for
evaluation of auditory and neurologic prognostic factors,
as well as to early diagnose children with auditory dysfunction.
FP-C-035
Guidelines for intensive care in the delivery room of
extremely premature newborns
G. Verlato, D. Gobber, D. Drago, L. Chiandetti, P. Drigo,
B.M. Amelia and the Working Group
Intensive Care in the Delivery Room of the Bioethic
Committee of the Department of Pediatrics; Department
of Pediatrics, University of Padua, Padova, Italy
Ethical problems related to neonatal intensive care of
extremely preterm newborns, #25 weeks gestational age
(GA) and at risk of disability are extensively debated; withholding
intensive care is considered appropriate for
newborns of GA ,23 weeks and of birth weight ,400 g
(International Guidelines for Neonatal Resuscitation,
Pediatrics 2000), but the importance of local and national
guidelines is underlined. The Bioethical Committee of our
Department promoted a multidisciplinary group to release
guidelines to help the staff in charge of neonatal intensive
care facing the medical and ethical problems related to an
extremely premature birth. The group comprised nurses,
physicians, ethicists and lawyers. The vitality limit, survival,
outcome and the ethical and legal aspects were
analysed according to the literature and the local context.
As a result, a document was produced and distributed to all
the health care professionals involved. Summary of guidelines:
we suggest at 222 GA, defined by the anamnesis and
the clinical evaluation of the neonate, to provide compassionate
care only; 223 GA, in the presence of detectable
vital signs, to practice immediate intubation, respiratory
support and to revaluate neonatal conditions; from 24
GA, to provide intubation, ventilatory support and cardiovascular
resuscitation. If clinical and anamnestic GA are
different, we proceed according to the best one. The importance
of correct information to parents, and the role of
compassionate care are outlined. An annual audit of guidelines
is considered mandatory.
FP-C-036
Parasagittal cerebral lesions in full-term newborns
F. Ciardo, E. Della Giustina, G. Cioni, M. Verdecchia, P.
Curatolo
Pediatric Neurology, Tor Vergata University of Rome,
Rome, Italy
Parasagittal cerebral injury (PCI) is a specific pathologic
lesion of full-term newborns suffering from hypoxicischemic
encephalopathy. PCI is characterized by necrosis
of the cortex (most vulnerable in the term infant), and close
subadjacent white-matter, especially in the parieto-occipital
areas of the brain; neuronal elements are most severely
affected. The injury is bilateral and although usually
symmetrical, may be more striking in one hemisphere
than the other. The spectrum of PCI is, however, wide,
including frequent symmetric involvement of basal ganglia
and thalami. Because of its unclear pathogenetic mechanism,
we have studied the clinical and neuroradiological
follow-up of four patients suffering from fetal distress, in
whom MRI scans had clearly demonstrated in vivo the
presence of parasagittal lesions due to perinatal hypoxicischemic
insults. We noted that: (1) the pattern of injury
seen on MRI seems to be related to the type and severity of
the insult, and very strongly to the gestational age at which
the insult occurred; (2) severe acute asphyxia is associated
with lesions in the basal ganglia, thalami, brainstem, hippocampus
and the corticospinal tracts around the central
fissure; (3) the infants were affected by severe white
matter, severe basal ganglia and thalamic injury, which
may have been caused by a more severe perinatal insult
occurring in combination with a chronic and repetitive
insult that involves the white matter; and (4) in the absence
of basal ganglia lesions, the motor outcome may be surprisingly
good even with extensive white matter loss.

Abstracts 439
FP-C-037
Early intervention promotes intellectual development in
asphyxiated newborn and premature infants
X.-L. Bao, S.-Y. Sun, R.-J. Yu, J.-T. Sun, S.-Z. Wei
Intervention of Asphyxiated Newborn and Premature
Infants Cooperative Research Group Pediatric department,
Peking Union Medical College Hospital, Chinese Academy
of Medical Sciences, Beijing, China
Objective: To evaluate the effect of early intervention on
the intellectual development in asphyxiated newborn and
premature infants. Methods: 119 cases of full term
asphyxiated newborn infants (Apgar score ,6 at 5 min
after birth) and 104 premature infants (gestational age ,37
weeks) were randomly assigned to early intervention group
and conventional care group. The normal control group
consisted of same number infants. Sex, mother’s educational
background; environmental condition and physical development
were not significantly different in above groups. A total
of 0–2 years early intervention program was compiled on
basis of the national and foreign material about 1 month
ahead of average development of the child. It included
motor, cognitive, speech development and social behavior.
Parents were instructed to carry out early intervention. At the
age of 1.5 and 2 years. All infants received infant development
tests (CDCC). The examiner did not know which infant
had received intervention. Results: At the age of 1.5 years,
average score of MDI in early intervention groups of
asphyxiated and premature infants were 14.6 and 13.8 higher
than that in conventional care groups respectively
(P , 0:01). MDI score in early intervention group caught
up with that in normal control group (P . 0:05). Conventional
care group were 9.7 and 11.5 lower than normal control
group, respectively (P , 0:01). There were 9 and 7.8%
infants in conventional care group mentally retarded while
none were mentally retarded in the early intervention group.
Conclusions: The results showed that the early intervention
could promote intellectual development of asphyxiated and
premature infants and it may be beneficial to the prevention
of mental retardation. Early and intensive intervention can
produce better results. Bringing parent’s initiative into full
play through deepening their understanding of the importance
of early intervention is the key to success.
FP-C-038
Protective effect of ligustrazine on hypoxic-ischemic
encephalopathy by NBNA
R. Zhu, D.-D. Zhuo
Zhong Lan Hospitol, WuHan University, WuHan, China
Purpose: To observe the protective effect of ligustrazine
on hypoxic-ischemic encephalopathy (HIE) by NBNA.
Methods: Sixty cases of HIE were enrolled and divided
randomly into two groups. The treatment course was 10
days. The change score of NBNA 20 were observed before
and after treatment. Thirty healthy cases were taken for
control. Results: The score of NBNA 20 in the tested
group was significantly (P , 0:01) higher than in the
control group. Conclusion: Ligustrazine has protection on
HIE, is a good Chinese drug for HIE in clinical practice.
FP-C-039
A study of the protective effect of magnesium sulfate on
the brain injury of hypoxia and ischemia in newborns
with birth asphyxia
F.-L. Zhao, Y. Tang
Department of Pediatrics, The Third Hospital of Beijing
University, Beijing, China
Objective: Perinatal cerebral hypoxia-ischemia remains a
frequent cause of disablement and death of newborn. The
aim of this study was to investigate the protective effect and
security of magnesium sulfate on the brain injury of hypoxia
and ischemia in newborn with birth asphyxia. Methods:
Thirty-nine mature newborns with birth asphyxia were
divided randomly into two groups: therapy and control.
The first group received magnesium sulfate infusions
comprising a loading dose of 200 mg/kg, followed by two
doses of 125 mg/kg at 24 and 48 h later. The occurrence
ratio of HIE, the difference of clinical index and the
instances of side effect were compared between the 2 groups
after the intervention. Results: (1) There was no significant
difference in the occurrence ratio of hypoxic ischemic encephalopathy,
EEG, CT and NBNA scores between the two
groups (P . 0:05), whereas the ratio of serious encephalopathy
in the therapy group was lower (P , 0:05). (2) No
serious side effect was found in the therapy group. Conclusion:
(1) Magnesium sulfate protected hypoxic-ischemic
brain injury in the newborn with birth asphyxia to a certain
extent. (2) The infusion doses were not associated with
serious side effects.
FP-C-040
The relationship among umbilical cord serum
interleukin-6, brain damage in the preterm newborn and
the neurologic prognosis
C.-X. Du, F.-L. Zhao
Department of Pediatrics, Third Hospital of Peking University,
Beijing, China
Objective: Brain damage in the preterm newborn is a
major risk of neonatal death and is often associated with a
variety of neurologic deficits in survivors, including CP.
Recently, the roles of maternal choriamnionitis and cytokine
produced in response to maternal infection in the
pathogenesis of preterm birth and neonatal brain damage
have become a major focus of attention. The purpose of
this study is to examine the relationship among neonatal

440
Abstracts
brain damage, maternal choriamnionitis and umbilical cord
serum interleukin-6 (IL-6) concentration, to evaluate the
clinical significance of examining umbilical cord serum
IL-6 concentration, and to provide a new method for diagnosing
of brain damage in the preterm neonate and preventing
neurologic deficits. Methods: The objects of this study
were 31 preterms. The procedure was: (1) the umbilical cord
serum IL-6 concentration of neonates was detected by
ELISA; (2) maternal choriamnionitis was determined by
histologic examination; (3) brain damage was diagnosed
by ultrasonography or CT in the first 3 days; and (4) following
up the neurologic development of these preterms within
3 years after their birth. Results: (1) Five preterms failed to
be followed up, and the loss rate was 16%. The incidence of
neurologic deficits was higher in preterms with brain
damage than in those without brain damage; (2) the incidence
of brain damage and the incidence of neurologic deficits
were higher in preterms whose mothers had
choriamnionitis than in those without maternal infection;
(3) umbilical cord serum IL-6 concentrations of preterm
newborns were higher in preterms with brain damage and
those with neurologic deficits; (4) among the preterm
neonates with brain damage, cord serum IL-6 levels were
significantly higher in the presence of choriamnionitis than
in the absence of choriamnionitis; and (5) using the results
of brain imaging examination as the standard, a cord serum
IL-6 level of $1.20 ng/ml was 80.0% sensitive and 75.0%
specific for identification of the brain damage associated
with maternal infection. Conclusions: These results show
that maternal chorioamnionitis associates with an increased
incidence of brain damage in preterm newborns. So it is
very important to prevent and treat maternal intrauterine
infection early in order to prevent the occurrence of brain
damage in preterm neonates and decrease the risk of neurologic
deficits like CP. IL-6 in umibilical cord serum might
be the link between maternal infection and neonatal
damage. These results provide a new practicable method
for preventing and diagnosing of brain damage in preterm
newborns and preventing of neurologic deficits. An increase
in umbilical cord serum level is a good index for brain
damage in preterm newborns. There is a clinical significance
to examine IL-6 concentration in umbilical cord
plasma of neonates.
FP-C-041
Long-term prognosis of convulsions in the newborn
L. Wei, C.-X. Du
The Third Hospital, Peking University, Beijing, China
Objective: To evaluate long-term prognosis in neonates
with convulsions and related factors. Methods: Fifty-six
infants with convulsions were studied. All of them were
subjected to systemic physical examination and intelligence
test, and some of them underwent CT and EEG
examinations. The relationship between prognosis, situation
of perinatal stage and convulsions was analyzed.
Results: CP developed in four infants, and five infants
(including two infants with CP) suffered from epilepsy.
Twelve infants had low IQ (three infants with CP and
one infant with epilepsy). In all infants, 15 infants had
nervous system sequelae. The rate of sequelae was 27%.
Neonatal convulsions caused by hypoxic-ischemic encephalopathy
were significantly different from those caused
by other factors. Additionally, prognosis was highly correlated
with convulsion styles, the number of seizure times
and seizure duration. Conclusion: A high correlation exists
between prognosis and etiology of neonatal convulsions.
FP-D
Neurodevelopmental Neurology
FP-D-001
Organising logopedic therapy for babies with unilateral
or bilateral brain lesion in the pre-speech period
V. Gec
Centre for Hearing and Speech, Maribor, Slovenia
Therapy was carried out in an experimental group of 30
babies in the pre-speech period and suffering from perinatal
unilateral or bilateral lesion at the age of 6–24 months.
The control group comprised 60 babies. Therapy is made
possible by early detection of factors of risk in the earliest
period of the child’s life. Important are: (1) function of
sucking, swallowing and later chewing in the course of
the feeding therapy; and it is also important to (2) improve
proprioceptive findings regarding speech musculature by
means of passive-active motor exercises, to develop a
correct breathing function and vocal activities. Simultaneously
with the simulation of articulation, phonation,
breathing in speech and phonematic hearing, attention
should also be given to the stimulation of speech kinaesthetics,
kinaesthetic observation and the kinaesthetic
scheme of speech musculature. After intensive medical
treatment, therapy is continued in the child’s home environment
guided by a speech therapist, but carried out by the
baby’s parents and surrounding. Based on existing knowledge
and personal experience we examined the basic
elements of logopedic diagnostics, therapy and stimulation,
which were classified into categories and applied to individual
patients, depending on their abilities. The data from
the reports and responses in the logopedic dispensary were
inserted into the protocol of the new-born at risk and the
protocol I had formed to meet the demands of this study.
Both protocols are related and include all the basic phases
of logopedic diagnostics and the results of treatment to 24
months of age. The protocols and the rest present a
complete medical-logopedic treatment and development
of the newborn at risk.

Abstracts 441
FP-D-002
An analysis of electroencephalographic characteristics
in 68 young infants and children with cerebral palsy
S.-M. Li, H.-C. Zheng, X.-G. Li
HeBei Children Hospital, Shijiazhuang, China
Objectives: To study the electroencephalographic characteristics
in young infants and children with cerebral palsy.
Methods: A total of 68 patients (45 boys, 23 girls) aged 4
months–6 years (mean 1 year 5 months) with cerebral palsy
was investigated. They all had clinical evaluation and routine
electroencephalographic studies. Results: electroencephalographs
are normal in 29 patients and abnormal in thirty-nine
with an abnormality rate of 57.4%. In those with abnormal
EEG findings 12 showed focal abnormality and 27 showed
widespread abnormality. All focal abnormal electroencephalographsshowedfocalepileptiformdischarges.Inwidespread
abnormal electroencephalographs, diffuse slow wave was
noted in 12 patients (three of them associated with focal
epileptiform discharges). Hypsarrhythmia was noted in ten
patients (one of them associated with extreme spindles),
with mental retardation in all. Depression of normal rhythms
wasnotedinthreepatients,leapingoutofbackgroundrhythms
and suppression burst of a rhythms was noted in two patients.
The abnormality rate of electroencephalographs in patients
with normal mental faculties is 29.4%. The rate of abnormal
EEG in those with delayed mental function is 83.3%. Conclusions:
The abnormality rate of electroencephalographs is high
in patients with mental retardation. Electroencephalography
might have an important role in the clinical predictions and
provide more objective indexes for the evaluation of brain
function in patients with cerebral palsy
FP-D-003
The etiologies analysis of 292 cerebral palsy children
M.-L. Liu, W.-Y. Wang, J.-Y. Yan
Department of Pediatrics, Affiliated Hospital of Medical
College of Chinese People’s Armed Police Force; Center
of Handicapped Children Recuperation, Tianjin, China
We had analyzed the etiologies of 292 cases diagnosed
cerebral palsy. Result: Among many pathogenic factors
related to cerebral palsy, premature delivery and neonatal
asphyxia was the most common. They accounted for about
49.96% cases. Conclusion: To strengthen perinatal period
care, prevent premature delivery and perinatal asphyxia was
the key to decease occurrence of cerebral palsy.
FP-D-004
Cerebral palsy in 1–6 years old children in the Leshan
area of Sichuan province: an epidemiological survey
Y.-Q. Zhong, W. Ju, D.-Z. Peng
Cheng Du Children Hospital, Cheng Du, China
Objective: To describe the epidemiological features of
childhood CP in Leshan area. Method: A cross-sectional
survey on the prevalence of CP in 1–6 years old children
was conducted using cluster sampling from eight Counties
or Districts of Leshan area. Results: The survey was done
among 148 723 children from the sampled locations and a
CP prevalence of 2.07‰ was found. The results showed that
among CP children there ware more male than female. There
were also more premature and post-term infants than term
infants, with the increased prevalence 22.21 and 5.4 times in
the former than the later, respectively. The prevalence of CP
in low birth weight infants was 16.32 times to that in normal
birth weight infants. Twin infants suffered CP 4.2 times more
frequently than single-born infants. Conclusion: CP prevalence
in 1–6 years old children in Leshan area and the epidemiological
features are comparable to those in advanced
countries. Better recognition of and improvement in gestational
and perinatal health care should be a critical way to
reduce the occurrence of CP in children.
FP-D-005
The diagnosis and outcome of cerebral palsy: a clinical
and neuroimaging study
B.-N. Zhan
Department of Pediatrics, Tongji Hospital, Tongji Medical
University, Huazhong; University of Science and Technology,
Wuhan, China
Objective: To explore the etiology, methods of early diagnosis,
and management of cerebral palsy. Method: clinical
data analysis, EEG and neuroimaging study (CT, MRI).
Results: Complete clinical data of 51 cases of cerebral palsy
were analyzed. Among them 20 cases had birth asphyxia, 20
cases were with positive history of maternal perinatal
diseases, 12 cases were premature/low-birth-weight/smallfor-gestational
infants. All 51 cases manifested different
extent of mental retardation and neuromotor dysfunction in
the follow-up, while the most common type of disability was
spasticdiplegia (40cases, 78.4%).Neuroimaging study for all
the cases (CT and/or MRI) revealed an 84.3% (43/51) of
abnormality, with brain atrophy as the most frequent finding
(34/51, 66.7%). A comprehensive management strategy was
adopted in the clinic. Among the 16 cases with long-term
follow-up, four (25%) had significant psychomotor improvement
and 12 cases (75%) showed moderate improvement.
FP-D-006
Electrophysiological study of deep coma and its outcome
in children
Yutaka Tomita a , Sachiko Fukuda b
a Department of Pathological Science and Technology,
School of Health Science, Yoshihiro Maegaki; b Department
of Pediatric Neurology, Faculty of Medicine, Tottori
University, Yonago, Japan

442
Abstracts
One of the major clinical features of brain death is deep
coma. Therefore, we conducted an electrophysiological
study of 31 children who had suffered from deep coma in
order to investigate its pathophysiological characteristics and
outcome. The patient age at coma ranged from 1 month to 10
years (mean 2 years 1 months). We compared results between
fair and poor prognostic groups. Subsequently, lack of P14,
N18 and N20, and amplitude reduction or vagueness of P13
in short-latency SSEPs in these comatose children strongly
suggested high risk in their future prognosis. SSEPs revealed
findings for a deep coma condition more specific than ABR
and blink reflex. In conclusion: SSEPs might be a very sensitive
and specific examination for predicting the long-term
prognosis of deep coma in children.
FP-D-007
Development of children born to drug-dependent
mothers
E. Berger a , Th. Elstner a , S. Fiala-Preinsperger a , B. Schuch b ,
G. Fischer c , M. Weninger d , G. Langer e
a Department for Neuropsychiatry of Children and Adolescents,
Neurological Hospital Rosenhuegel, Vienna; b Clinic
for Neuropsychiatry of Children and Adolescents, University
of Vienna; c Clinic for Psychiatry, Outpatient Dep. For
Drug Abuser, University of Vienna; d Clinic for Paediatric
and Adolescent Medicine, University of Vienna; e Clinic for
Gynaecology and Obstetrics, University of Vienna, Austria
Since 1995 we observed in a prospective longitudinal study
thedevelopment of130children (between 6weeksand6years
of age) of drug dependent mothers. We must take into account
a combination of different risks: unspecific and drug-specific
biological risk as the root of neurological risk and multiple
psychosocial risks. We designed the follow-up in a biopsycho-social
frame: The combination of comprehensive
care with clinical evaluation – in a ‘network of helpers’
(drug care, obstetric care, neonatologic care, developmental
care and psychosocial care). We define the risk on different
levels by the following test-battery: pre- and perinatal risk:
drug levels in urine, dosis range of drug-substitute, intrauterine
dystrophy, prematurity; postnatal risk: intensity(Finegan-
Score) and duration of symptoms of detoxication; developmental
parameters: neurological investigations (Prechtl,
Beintema), general movements (Prechtl), sensorimotor and
cognitive investigations (KABC-Scale), Parent Infant Global
Assessment Scale (PIRGAS), Zero to three – diagnosis, child
behavior problem lists (CBCL). Preliminary results: 95 children
(born between march 1995 to November 1999) are
included for evaluation. The biological risks (neurological
development) are very low, if the drug substitution is correct;
the risks are increasing by additional use of benzodiazepines
and cocain. The psychosocial risks are increased: 35% of relationship
disorders and 20% of psychopathological disorders
in the first 3 years. Summarising we can say, that the approach
of comprehensive care can minimise the developmental risks.
FP-D-008
Investigation on the pathogenic factors and clinical
features of cerebral palsy in Shenzhen
L. Chen, J.-X. Liao, B. Li, T.-S. Huang
Epilepsy Center and Department of Pediatric Neurology,
Shenzhen Children’s Hospital and Shenzhen Institute of
Pediatrics, Shenzhen, China
Objective: To explore the pathogenic factors and clinical
features of CP in Shenzhen. Methods: To analyze retrospectively
CP patients’ clinical data in the outpatients department
of Shenzhen Children’s Hospital. Results: A total of
110 cases were investigated. Thirty two had premature birth
history, 44 had low birth weight (less than 3000 g) and 12
had congenital brain malformations, and 15 had perinatal
asphyxia history. Most patients with premature birth or low
birth weight history developed spastic CP and had the cerebral
periventricular leukomalacia tested by computerized
scanning in neuropathology. Most patients with congenital
malformations had small head circumference (range 39–
44.5 cm, median 43 cm, n ¼ 9) and mental retardation.
Conclusion: The common pathogenic factors of CP in
Shenzhen are premature birth, low birth weight and congenital
brain malformations. Asphyxia is now less important
as a pathogenic factor than before. Periventricular leukomalacia
is a common neuropathologic lesion.
FP-D-009
Pre-placement neurodevelopmental screening in
international adoption: key to improved outcome
G.W. Diamond a,b , Y. Senecky a , D. Inbar a,b , M. Nas c , H.J.
Cohen b
a Center for Child Development, Schneider Children’s Medical
Center, Petah Tiqva, Israel; b CERC, Albert Einstein
College of Medicine, Bronx, NY, USA; c Fundatie Prima
Generatie, Bucuresti, Romania
Background: Young, institutionalized children, adopted
from abroad, constitute a high risk, special needs population.
Those who underwent pre-adoption comprehensive
medical and neurodevelopmental testing demonstrated
improved adjustment to their new home environment and
were treated earlier for identified medical and neurodevelopmental
impairments. Objective: To determine the character
and extent of medical and neurodevelopmental
impairments most prevalent among a cohort of young children
who were candidates for adoption in East European
orphanages in order to provide improved treatment and
support services for them and their adoptive families
upon arrival in their new homes. Surveyed also were
those disabilities militating against adoption of select children.
Design: Sixty two East European children, age 3
months–4.5 years (median age: 11 months) were screened
by physicians while still in their original residential

Abstracts 443
settings. Evaluations comprised examinations using standardized
assessment tools, and laboratory analysis of
blood samples, including genetic screening. Results:
Eleven children (18%) were not placed in adoptive
homes abroad due to significant medical illness or developmental
delays. These included hepatitis B and C infection,
MR, CP, and positive family psychiatric history.
Parents reported greater satisfaction with the pre-adoption
method of screening.
FP-D-010
Exploration study to find out increased risk of later
developmental delay in neonates who received intensive
care
S. Usman, N. Somani, S. Raltani
The Age Khan University, School of Nursing, Karachi, Pakistan
Many of the health care institutions are equipped and
staffed to provide high technical and quality care to the
high-risk neonates to enhance their survival rate, but there
are number of observed factors, i.e. prematurity, low birth
weight, maternal related diseases and nutritional status to
determine the quality of life of a neonate. The Main purpose
of this study is to test the validity and reliability of the tool,
Jerri and Richi (1993) in our setting, what risk of developmental
delay is involved for neonates who receive intensive
care in our Hospital. The data was collected retrospectively
by convenient sampling. The inclusive criteria is based on
neonate with weight ,1000 g, and gestational age more than
34 weeks. Jerri and Richi (1993) used a screening tool to
identify neonates at risk for cognitive, motor and physical
development delay. The same tool was adopted in this study.
The tool is comprises of seven variables. The variables are
further divided to determine the degree of intensity that is
assigned score. Neonates who score more than five are
considered to be at a higher risk of developmental delay
than others. In conclusion, the study did not show a significant
result because of small sample size and selection of
gestational age, i.e. .34 weeks. Based on the findings,
recommendations are suggested to replicate the study on a
larger sample size.
FP-D-011
Developmental coordination disorder in schoolchildren
with extremely low birthweight
H. Hara, T. Takamura
The National Institute of Special Education, Kanagawa,
Japan
As a result of improving outcome of extremely low
birthweight (ELBW; birthweight ,1000 g) infants, it
becomes more important to evaluate their psychomotorbehavioral
outcome up to school ages. In the cohort of
100 ELBW children without severe neurological sequelae,
we found out thirty-two, 25 boys and seven girls, diagnosed
as having developmental coordination disorder
(DCD) by age of 6. The motor development of 86 children
out of the original cohort was assessed on Kobayashi-Frostig
Movement Skills Test Battery (MSTB, 1989) at ages of
6–7 and/or 8–9. Fine and gross motor skills of the DCD
group were significantly inferior to those on the non-DCD
group at both evaluation ages. Although the means of DCD
group in all the MSTB scores were below the normal
ranges, the improving rates between the first and second
evaluations were approximated with those of non-DCD
group. Interestingly, the gross motor scores of ELBW
boys increased more steadily than those of ELBW girls
from 6–7 to8–9 years old. Whereas MSTB scores of the
ELBW girls marked higher than those of the boys, the
differences became smaller at the second evaluation. A
gender factor might affect gross motor development of
the ELBW children during early school age.
FP-D-012
Psychological intervention in habilitation process with
cerebral palsy preschool children
E.S. Julia, M. Velichko, L. Zeldin, A. Bitova
Russian Federation President’s Medical Center, Center for
Curative Pedagogics, Moscow, Russia
CP is the most common and severe neurological disability
in children. Curative factors bordering pathogenic therapy
and correction of neurological defect is still quite actual.
Purpose: To check up the hypothesis of evolution of
motor competence of CP patients in habilitation work
process including sensory stimulation. Material and methods:
We studied 12 children with different type of CP at the
age of 16 months–6 years (I group) and control group of
seven children with CP. Patients of 2nd group got standard
treatment program and patients of 1st group got modified
one. We pointed the level of motor disabilities (1–5 level of
disability) and investigated not only motor disorders but
also sensorial, social, cognitive, perceptual ones as well as
to get more insight in the developmental evolution of the
behavioural phenotype and analysed relations of ‘therapistpatient’
during habilitation process. The follow-up period
was 2 years. Results: As we got disappointed of the existing
relations ‘patient-therapist’ where the patient with CP
played predominantly passive role. The ‘model of action’
was desired and it allows the patient plays the role of
precosiour of motor competence improvement (I group-
66%, II group-43%). Conclusion: We carry out the analysis
of system of the relations ‘patient-therapist’ which shows
the vertical interaction was the dominant form and patient
had passive role. We suggest adding methods of sensorial
stimulation into standard treatment program of CP
preschool children so it is the essential and necessary part
of it.

444
Abstracts
FP-D-013
Intellectual development of premature infants and
effects of early interventions
L.-F. Liao, R.-Y. Huang, C.-W. Yang
The First People’s Hospital of Shunde, Daliang Town,
Shunde City, Guangdong, China
Purposes: To compare intellectual developmental status
between natural delivery and abnormal delivery premature
infants. To study the effect of early interventions on promoting
their intellectual development. Objectives and methods:
One hundred twenty infants, including 54 natural delivery
premature, 39 abnormal delivery premature (terminate gravidity
artificially), 37 normal full term infants, with average
gestation age of 35.3, 35.9 and 40.0 weeks, respectively, were
assigned randomly into intervention or control group. The
intervention group received early intellectual interventions
beginning from newborn period. All parents of these infants
joined regular routine children health care instruction course
at outpatient clinic. Using Gasell infants diagnosis measuring
scale to check intellectual DQ regularly, five observing
indexes are included: gross motor, fine motor, cognitive,
communication and language, social ability. Results:
Among all six factors of infants’ basic situation (monthly
age, body weight at born, mother’s age when giving birth,
gestational complications, type of delivery, complications of
new-born infants), only infants’ monthly age has influence on
their intellectual development. After adjusting monthly age,
premature delivery seems to influence gross motor and
cognitive ability only (that means lower scores of these two
factors in premature infants) but not influence other indexes.
Early interventions strongly influence all five intellectual
indexes. The intervention group got remarkably higher
scores on all indexes than the control group. Conclusions:
Early interventions promoted intellectual development of
premature infants. It was plausible to promote infant’s intellectual
development on their gross motor and cognitive ability
effectively through training.
FP-D-014
A study about the effects of external hydrocephalus on
neurological and psychomotor development in infants
J.-N. Mai
Neurological and Rehabilitating department, Guangzhou
Children’s Hospital, Guangzhou, China
Objective: To study the effects of external hydrocephalus
(EH) on neurological and psychomotor development of
infants. Methods: Four hundred and twenty-two infants
with EH were assessed with head CT scan and divided into
three groups based on the width of the subarachnoid space
(mild, moderate and severe groups). All infants were
assessed with Gesell development scale, TCD, BAEP and
EEG at age of 4, 8, 18 and 24 months. Results: Two hundred
and sixty-five were in mild group; 119 were in moderate
group and 38 were in severe group. One hundred and
twenty-six Infants (47.5%) in mild group appeared slight
abnormal neuropsychological behaviors within 6 months
age periods, transient motor delay within 12 months age
periods; EEG and TCD were normal and enlargement of E
space disappeared over 24 or 30 months age; 57 infants
(21.5%) appeared 1–2 episodes of convulsion with fever
during 10–24 months age and 15 infants (5.6%) were diagnosed
as seizure. At age of 8 months age, 43 infants (36.1%)
in moderate group had abnormal EEG and in TCD, 86
(72.2%) showed abnormal blood flows in cerebral posterior
or/and middle arteries; and 29 infants (76.3%) in severe
group had abnormal EEG and twenty-one (55.2%) showed
abnormal TCD. Among 157 infants in moderate group and in
severe group, 143 infants (91.0%) had motor delay; at 2 years
age 56 infants (35.6%) had motor dyscoordination with some
neurological soft signs, 46 infants (29.2%) diagnosed as CP;
35 infants had (22.2%) speech problems; only 15 infants
(9.5%) encountered convulsion and five (0.02%) were diagnosed
as seizure. Conclusion: Infant in mild group had transient
effects on infants in motor and neuro-psychological
behaviors. Moderate and severe groups had significant
effects in motor functions and neuro-psychological behaviors
which are some functions of frontal lobes. But long
term effects needs further follow-up.
FP-D-015
1:2 case-control study of risk factors for childhood
cerebral palsy
X.-J. Zhou, Z.-Y. Zhao, Q.-X. Shui, K. Chen, K.-H. Zheng
The Children’s Hospital, Zhejiang University School of
Medicine, Hang Zhou, China
Objective: To investigate the possible risk factors for CP in
children. Methods: A population-based 1–2 case-control
study was performed within 66 townships of 15 cities or
counties in Zhejiang Province, from October to November
1998. Results: Statistically significant variables associated
with an increased risk for childhood CP were neonatal
diseases, maternal diseases, low birth weight, maternal irregular
menstruation, receiving toxic substances in pregnancy,
malnutrition in pregnancy, paternal ages. It was highly
significant that mothers perinatal health care was a protective
factor for CP in children. Conclusions: The prevention and
elimination of the possible risk factors for CP might be
expected to reduce the morbidity of CP.
FP-D-016
Neuromental development of term non-asphyxiatd small
for gestational age (SGA) children at the age of four
I. Prpić, I. Vlašić-Cicvarić, E. Paučić-Kirinčić, D. Fiket
University Children Hospital ‘Kantrida’ and Medical
Faculty of University of Rijeka, Rijeka, Croatia

Abstracts 445
The aim of the study was to establish whether the intrauterine
growth retardation (IUGR) has direct effect on
neuromental development in children at age of 4. Term
(38–42 weeks of gestation) small for gestational age children
(SGA) without any detectable risk factors for IUGR
were analysed at the age of four. SGA infants were defined
by birth weight less than 10 percentile considering gestational
age, sex and mother parity, using normative data
from proper population. Control group was appropriate
for gestational age infants, without any risk factors for
altered neuromental development. Retrospectively
collected data were compared by parent’s statement, moreover,
all parents filled specially prepared questionnaire to
avoid possible socio-economic influence on child’s neurodevelopment.
There were 40 SGA children and 20 children
in control group which exams and tests were compared
using standard statistical methods. At the age of four of
SGA children still had statistically significant lower height
and weight than control group children, as it was at the
newborn period. There were no statistically significant
differences in other (sex, age, socio-economic environment)
parameters. All children had normal neurological
exam. Psychological tests of showed adequate neuromental
development of SGA children, but statistically significantly
lower than control group children. These results proved
that SGA children have delayed neuromental development
comparing with their mates, which is a direct consequence
of IUGR. SGA infants need long term neuropsychological
follow up and advising. Further researches needed to determine
pathophysiological mechanism of delayed/decreased
CNS maturation caused by IUGR.
FP-D-017
The etiologic spectrum of cerebral palsy: a
contemporary perspective
M.I. Shevell, A. Majnemer, I. Morin
Montreal Children’s Hospital, McGill University,
Montreal, Canada
Objective: CP is an established symptom complex which
results from heterogeneous etiologies. Our understanding
of the relative contribution of etiologies to the occurrence
of CP is largely derived from studies lacking systematic
neurological evaluation or the application of contemporary
imaging modalities. Methods: Over a 10 years inclusive
period, the case records of all consecutive patients diagnosed
with CP in a single pediatric neurology practice were
reviewed with reference to clinical features and etiologic
determination. Results: A total of 217 cases of CP were
identified (129 male, 88 female) – 77 (35.5%) spastic quadriplegic
(Q), 68 (31.3%) spastic hemiplegic (H), 39 (18%)
spastic diplegic (D), 5 (2.7%) spastic monoplegic (M), 12
(5.5%) mixed (Mx), 12 (5.5%) ataxic-hypotonic (AH), 2
(0.9%) dyskinetic (Dk), 2 (0.9%) Worster-Drought
syndrome (WD). Overall etiologic yield was 82.0% varying
according to type of CP; 50% Dk, 59% D, 80% M,
80.9% H, 90.9% Q, 91.7% AH and 100% Mx/WD. As an
identified cause, the top five etiologic entities identified
were: periventricular leukomalacia 24.9%, intrapartum
asphyxia 21.7%, cerebral dysgenesis 17.1%, intracranial
hemorrhage 12.9%, vascular 9.7%. While a single etiology
was apparent in 144 (66.4%) of the cases, multiple etiologies
were felt to be contributory in 34 (15.6%) of the cases.
The etiologic profile varied according to such features as
the type of CP, gestational age and the source (high risk
neonatal population or not) of the patients. Features of the
child’s CP such as microcephaly, neonatal difficulties, prior
or co-existing epilepsy and high-risk source were found to
be predictive of eventual etiologic yield. Conclusion: This
contemporary evaluation of CP etiologic yield suggests
that it is much higher than previously reported and varies
depending on key clinical features suggesting strategies for
prevention, identification and treatment of this symptom
complex.
FP-D-018
Predictors of developmental disability following openheart
surgery in infants with congenital heart defects
A. Majnemer, C. Limperopoulos, M.I. Shevell, C. Rohlicek,
B. Rosenblatt, C. Tchervenkov, H.Z. Darwish
Montreal Children’s Hospital, McGill University,
Montreal, Canada
Objective: Neurodevelopmental disabilities are increasingly
reported in infants with congenital heart defects
requiring open-heart surgery. This study describes the
prevalence of persistent developmental impairments 1
year following surgical correction, and identifies risk
factors. Methods: In this prospective study, 131 infants
were recruited and assessed in a blind fashion preoperatively,
following surgery before discharge, and 12–18
months later using standardized developmental assessments
and neurological examination. Results: At a mean
age of 19.1 ^ 6.6 months, 41% had abnormal neurologic
examinations. Motor delays were noted in 42%, and 23%
had global developmental concerns. Using the WeeFIM
measure, moderate disability in functional activities was
documented in 37% but only 6% were severely disabled.
Socialization and daily living skills were delayed in .40%.
Factors enhancing risk for long-term developmental
disabilities included: pre and postoperative neurodevelopmental
status, microcephaly, type of heart lesion, length of
circulatory arrest time, age at surgery, and days in the ICU
(univariate and multiple regression analyses: P , 0:05).
Conclusion: Although severe deficits are rare, ongoing
mild to moderate developmental disabilities are common
in infants undergoing open-heart surgery. Risk factors
suggest that etiology is likely multifactorial and includes
brain injury prior to, during and following surgical correction
of the defect.

446
Abstracts
FP-D-019
How early is it possible to predict cerebral palsy and to
differentiate between the spastic and the dyskinetic
forms?
C. Einspieler a , G. Cioni b , A.F. Bos c , F. Ferrari d , H.F.R.
Prechtl a
a Department Physiology, Graz University, Graz, Austria;
b Division of Child Neurology and Psychiatry, University
and Stella Maris Scientific Institute, Pisa, Italy; c Department
of Paediatrics, Division of Neonatology, Groningen
University, Groningen, The Netherlands; d Department of
Obstetrical, Gynaecological and Paediatric Sciences, Division
of Neonatology, Modena University, Modena, Italy
The fetus and young infant are characterised by a repertoire
of distinct spontaneous movement patterns. One set of
these patterns is the GMs. They emerge at 10 weeks postmenstrual
age and continue in a similar pattern until the end
of the 2nd month post term. Around this time GMs change
into the fidgety pattern. From 3 to about 5 months these
fidgety movements (FMs) are present in normal awake
infants. They are circular movements of small amplitude,
moderate speed, and variable acceleration of neck, trunk,
and limbs in all directions. A number of studies demonstrated
that any impairment of the young nervous system
changes the GM quality. Applying the GM assessment longitudinally
in 259 infants revealed that two age-specific GM
abnormalities reliably predict spastic CP: (a) crampedsynchronised
GMs (all limb and trunk muscles contract
and relax almost simultaneously and GMs appear to be
rigid). If this pattern exists over several weeks during
preterm and term age, spastic CP will develop at later age;
(b) and the absence of FMs. In addition, the absence of FMs
is also predictive for dyskinetic CP. In contrast to spastic
cases, later dyskinetic cases have ‘arm movements in
circles’, finger spreading, and a lack of limb movements
towards the midline. By qualitative assessment of GMs
and concurrent movements we early identified infants at
high risk for spastic or dyskinetic CP, which is of significant
clinical relevance because the two types of cerebral palsy
ask for different management and early intervention.
FP-D-020
The neonatal and early developmental outcome of very
low birth weight infants ventilated at a private hospital
in South Africa
B. Laughton, A. Crafford, R. Alexander, G.F. Kirsten, P.D.
Muller, J. Smith
Tygerberg Children’s Hospital, Tygerberg, Western Cape,
South Africa
Introduction: The only documented outcome for VLBW
infants in South Africa are for those born at tertiary state
hospitals. Private hospitals have better resources, and we
need accurate information on the outcome of infants managed
at these institutions. Objectives: To determine the
morbidity, mortality and developmental outcome of
VLBW infants admitted to the Panorama NICU. Patients
and methods: 99 VLBW infants admitted between 1 January
1999 and 31 December 2000 were followed prospectively.
The infants were followed up at 4.5, 8, 12 and 18 months by
a paediatrician and a physiotherapist. Development was
assessed on the Molteno Scale (before 24 months corrected
age) and the Griffiths Scales at 24 months corrected age.
Results: (Table 2). Conclusion: The mortality rate (9%), and
early motor developmental and cognitive outcome is similar
to that reported for VLBW and extremely low birth weight
infants managed in first world countries. The prevalence of
ROP and IVH is significantly lower.
FP-D-021
The neurodevelopmental outcome at 6 years of infants
,1250 g electively not admitted to the neonatal intensive
care unit
J.I. van Zyl, G.F. Kirsten
Tygerberg Children’s Hospital, University of Stellenbosch,
Tygerberg Hospital, Tygerberg, Western Cape, South Africa
Introduction: Infants with birth weights ,1250 g are
selectively admitted to intensive care because of limited
resources. Objective: To compare the neurodevelopmental
and cognitive outcome at 6 years of age of infants ,1250 g
Table 2
Birth weight and no. (%) , 1000 g (40) 1000–1499 g (59)
Mean gestational age (weeks) 7.2 (r: 25–32) a 29.4 (r: 26–36)
Mean birth weight (g) 814.1 (r: 480–880) 1229.0 (r: 1000–1480)
IVH grades 3 and 4 (%) 3 (8) 3 (5)
Survival – total (%) 33 (83) 57 (97)
ROP grades 3 and 4 (%) 2 (6) b 0
Patients followed up (%) 28 (84) 43 (75)
Definite CP (%) 3 (11) 3 (7)
DQ .80 (%) c 24 (86) 43 (100)
a
b
c
r ¼ range.
None blind.
DQ ¼ developmental quotient.

Abstracts 447
who received neonatal intensive care during the first 48 h of
life (NICU group) to those who received high care (neonatal
ward, NW group). Methods: 178 infants were admitted of
whom 60.4% (NW group) and 80.5% (NICU group) survived
(P ¼ 0:0004). Disability was defined as CP, sensori-neural
(SN) deafness and a general developmental quotient (GDQ)
,80. Results: At 6 years of age 20% were disabled; [NW
group 12% versus NICU group 27%, P ¼ 0:08]. The NW
group had significantly less physical disability (CP and SN
deafness) than the NICU group [5 versus 18% P ¼ 0:047].
The mean GDQ of the Griffiths Mental Developmental
Scales did not differ between the NW and NICU groups
while the locomotor subscale quotient was significantly
higher in the NW group [NW: 107.5 NICU: 93.9,
P ¼ 0:007]. The NW group had significantly less children
with a total impairment score #15%. Movement ABC Test,
only physically normal children tested than the NICU group
[NW: 25%, NICU 50%, P ¼ 0:021]. Conclusions: Elective
refusal to the NICU resulted in a higher mortality rate, but did
not impact on the cognitive outcome. NICU admission
however did result in more physical disabilities and worse
scores for the physically normal children on the Movement
ABC Test. The reason is probably multifactorial and could
include compromise on admission and postnatal complications
of ventilation.
FP-D-022
Observation on pre- and postnatal maternal use of
sertraline, causing transient floppy infant syndrome
I. Sos
Department of Developmental Neurology and Neurohabilitation
Pediatric Institute ‘Svábhegy’, Budapest, Hungary
The SSRI treatment of some psychiatric diseases spread
worldwide, because of its effectiveness and few adverse
effects. By former opinions there is not known side effects
of the treatment of the pregnant or nursing women on the
fetuses, and on the breast fed infants. Our patient’s mother
had used sertraline 50 mg/day from the second trimester of
her pregnancy. The pregnancy and labour was uneventful,
but the neonate was floppy infant. This clinical picture
persisted until the sertraline uptake stopped. Some weeks
later the baby was symptom free. We shortly discuss the
possible adverse effects of the early serotoninerg excess
on the developing human brain.
FP-D-023
Elementary sensorimotor functions in clinical practice
F. Katona, M. Berenyi
Department of Developmental Neurology Pediatric Institute
‘Svabhegy’, Budapest, Hungary
Stimulation of the striopallidal system through vestibular
and reticular channels triggers a series of complex automatically
repeated continuous movements. These are: unsupported
sitting up and sitting in the air, unsupported crawldown
and semi supported crawl-up on a slide, supported
rotation, assisted creep and walk, and others. Activation of
these complex movements can be achieved in neonates from
the 27th–30th weeks of gestation, even in seemingly hypotonic
prematures, and in infants during the first two trimesters.
Elementary sitting, crawling, walking imitate later
human-specific movements and are most likely their ontogenetic
precursors and these elementary activities depend upon
the integrity of the basal ganglia. Authors present evidence
by examinations of various anencephalus neonates. In an
anencephalic neonate whose brain developed up to the rostral
part of the pons all primitive reflexes are present, but none of
the complex elementary functions are elicitable. Examination
of elementary forms of sitting, crawling, walking, rotation
in several weeks old infants offers the possibility to
reveal differences in muscular activity, tonus and coordination
during active movements. Authors illustrate the application
of elementary sensorimotor poly-electromyography
(EMG) activities to diagnose young infants, also applying
videotape, and computerised motor analysis. Evaluation of
sensibility-specificity relation is presented by statistics of
2000 brain injured infants compared to normal control
groups.
FP-D-024
Symptomatic epilepsy in brain injured young infants
M. Berenyi, F. Katona, E. Gisztl, I. Sos
Department of Developmental Neurology, Pediatric Institute
‘Svabhegy’, Budapest, Hungary
Out of 2000 brain injured infants 450 produced symptomatic
epilepsy. From this 450 the number of continuous
epileptic activity from birth was 60 and the other 390
presented their first convulsions with positive EEG
between 4 and 8 months of life. In 300 cases the foci of
the convulsive activity change not only from one hemispherical
lobe to another (178 cases) but from one hemisphere
to the other (36 cases). In 230 cases West type
epilepsy was detected, among them 170 with unspecified
movements during convulsions, nevertheless with classic
EEG signs. In 150 cases the development of epilepsy
was followed up from the first signs of sharp waves to
wave depression and finally to hypsarhythmia. All infants
received in our service regular anticonvulsive therapy
(carbamazepine, vigabatrin, ACTH, etc.). Summary: (1)
Occurrence of symptomatic epilepsy in brain injured
infants is a warning sign predicting serious outcome in
sensorimotor and cognitive development despite any
improvement reached by early neurotherapy. (2) In the
majority of cases abnormal precognitive development
preceded the onset of epilepsy and is not a consequence
of epileptic fits. (3) Serial video-EEG and/or polygraphy
are necessary to follow changes in the nature and location
of bioelectric activity in the injured developing brain.

448
Abstracts
FP-D-025
Effect of cerebrolysin and simple training method at
home on children with cerebral palsy
J.-L. Tang, D. Wu
Pediatric department, The first hospital affiliated to Anhui
medical university, Hefei City, China
The purpose of this study was to find a better and
cheaper way to cure children with cerebral palsy. There
are 266 cases of children with cerebral palsy from 3
months to 10 years old. They were divided into four
groups: cerebrolysin group, simple training method
(STM, which designed by ourselves) group, cerebrolysin
plus STM group and control group which were follow-up
in 5 years. Cerebrolysin was transfused through vein to the
patients, 5 ml/day for no more than 1-year-old children, 10
ml/day for kids older than 1-year-old children. We define 3
months as period of treatment. At the same time we regularly
hold short courses of STM method for the children
with Cerebral palsy and their parents from October 1992.
Children with cerebral palsy and their parents attended to
the course. Some simply training methods of STM method
were introduced to the parents-home conductors, and then
the parents began to train the CP children at home. The
results show that STM plus cerebrolysin is most effective
in all groups (P , 0:01). Both cerebrolysin group and STM
group have show more effective than control groups
(P , 0:01): but the cerebrolysin group is more effective
than STM groups in young children group (age ,3 years
old) and they have no difference in old children group (age
3 years old).
FP-D-026
Case-control study on 89 cases of childhood cerebral
palsy
Q.-X. Shui, X.-J. Zhou, Z.-Y. Zhao
The Children’s Hospital, Zhejiang University School of
Medicine, Hang Zhou, China
Objective: To investigate the early identification of CP
in children. Methods: One to two case-control study and an
analysis on the symptoms and signs of 89 children with CP
were done. Results: There were 69 (77.6%) cases with
spasticity type, 11 (12.4%) cases with hypotonia type, 5
(5.6%) cases with the mixed type, 2 (2.2%) cases with
athetosis type, and 2 (2.2%) cases with ataxia type. And
there were 74 (83.1%) children with mental retardation, 20
(23.3%) children with psychological behavior disorders
and 13 (14.8%) children with epilepsy. The high-risk
factors included neonatal diseases, maternal malnutrition,
low birth weight, maternal irregular menstruation, older
father, absence of perinatal health-monitoring, and contacting
toxic substances in pregnancy. Conclusion: The four
main points for earlier identification of CP included
detailed medical history, understanding the normal motor
development of infants, grasping four characteristic manifestation
of abnormal motor of CP, and selective accessory
examinations.
FP-D-027
Morbus Little can be successfully treated – case report
K. Boskovic a , N. Naumovic b , D. Filipovic b , S. Tomasevic a ,
N. Mesanovic a
a Clinical Center Novi Sad, Medical Rehabilitation Clinic;
b University of Novi Sad, Medical Faculty, Department of
Physiology, Novi Sad, Yugoslavia
The Morbus Little (ML) or spastic infantile diplegia is
very frequent form of children’s cerebral palsy. Treatment
of that disease has multidisciplinary approach and rehabilitation
team with many members. The aim of this article
was case report of one patient with ML whose result of
treatment was very successful. The patient MM, female,
was born in 1974 year with 32-gestation week’s, 1300-mg
weight and with signs of cerebral hemorrhage. During 1st
year of life motor deterioration, without mental backwardness
was noticed. The diagnosis of ML was established.
Immediately the kinesitherapy for management of muscle
spastics was used and also further stimulation of basic
forms of sitting, standing and walking. But strong spastic
changes of plantar flexor muscles of the foot and knee
flexor and adductor muscles independent walk made
impossible. When patient was 4 years old, the surgical
lengthening of Achilles tendon and adductor tendons was
made. By operation the ‘scissors’ walk was moderated. The
further aim of treatment was independent walk, which is
realized in the patient’s age of 6 years. The walks was
spastically paraparetic, with massive mobilization of
whole body and with rely on interior part of the foot.
Despite to the continuous treatment, in age of 10 years,
patient has second surgery with lengthening knee flexor
and also adductor muscles. The rehabilitation treatment
was applied further with different physical procedures
(hydro-, heating-, exercise- therapy) usage. Because of
very late start of standing and walking patient’s articulations
development was insufficient, and arthritic changes of
the hips appeared in age of 16 years. Until now patient was
under rehabilitation treatment with the aim of the muscle
strengthening, preservation of the articulation motion and
improving of all kind of balance. The result of this long
and difficult treatment is person of 27 years, graduated
psychologist, completely independent in activities of
daily living. So, we can conclude that ML is very difficult
and complex disease which demands continuous, persistent
and multidisciplinary therapy with active participation of
patient, its family and wide social community, but it can be
treated successfully.

Abstracts 449
FP-D-028
Combined conductive education and therapeutics of
neurophysiology to improve and evaluate the motor
function in children with cerebral palsy
C.-M. Zhao, Y.-P. Zhang, W. Liao, Y.-P. Ai, M. Xi, L. Yao
The Pediatrics, The Xinqiao Hospital, Third Military Medical
University, Chongqing, China
Objective: To observe combined conductive education
and neurophysiology therapies in the improvement of the
gross motor function in CP patients. Methods: Group one in
23 cases of CP children treated with conductive education
and therapeutics of neurophysiology and group two in 25
cases of CP children treated only with Bobath and Vojta
processes all of cases are 1–3 years old. Results: The results
showed that the improvement of patients’ motor function
can be seen in both groups (P , 0:01). The function
improvement in group one after treatment is much better
than group two (P , 0:05). Conclusion: The results show
that the combined conductive education with therapeutics of
neurophysiology in the improvement of the gross motor
function in CP patients is better than Bobath and Vojta
processes.
FP-D-029
Some new approaches to an estimation of a functional
condition of a spinal cord at children with cerebral palsy
syndrome
T.E. Cimbal, A.G. Remnev
Altai Diagnostic Center, Barnaul, Russia
We observed 53 children with an age between 7 and 15
years with cerebral palsy syndrome (CPS). All patients were
divided into two groups: 24 patients with clinical attributes
bottom spastic paraplegia (first group) and 29 patients with
clinical attributes spastic hemiplegia (second group). We
studied a functional condition of the pyramidal ways
(PW) at magnetic stimulation (MS) of cerebrum cortex
and lumbar region of spinal cord and afferent ways of spinal
cord at MS of cervical and lumbar departments of spinal
cord. We used Magstim-200 (Magstim, UK) and Sapphire
2M (Medelec, UK). As a result of research the attributes of
infringement of realization of excitation on PW at 20
patients of the first group (mainly bilateral changes) and
12 patients of the second group (at seven patients bilateral
changes) as increase of time of the central realization till
21–36 ms were registered. At research of a functional condition
AWSC at 22 patients of the first group and 20 patients
of the second group we have registered decrease of speed of
distribution of excitation on AWSC till 34–49 m/s. Thus,
through a method MS the definition of a defeat of various
conducting ways of a back brain is possible. At the patients
with clinical attributes spastic hemiplegia the attributes of a
defeat of the ‘healthy’ party were registered. It means, that
the functional changes not always correspond clinical.
FP-D-030
The study of Chinese children’s vocabulary development
W.-L. Liang a , B. Hao a , S. Wang a , Y.-Q. Jiang a , Z.-X.
Zhang a , Q.-H. Zuo a , T. Tardif b , P. Fletcher c
a Peking University Fist Hospital, Beijing, China; b University
of Michigan, Ann Arbor, MI, USA; c University of Hong
Kong, Hong Kong, China
Objective: To conduct a norm study of Chinese communicative
development inventory (CCDI) and to study
Chinese children’s vocabulary development. Method:
Using CCDI – Putonghua version), Parents of 1056 toddlers
were asked to report their children’s language abilities.
Results: Sixteen-month-old children can say an average of
79 words appearing on the CCDI, including 38 nouns and 21
verbs. By 30 months, children can say an average of 709
words on the CCDI, including 334 nouns and 117 verbs. The
speed with which children acquire nouns and verbs is similar.
After 19 months of age, children also began to use
classifiers question words and pronouns. For most types of
the words, the mean production scores tended to be higher
for females than for males of the same age. Conclusions:
From 16 to 30 months, the development of the children’s
words is rapid for content words, including both nouns and
verbs. After 19 months, Chinese-speaking children also
begin to use function words, including classifiers, question
words and pronouns.
FP-D-031
Birth asphyxia: still a major risk factor for cerebral
palsy in Bangladesh
S. Akhter, M.-M. Rahman, K. Akhter, M.N. Islam
Department of Paediatrics, Bangabandhu Sheikh Mujib
Medical University, Dhaka, Bangladesh
This study was done to assess some of the identifiable
risk factors for CP. Four hundred and fifty children suffering
from CP attending over 2 years 3 months time period in
the our-patient department of Bangabandhu Sheikh Mujib
Medical University in Dhaka, Bangladesh -were studied
retrospectively. Information regarding antenatal, natal and
postnatal period was collected by taking detailed history.
Physical examinations were done to confirm the diagnosis.
In some of the cases relevant laboratory investigations
were done. Mean age was observed to be 2 years 6 months.
Male to female ratio was 2:1. Most common type of CP
was spastic (82.2%). Diskinetic, hypotonic and mixed were
also found. In the spastic group, diplegia comprised the
maximum number of cases (34%). Tetraplegia (27.3%)
and hemiplegia (19%) were also common. Majority of
the risk factors were natal. Birth asphyxia was found in
43.8% of all the cases making it the most common identifiable
risk factor. Other important natal factors were low
birth weight (18.7%) and preterm (15.8%). Postnatal

450
Abstracts
factors like neonatal convulsion (14%), neonatal jaundice
(9%) was responsible in 34% of cases. Prenatal factors
were identified in 23% of cases. In 13% of cases there
was no antenatal check up at all and in 78% of cases
delivery was conducted at home. From the study it can
be concluded that birth asphyxia has still remained as a
major risk factor for CP in a developing country like
Bangladesh. An integrated antenatal and perinatal care
might reduce the rate of CP.
FP-D-032
Effective analysis of part radicotomy in convulsive palsy
Z.-X. Wang
China-Japan friendship Hospital, Beijing, China
Objective: To explore the effect of part radicotomy in
spastic cerebral palsy and the significance of combination
with rehabilitation. Subjects and methods: 43 cases with
spastic cerebral palsy aged from 2 to 14 years old were
enrolled in the study. All underwent selective part radicotomy,
of that 19 cases were combined with rehabilitation
after operation. Effects were compared between the cases
with only surgical group and the group combined surgery
and rehabilitation. Results: Most of them had asphyxia
history (19 cases 44.4%) and premature birth (12 cases
25.96%). Part radicotomy had effect on 51.2% of 44
cases. However, in contrast to the effective rate of the
only operation group, the combined with rehabilitation
group had much better result (P , 0:05). Conclusion: The
part radicotomy was one of effective therapy for spastic
cerebral palsy. Operation combined with rehabilitation
was more effective than only operation.
FP-D-033
Developmental screening in infancy
F. Soleimani
University of Social Welfare and Rehabilitation Sciences,
Tehran, Iran
In this study we screened 6000 infants between 4 and 18
months by infanib scoring. In this assessment infants
divided to three groups; Normal, transient and Abnormal
by 20 items that based on French angles, reflexes, tone
and posture. The transient group was visited 1 month later
and the abnormal group was referred to pediatric neurologist
for diagnostic evaluation and to Rehabilitation Center for
treatment. In the beginning the Infanib was selected because
of high validity and reliability in the previous studies, and in
this study we also found out that it is the same in Iran. thus
the Infanib is proposed as an appropriate screening test in
the developing countries because of quantified, reliable
measure of body tone and posture, short time for examination
and the ability of occupational, physical therapist,
nurses and physician to perform it.
FP-D-034
The outcome of the term infants with low-Apgar score
and the predictor to the cerebral palsy
K.-W. Jiang, Q.-X. Shui
Department of Neurology, Affiliated Children’s Hospital of
Medical College, Zhejiang University, Hangzhou, China
To study the factors and the predictors correlated with the
long-term prognosis of the term infants with low-Apgar
score. We carried out a dynamic prospective study on a
cohort of 252 cases in a year. The Infant Motor Screen
test and the children’s development milestones evaluation
were applied at 6 months and 1-year-old, respectively. Five
died, and seven lost during follow-up. Of 240 survivors, 19
infants at 6 months were abnormal, and 15 infants at 1 year.
Compared to the control group, the differences were significant
(P ¼ 0:019 and 0.067, respectively). Four factors
(low-Apgar scores #3 at 1 min and #6 at 5 min, cranial
injury, intrauterine distress and cord wrapped around neck)
were correlated with the long-term prognosis. The neonatal
signs were predictors to the prognosis. Discriminant analyse
indicated that combination of convulsion, apnea, staring,
and spontaneous movements may predict 91.3% CP cases
at 1 year. The special neurobehavioral abilities at 6 months,
such as controlling the trunk at sitting, Moro reflex, may
predict 97.9% CP cases. Duration of severe asphyxia and the
encephalon trauma due to asphyxia were correlated to the
long-term prognosis in term neonates with low-Apgar score.
The neonatal abnormal signs had a positive predictive value
to the long-term prognosis, and a serially observation
showed a more precise prediction to the CP.
FP-D-035
The role of positioning and movement therapy for
infants with gross motor delay due to hypoxic-ischemic
encephalopathy
B. Bayasgalantai, P. Otgonbayar
Maternal and Child Health Research Center, Bayangol
Duureg, Ulaanbaatar, Mongolia
The incidence of infant developmental delay are numerous
in our country: this fact is justifiably one of the major issues
that draws the attention of pediatricians, neurologists and
researchers. As some researchers believe the main cause of
infants disabilities are severe hypoxic-ischemic encephalopathy/HIE/and
cerebral palsy. Objectives: (1) To identify
types of gross motor delay in infants due to HIE. (2) To
investigate role of positioning and movement therapy as an
early intervention in infants with HIE. Study design: We
followed 120 neonates with HIE to identify gross motor
delay, with regular neurological examination by a neonatologist
until 12 months of age: at discharge and follow-ups at 3,
6, 9 and 12 months of age. Then developmentally tested the
children using special scales. We provided a case-controlled,

Abstracts 451
quasi-experimental study. Results: Among the 120 children
followed 68 were identified as having a gross motor delay.
There are differences of muscle tone in 40.8–59.2% of the
children; inability to hold a head, and inability to sit and walk
in 24–62% of children. We have used positioning, sensory
stimulation and motor developmental intervention
approaches individually for each infant. Developmental
outcomes after motor developmental intervention
approaches were good: we have observed results after one
to two courses of therapy. Conclusions: HIE is the most
common disease in the neonatal period which causes developmental
delay. Each infant with HIE should be followed
with the Denver test during the 1st year of life. Positioning
and motor developmental intervention approaches are effective
for the therapy of developmental delay. We developed
standards of treatment for gross motor delay due to HIE
among infants.
FP-D-036
Cutaneomuscular reflex in the lower limbs of patients
with cerebral palsy and its clinical significance
Z.-M. Jiang, L.-Y. Shan, X.-H. Li
Prevention, Treatment and Rehabilitation Center for Child
Cerebral Palsy in Heilongjiang, Jiamusi, Heilongjiang,
China
To study the abnormal cutaneomuscular reflex (CMR)
changes recorded in the lower limbs in children with CP,
provide the index for clinical diagnosis. CMR were recoded
in the lower limbs in 80 cerebral palsy children aged 3 , 5
years and 30 normal children with the same age. Compared
with the control group, the latency of I1 and E2 of patients
with spastic cerebral palsy prolonged (P , 0:05). The
amplitude ratio of E2 and E1 reduced (P , 0:05). The
abnormal degree of CMR in spastic hemiplegia children
accorded with degree of clinical hemiplegia. There were
significant differences in the latency of E2 and the amplitude
ratio of E2 and E1 between the mild and the severe sides or
between side of health and hemiplegic side (P , 0:05 and
P , 0:01). Compared with the control group, the amplitude
rate of E2 and E1 increased in the patients with athetoid CP
(P , 0:05), the amplitude of E1 increased significantly
(P , 0:01). These results suggested that the main damage
brain area of spastic cerebral palsy was motor cortex and
pyramidal tract; the main lesion area of athetoid cerebral
palsy was extrapyramidal system. CMR may be used as
objective index for diagnosis of cerebral palsy.
FP-D-037
The evaluation of developmental age in infants in
Tehran city
P. Karimzadeh
University of Social Welfare and Rehabilitation Sciences,
Tehran, Iran
The differentiation of human being in life that leads to
somatic, mental, verbal and social promotion is called
development. Development in childhood period (specially
first and second year of life) is more rapid than the other
stages of life. We decided to evaluate the developmental
age in infants because there is no study in Iran. We studied
the developmental age in 611 infants in respect of Grasping
age, Crawling age, sitting age, walking age and social age.
Before study, infants were examined by pediatrician and all
of cases with genetic, congenital and acquired disorders
were excluded. A descriptive-analytic study was carried
out to evaluate the developmental age in infants in Tehran
city. We used Munich functional Diagnostic test. The
information collected using checklists. The results showed
that the level of developmental age in Iranian infants in
comparison of the results in other countries is upper. In our
studies with increase in educational level in mothers, we
had increase in medians of DQ in infants. The median of
DQ was 115.51 The median of grasping age, crawling age,
sitting age walking age, perception age, speech age, speech
comprehension age and social age were 2, 4, 7, 12, 5, 7, 10,
10 month.
FP-D-038
Hyperbaric oxygen therapy in Malaysian children with
cerebral palsy: a pilot study
M. Thambyayah a , C.T. Lee c , K. Thillainathan b ,S.T.
Cheoh b , K.M. Letchumi b
a Paediatrician, Hospital Selayang, Malaysia;
b Occupational
Therapist, Developmental Nurse/Physiotherapist,
Hospital Klang, Malaysia; c Diving Medicine Physician,
Armed Forces Hospital Lumut, Malaysia
Hyperbaric oxygen therapy (HBOT) has been used an
adjunct to treat children with cerebral palsy in several countries.
Numerous anectodal reports and few randomized trials
indicate that it works, despite ongoing controversies. In this
open-label, non-randomized study, 20 children, aged from
3.5 to 14.8 years, (mean 7.5 years) who had varying types of
cerebral palsy,(spastic ¼ 13, dystonic ¼ 3, ataxic ¼ 1,
mixed ¼ 1) were treated at Armed Forces Hospital Lumut
since May 2000, with 1.5 ATA hyperbaric oxygen, for a
total of 100 treatments (40 1 40 1 20), administered for 1
our, twice daily, 5 days a week, with a 12 week break in
between the three sessions. The team consisted of a child
neurologist, diving medicine specialist, occupational therapist,
developmental nurse and physiotherapist. The
outcomes used to measure were modified Ashworth Spasticity
Scale, (MAS) modified tests of gross motor and fine
motor function, parental reporting/satisfaction scale and
video-recording – these were employed before and after
HBOT. Eighteen children completed the entire treatment,
evaluations and assessments in Dec 2001. There were
significant improvements in alertness, overall communication
and cognition, drooling and also in fine motor more

452
Abstracts
than gross motor testing. Spasticity reduction was maintained
for about 12 weeks. The mean pre-HBOT MA in
hamstrings 1.2 and mean post HBOT MAS: 0.7 (42 %
improvement) were minimal (middle ear and sinus barotrauma).
HBOT is well tolerated in children with cerebral
palsy and there were significant improvements in several
areas of neurological functions.
FP-D-039
Evaluation of rehabilitation to children with cerebral
palsy
O. Nitta, M. Aoyama, M. Miyao, Y. Iwasaki, M. Shibata, M.
Yamamoto, K. Miura
Tokyo Metropolitan University of Health Sciences, Tokyo,
Japan
Purpose: Various rehabilitation programs have been
provided to children with cerebral palsy. However, there
has been no sufficient evaluation whether these programs
were adequate or not. Main reasons of this insufficient
evaluation may involve very long clinical course of the
disease, i.e. cerebral palsy, and the absence of objective
criteria to evaluate the programs. Thus, we examined the
rehabilitation programs during the life cycle of disabled
children from the birth to the adult. Furthermore, we
inquired whether these programs were appropriate for
their parents. Subjects and methods: We have made an
investigation based on a questionnaires sent out to the
parents of 110 handicapped children with cerebral palsy
(mean age, 12.26 years; range, 6–18 years) enrolled in
schools for physically handicapped or mentally retarded
children in Tokyo Metropolitan area. We inquired what
kind of rehabilitation programs their children received at
each class of age, and on whether they thought the programs
were appropriate or not. Results: In effective responses, 46%
of parents answered that contents of the programs were
‘inappropriate’ or ‘equivocal’. The reasons of the inappropriateness
involved ‘insufficient frequency’ and ‘inconsistent
programs by various therapists’.
FP-D-040
Do pre- and perinatal factors predict a need for special
education?
M. Mannerkoski, T. Autti, L. Åberg, M. Hoikkala, K.
Kuismanen, H. Heiskala
Departments of Child Neurology and Radiology, Helsinki
University, Hospital for Children and Adolescents, Helsinki,
Finland
Our purpose was to see whether some pre- or perinatal
factors as recorded in the school health care units could
serve as predictors for a need for special education? We
used a random and representative sample, altogether 900
pupils, of children attending classes for special education
in Uusimaa (population 1.4 million), Finland. This survey
was carried out in 1997–1998 in 19 different schools. The
teaching programs of these children (born between 1980 and
1993, boys 64% and girls 36%) were: (1) education modified
for specific neurological disabilities (23%); (2) adjusted
education (46%); and (3) training education (30%). A
random sample of children (N ¼ 300) in mainstream education
was used as a control. In preliminary analyses the most
important predictors were abnormalities detected after birth.
Also birth weight, birth height and occipitofrontal circumference
and Apgar score correlated with future academic
problems. More thorough regression analyses will be
presented and discussed.
FP-D-041
Treatment of spastic cerebral palsy by local injection
with different doses of Botulinum Toxin-A
B.-Q. Gao, W.-L. Yang, Y.-J. Wang, B.-J. Zhu, Z.-G. Zhao,
X. Deng, G.-F. Wang
Department of pediatrics, Beijing Tiantan Hospital Affiliate
of Capital University of Medical Science, Beijing, China
Objective: To evaluate the efficacy of local intramuscular
injections of three different doses of botulinum-A toxin in
the management of the spasm of cerebral palsy. Methods:A
total of 120 children with cerebral palsy were averagely
divided into three groups: group 1, group 2 and group 3.
Three different doses of BTX-A (group 1 1 m/kg, group 2 2
m/kg, group 3 3 m/kg) were administered. The effect of
treatment was evaluated by Physician Rating Scale method.
The difference among these groups was calculated by
‘analysis of variance’. The tolerance to BTX-A and the
side effects were also assessed. Results: Reduction on
spasm of muscle became apparent after injection in all
patients. Large doses of BTX-A (2 and 3 m/kg) were more
effective than small doses. No markedly side effects
occurred. Conclusion BTX-A may be proved as a safe and
effective treatment for the cerebral palsy.
FP-D-042
Neurological complications in child abuse at King
Chulalongkorn Memorial Hospital
S. Phancharoen a , T. Desudchit a , V. Pongpanlert a ,N.
Thadakul a , S. Chittinand a , N. Suwanwela b , S. Kaoropthum c
a Department of Pediatrics,
b Department of Radiology,
c Department of Surgery, Faculty of Medicine Chulalongkorn
University, Bangkok, Thailand
We studied neurological complications abused children
in King Chulalongkorn Memorial Hospital between Jan 1,
1997 to December 31, 2001. There were 65 cases in
suspected child abuse and neglected, 37 girls and 28
boys. Neurological problems were seen in 16 cases
(24.61%). The average age was 4.75 months (12 days–10

Abstracts 453
months). There were seven girls and nine boys. The most
common presentations were lethargy (five cases ¼ 31.25%)
and convulsion (five cases ¼ 31.25%). The neurological
examination and imaging revealed subdural hematoma
(ten cases ¼ 62.5%), retinal hemorrhage (four
cases ¼ 25%), and external physical injury only two
cases (12.5%). One serial imaging change revealed nonaccidental
head injured (Shaking Baby syndrome). Two
patients died shortly after hospital admission due to severe
brain edema. During a follow up 2 months–4 years (mean 2
years), nine cases (56.25%) had delayed development, six
cases (37.5%) had epilepsy and two cases (12.5%) had V-P
shunt. Only two cases (12.5%) were normal development.
High degree of clinical suspicious and early recognition of
this condition and team approach may improve outcome
and prevent child abused in the future.
FP-D-043
Differences in developmental trajectories as a function of
age and weight at birth in premature infants
J.M. Gardner a , B.Z. Karmel a , A. Harin b , A. Barone b , E.M.
Lennon a , M.J. Flory a , H. Phan a
a NYS Institute for Basic Research, Staten Island, NY; b St.
Vincents Catholic Medical Centers of NY, St. Vincent’s
Staten Island, Staten Island, NY, USA
Major advances in pre- and postnatal care in the last
decade have produced a cohort shift in the numbers and
types of infants surviving, with a growing number having
shorter gestations, lower birth weight (BW), and the products
of multiple birth. However, long-term outcome in this population
is poorly understood. This presentation will concentrate
on early medical indices used for predicting
developmental outcome over the first 2 years in 287 surviving
infants born ,32 weeks gestational age (GA) weighing
,1500 g, who were part of a follow-up study of neurofunctional
outcome of at-risk infants (28% were ,27 weeks and
20% were ,751 g). Infants with genetic or congenital
abnormalities were excluded. Bayley Scales of Infant Development
(BSID) were administered every 3 months from 4 to
25 months. Multiple regression indicated no significant
differences in GA or BW as a function of gender, ethnicity,
maternal education, exposure to cocaine in utero, or level of
prenatal care. Using hierarchical linear analyses, and modeling
performance as either a constant or a linear function over
age, we found that as GA or BW decreased, the Mental
Development Index (MDI) decreased regardless of age at
test even when corrected for inherent age trends in the
BSID. However, the PDI showed an accelerated decline as
infants got older, with the youngest and smallest infants at
birth declining fastest. Although increased severity of CNS
injury also predicted decreases in MDI and PDI across age, it
did not produce an accelerated decline in PDI. Combining
severity of CNS injury with degree of immaturity predicted
the developmental trajectories of outcomes best.
FP-D-044
Predicting cognitive and motor outcome in the 2nd year:
the role of early measures of attention, and elicited and
spontaneous movements
B.Z. Karmel, J.M. Gardner, E.M. Lennon, M.J. Flory, H.
Phan, I. Miroshnichenko, R.L. Freedland
NYS Institute for Basic Research, Staten Island, NY, USA
Defining the consequences of CNS injury in neonates is
important for predicting both short-term recovery from
acute problems and long-term outcome. We report relations
among three procedures: arousal-modulated attention
(AMA), neurobehavioral exam (NB), and spontaneous
general movements (GM), to each other and to Bayley
Scales of Infant Development over the first two years.
The sample consisted of 70 high-risk infants studied longitudinally
(mean GA ¼ 29.6 weeks; mean BW ¼ 1284 g;
CNS injury: none ¼ 12%, mild ¼ 54%, moderatesevere
¼ 34%). Infants were tested at hospital discharge
and at 1, 3 and 4 months. Both concurrent and predictive
validity of measures were demonstrated for our AMA
visual preference procedure, our neonatal NB emphasizing
elicited motor behaviors, and Prechtl’s assessment of GMs.
Although CNS injury was associated with each measure in
neonates, relations changed over time. Thus, CNS findings
alone were insufficient to predict later outcome. Early
behavioral evaluations were better predictors into the 2nd
year and indicated that: (1) high-risk infants who
performed normally as neonates had normal development;
(2) those with early transient abnormalities appeared to
normalize by 4 months, but started to show deficits by
13–16 months; and (3) increased degree and/or duration
of abnormal early behavior predicted the worst outcome.
Use of multiple measures in the neonatal period, including
attention and autoregulation along with elicited and spontaneous
movements, as well as multiple assessments across
early infancy, provide the most useful approach for evaluating
recovery from CNS injury, and for predicting later
mental and motor performance.
FP-D-045
Transsynaptic degeneration of lateral geniculate bodies
in children affected by cerebral visual impairment
S.M. Bova a , E. Fazzi a , S.G. Signorini a , C. Uggetti b , P.E.
Bianchi c , G. Lanzi a
a Department of Child Neurology and Psychiatry – IRCCS
C. Mondino, Pavia, Italy; b Paediatric Neuroradiology Unit-
IRCCS C. Mondino, Pavia, Italy; c Department of Ophthalmology
– IRCCS University of Pavia, Italy
Cerebral visual impairment (CVI) is the leading cause of
bilateral visual impairment in Western countries. It is characterised
by bilateral impairment of visual functions caused
by damage to the CNS. A common cause is PVL following

454
Abstracts
perinatal hypoxic-ischemic brain injury. Diagnosis of CVI
is based mainly on clinical signs but damage to the posterior
visual pathways is frequently documented on brain MR
in children with PVL. Lateral geniculate body (LGB) is the
thalamic relay nucleus of the geniculate visual pathway.
Lesion at this level have frequently been demonstrated
by pathological examinations - following transsynaptic
degeneration of these nuclei secondary to pregeniculate
or postgeniculate interruption of visual pathways - but
seldom documented on brain MR. In this study we investigate
MR alterations of visual pathways and in particular
of LGB in 20 children affected by CVI and cerebral palsy.
The presence of a small, symmetric area of T2 prolongation
in the exact site in which the LGB are located-consistent
with gliosis – was considered indicative of LGB
damage. Neurophthalmological findings are compared
with a control group of 20 children with similar clinical
and neuroradiological picture, except for LGB damage.
Comparison between the two groups shows higher incidence
of optic nerve atrophy, lower visual acuity, and
higher incidence of disorder of ocular motility (saccades,
and smooth pursuit) in children with LGB damage. These
findings suggest that MR signal alterations in the LGB,
probably attributable to transynaptic degeneration, can
also extend to the optic nerve and lead to poorer visual
outcome.
FP-D-046
The correlations of motor functions with speech and
psychosocial functions in the children with cerebral
palsy
C.-L. Chen a , I.-C. Chen b , C.-Y. Wu a , C.-Y. Chung a , C.-H.
Chen a , S.-C. Chen b
a Department of Physical Medicine and Rehabilitation,
Chang Gung Memorial and Children Hospital, Taoyuan,
Chinese Taipei; b Department of Physical Medicine and
Rehabilitation, Taipei Medical University Hospital,
Chinese Taipei
CP is characterized by non-progressive motor impairment.
The developmental functions varied in the children
with spastic CP. This study was to investigate the correlations
of motor functions with speech and psychosocial
functions in the children with CP. We collected 179 children
with CP, aged less than 6 years. We used the Chinese
Child Development Inventory to assess the developmental
functions with 8 domains, including GM, fine motor (FM),
expressive language (EL), concept comprehension (CC),
social comprehension (SC), self help (SH), personal social
(PS), and general development (GD). CP children were
divided into two groups: SD and spastic quadriplegia
(SQ). We compared the DQ between the two groups.
The correlations of different developmental functions
were analyzed by Pearson’s correlation. We found eight
DQs in the SD group were higher than SQ group
(P , 0:01). The DQs of GM and FM in the SD group
were 47 and 75%, respectively. While those in the SQ
group were 23 and 38%, respectively. The DQs of the
other functions in the SD group were 62–83%, while
those in the SQ group were 31–57%. The speech functions
(EL, CC, SC) were correlated more with FM function
(r ¼ 0:6–0:8, P , 0:01) than GM function (r ¼ 0:4–0:7,
P , 0:01). The other functions (SH, PS, GD) were also
correlated more with FM function (r ¼ 0:8, P , 0:01)
than GM function (r ¼ 0:68–0:78, P , 0:01). Our findings
suggest the SD group had better motor, speech and psychosocial
functions than SQ group. The fine motor function
may provide us as the predictor for speech and psychosocial
functions in the children with CP.
FP-D-047
Etiologic determination of 1088 children with
developmental delay
C.-L. Chen, I.-C. Chen, A.M.-K. Wong, C.-Y. Chung, S.F.-
T. Tang, C.-H. Chen
Department of Physical Medicine and Rehabilitation,
Chang Gung Memorial and Children Hospital, Kwei-
Shan, Tao-Yuan, Chinese Taipei
Children with developmental delay (DD) had varieties in
developmental function. The purpose of this study was to
analyze the etiologic diagnosis and risk factors in children
with different functional delay. We collected 1088 children,
aged less than 6 years, who underwent assessments
of developmental function, etiologic diagnosis, and risk
factors. All children were classified into five functional
delay groups: speech, motor, pervasive, global, and nonspecific
DD. The etiologic diagnosis was classified into
brain, neuromuscular, genetic, psychomental, and other
disorders. Differences of etiologic diagnosis and risk
factors of five functional delay groups were determined.
Most children had global (45.3%), motor (22.3%) and
speech (17.3%) delay, the other children had non-specific
(10.6%) and pervasive (4.5%) delay. Approximately 61.5%
of children were associated with biological factors (20.0%
of neonatal insults, 19.9% of genetic defect/congenital
anomaly, 13.5% of central nervous system lesions, 3.5%
of prematurity/low birth body weight, and 4.8% of other
factors). Most children with motor delay were brain disorders
and associated with major risks of neonatal insults
(P , 0:05). While most children with global delay were
brain or psychomental disorders and associated with
major risks of genetic defect/congenital anomaly or neonatal
insults (P , 0:05). Our findings suggested there were
heterogeneous risk factors and etiologic diagnosis in children
with different functional delay. These data could
provide us to investigate the underlying diseases for children
with developmental delay.

Abstracts 455
FP-D-048
Glasgow scale relationships in children with traumatic
brain injury
C.-Y. Chung, C.-L. Chen, M.-K. Wong, L.-C. See, F.-T.
Tang
Department of Physical Medicine and Rehabilitation,
Chang Gung Memorial and Children Hospital, Kwei-
Shan, Tao-Yuan, Chinese Taipei
The purpose of this study was trying to find out the
proper cutting point of GCS as a determinant of outcome
for children with traumatic brain injury (TBI). We
followed 319 children, aged 2–10 years old, with TBI.
The outcome of the children was evaluated using modified
Glasgow Outcome Scale (GOS). The GCS and GOS of the
children were studied in relation to clinical associated
injury and computed tomography scan features. Using
statistical analysis, we investigate the correlation of the
GOS with GCS. We found that a cutting point of GCS at
five got the best correlation of the GOS with GCS. Children
with TBI and GCS equal to or below five had worse
outcome while those with GCS above five had better
outcome. In the associated injuries and neuroimage
features, indicators like chest trauma, basal ganglion
lesion, cerebellar lesion, brainstem lesions, subdural
hemorrhage and other intra-cranial hemorrhage were
significantly correlated with lower GCS. Children had
chest trauma, abdominal trauma, frontal lesions, basal
ganglion lesions, cerebellar lesions, brainstem lesions,
subdural hemorrhage and other intra-cranial hemorrhage
also had worse GOS. We conclude that a cutting point of
GCS at five can best predict the outcome of TBI in children.
The best predictors of outcome from TBI are the
GCS score and other indictors of overall severity of brain
injury.
FP-D-049
Antenatal risk factors associated with the unfavorable
neurological status in newborns and at 2 years of age
T. Stelmach, E. Kallas, H. Pisarev, T. Talvik
Tartu University Clinics, Children’s Clinic; Department of
Pediatrics, University of Tartu, Tartu, Estonia
The aim of this prospective cohort study was to evaluate
the influence of different antenatal and birth factors on the
neurological signs at first days of life and the neurological
outcome at 2 years of age. Patients and methods: the study
group consisted of 390 children drawn from the cohort of
828 consecutive live births between October 1st 1998 and
March 31st 1999 at the Women’s Clinic, Tartu University
Clinics, Estonia. The data about all potential antenatal risk
factors were abstracted from pregnancy and delivery
records and registered in the database. The data about
any birth complications and the neonatal course for all
children were also collected from the hospital registry.
Crude Odds ratio estimates with 95% confidence intervals
were used to measure the associations between different
antenatal factors and the neurological symptoms, both in
newborn period and at 2 years of age. Significance level
P , 0:05 was considered acceptable. At 2 years of age 49
children from 390 exhibited adverse neurodevelopmental
outcome (CP and other developmental disorders). The
development of remaining 341 children was age-appropriate.
Comparative analysis of risk factors was conducted.
The strongest correlation with further development of HIE
at first days of life was found in such antenatal factors as
trichomoniasis during pregnancy (OR, 4.34; 95% CI, 1.32–
14.23,) and acute respiratory disease (temperature . ¼ 388
C) in II half of pregnancy (OR, 2.86; 95% CI, 1.08–7.58).
From different antenatal factors, only bacterial vaginosis
(diagnosed both clinically and microbiologically)
combined with threatening abortion in 1st half of pregnancy
(OR, 4.96; 95% CI 1.35–18.26) had significant association
with adverse neurodevelopmental outcome at 2
years of age. Arterial hypertension during pregnancy,
acute respiratory disease, intrauterine growth retardation,
edema, proteinuria, hypertension (EPH)-gestosis and preeclampsia
were not associated with adverse outcome at 2
years of age. Presence of at least one complication at delivery
placed the child at risk of adverse neurological
outcome (OR, 2.44; 95% CI, 1.32–4.54) at 2 years of age.
FP-D-050
Premature rupture of membranes and neonatal
neurological outcome in a cohort of preterm births: an
ongoing study
C. Arpino a,b , A. Di Paolo c , C. Ticconi d , S. Brescianini e ,D.
Poveromo b , E. Piccione d , P. Curatolo b
a ‘E. Litta’ Rehabilitation Center for Developmental
Disabilities, Grottaferrata, Rome, Italy; b Pediatric Neurology,
‘Tor Vergata’ University of Rome, Italy; c Department
of Pediatrics, Section of Neonatology, ‘Tor Vergata’
University of Rome, Italy; d Department of Surgery, Section
of Obstetrics and Gynaecology, ‘Tor Vergata’ University of
Rome, Italy; e National Institute of Health, Rome, Italy
Premature rupture of membranes (PROM) is associated
to 30–40% of preterm deliveries and it is complicated by
chorioamnionitis in up to 70% of cases. Data from medical
literature suggest that the risk of adverse neonatal neurological
outcome is higher in preterm births exposed to
PROM compared to non exposed preterm birth. The objective
of this study is to evaluate the effect of premature
rupture of membranes, on neonatal neurological outcome
in preterm births. To this end, we conducted a retrospective
cohort of preterm births, including all consecutive
preterm newborns (n ¼ 600; gestational age ,37 weeks),
referred to the Pediatric Unit, Neonatal Intensive Care and
Nursery, of the ‘Tor Vergata’, University of Rome, in the

456
Abstracts
period 1996–2001. The source of information was represented
by clinical records of newborns (all clinical information
relevant to outcome evaluation and control for
confounding) and newborns’ mothers (history of pregnancy
and delivery, in particular onset, duration and
complications of PROM). Up to now, 109 newborn-andmother
pairs have been enrolled into the study. Overall,
32% of preterm births were exposed to PROM. Adverse
neurological and respiratory outcome (adj RR: 1.5; CI:
0.4–5.7; adj RR: 1.5; CI: 0.9–2.5) tended to occur more
frequently in preterm births with PROM. Our preliminary
results suggest that adverse neurological and respiratory
outcome have a higher incidence among preterm newborns
exposed to PROM.
FP-D-051
The early home-based intervention program on children
with fetal alcohol syndrome and fetal alcohol effect in
east Taiwan
H.-T. Kuo
Research Institute and Faculty of Early Education and
Care, Chaoyang University of Technology, Chinese Taipei
Fetal alcohol syndrome (FAS) is a clinical diagnosis
characterized by fetal pre-and postnatal growth delay,
dysfunction of central nervous system, and facial
dysmorphism due to maternal alcohol consumption during
pregnancy. Fetal alcohol syndrome was described by
Rouquette (1957), Lemoine (1968), and Jones (1973).
The fetal alcohol effect (FAE) was then proposed as a
mild form of alcohol teratogenesity by Smithells and
Smith (1984), Abel and Sokol (1987), and Streissguth
and Ladue (1987). The study was performed in Hualien
between 1997 and 1998. The subjects of the study included
six children with age under 6 years old, who lived in
Hualien County. There are five FAS and one FAE children
in our study group. We used Bayley’s Scale of Infant
Development (BSID, II), and CCDI for objective and
subjective developmental assessment. For learning status
we use Individualized Education Program Screening
Checklist (IEPSC) as assessment instrument. The Curriculum
base program including active and quiet activities was
arranged. At the end, the result was compared. The result
of BSID, CCDI, and IEPSC all shows no significant difference.
But, the results of CCDI and IEPSC items show
developmental progress in two assessment sessions. Our
conclusions on this study are (1). The FAS/FAE children
all shows subnormal mentality and is high-risk group for
future developmental disability, so they need early intervention
(2). Although the intervention effect is not statistically
significant, but there is qualitative developmental
progress. For the purpose of promoting developmental
potential and reducing load of family and society, we
would still like to suggest early intervention for all those
children.
FP-D-052
The numerical features of syndrome of hypertension
hydrocephaly and syndrome of hydrocephaly among
children with perinatal lesions of nervous system
B.A. Abeuov, Sh.A. Bulekbayeva, G.A. Mukhambetova,
A.S. Karimova, D.R. Purev
Kazakh National Medical University, Almaty, Kazakhstan
Quite often many symptoms of perinatal brain lesions are
barely recognizable and clinically not obvious on the early
stage, although they make effect on the neuro-psychological
growth of the child. In the current report we present the
results of clinical observation, diagnosis and treatment of
1087 patients aged from 1 month to 4 years with perinatal
pathology of central nervous system. To determine the aetiology
of the pathological process the anamnesis of the patients
was carefully learned by inquiring parents with the help of
specially developed questionnaire. The results of the
research revealed that different pathogenic factors are
present in perinatal period in 1045 cases, among which the
intranatal hypoxia is on the first place, on the 2nd place is the
combination of the hypoxia in the 2nd half of the pregnancy
with the trauma during travail, on the 3rd place – birth
trauma. In 3.8% of cases the cause of the nervous system’s
pathology was not succeeded. It is not enough to make a
visual qualitative analysis of CT-scans to determine the
pathological substrate. That’s why we used the quantitative
method which has revealed the following: the dilation of the
ventricular system, especially of the index of anterior horns
of the lateral ventricles (IAHLV) on (17.4), took place. The
indices of cerebral coat at the level of anterior horns and
corpuses of lateral ventricles were reduced accordingly.
The reduction at the level of posterior horns was less noticeable.
Also the considerable dilation of all liquor areas was
determined with the high degree of certainty. The considerable
dilation from the direction of subarachnoid interspace
and apertures of Silvii was revealed in the group of patients
with hydrocephaly. Among children with syndrome of
hypertension-hydrocephaly the indices of ventricular system
increased moderately, which caused the reduction of indices
of the cerebral coat. IAHLV most changed – on (14.3), that is
essentially less than in the group of children with syndrome
of hydrocephaly. Thus, syndrome of hydrocephaly in
comparison with syndrome of hypertension-hydrocephaly
in cases with perinatal cerebral lesions characterizes by
more considerable changes of the indices of the ventricular
system, indices of cerebral coat and sizes of liquor areas.
FP-D-053
Congenital pathology of nervous system in children
U.A. Karimov, N.A. Zakirova, D.S. Aripova, M.K.
Saidakhmedov
Scientific Research Institute of Pediarics, Tashkent, Uzbekistan

Abstracts 457
Analysis of congenital pathology of nervous system in
children under conditions of Tashkent region revealed the
level of the most occurring congenital pathologies of
nervous system at the time of birth. Among 346 live-birth
newborns Spina bifida was noted in 6.9%, among 80 stillborns-in
2.5% and among 40 newborn dead during 0 , 6
day after birth-in 6.2% of cases. Microcephaly was found in
3.7, 10 and 2.5% of newborns, respectively. Hydrocephalyin
4.6, 10 and 1.25% of cases, respectively. The Down
disease was determined in 5.8% and disease of peripheral
nervous system-in 3.5% only in livebirth newborns. Thus,
more early diagnosis before school age and prophylaxis of
congenital pathology of nervous system with determination
of risk factors are very important for reduction of abovementioned
indicators of nervous system disease in children.
FP-D-054
Identification of congenital pathology of nervous system
in children in relation to age in the family polyclinics
N.A. Zakirova, U.A. Karimoy
Scientific Research Institute of Pediatrics, Tashkent, Uzbekistan
Long-term observation of 223 340 children aged from 1 to
14 years in the family polyclinics in Tashkent (Uzbekistan)
revealed congenial diseases and malformations of different
organs and systems in 778 (0.35%) children. Among them the
diseases of nervous system have the special place. Among the
children with congenital pathology of nervous system being
under long-term observation of neuropathologist, we carried
out analysis of their incidence in relation to age. It was found
that Down disease was diagnosed more often in children
under 3 years of age, microcephaly was determined in all
the age groups with equal frequency, DYN was noted more
frequently among children aged from 3 to 7 years. Thus, the
data obtained allow to organize rehabilitation of congenital
pathology of nervous system in relation to age and dynamic
observation in all the age groups for determination of congenital
diseases of nervous system.
FP-D-055
Neuropsychological outcome of 9–10 year-old children
with intrauterine growth retardation (IUGR):
preliminary findings
R. Geva a,b , R. Eshel a , Y. Leitner a , A. Fattal-Valevski a ,M.
Varon a , Y. Vila a , O. Bitchuniski a , B. Radiano a ,K.
Goldberg a , S. Harel a
a Institute for Child Development and Pediatric Neurology
Unit, Division of Pediatrics, Dana Children’s Hospital, Tel
Aviv Sourasky Medical Center and Sackler school of Medicine,
Tel-Aviv University, Tel Aviv, Israel; b Department of
Psychology, Bar-Ilan University, Ramat-Gan, 52900, Israel
The objective of the study was to trace the neuropsychological
profile of 9–10 years-old children with IUGR
(N ¼ 56) compared with children who are matched for
gestational age and socio-economic factors (N ¼ 31).
Fifty-six children with IUGR were followed-up prospectively
to the age of 9–10 years. They underwent comprehensive
neuropsychological evaluation (lasting 4.5–5 h).
The preliminary results show that the cognitive competence
of most children with IUGR are within normal limits,
however their school achievements are significantly low,
evident in general knowledge, reading decoding and
comprehension and arithmetic. The neuropsychological
analysis showed that these difficulties arise from lower
verbal skills (Ps range ¼ 0.001–0.023), a deficit in shortterm
memory span for auditory stimuli (P , 0:026),
visuo-motor perceptual deficits (P , 0:01), graphomotor
difficulties (P , 0:004) and deficits in executive functioning
(P , 0:003). Both risk groups had difficulties in visual
attention and short-term memory for visual abstract material
as well as executive rigidity. Hence, three neuropsychological
domains impede learning in children with IUGR:
short-term memory for verbal material as proposed by
Pennington (1991), deficiency in executive functioningwas
suggested by Barkley (1997) and visuo-motor, primarily
graphomotor deficits-indicated by Denckla.
FP-D-056
Cerebral palsy and twin pregnancy: zygosity, risk
factors, clinical course and MRI findings
D.I. Zafeiriou, E. Vargiami, A. Vardarinos, E. Pavlou, E.
Kontopoulos, I. Tsikoulas
1st Department of Pediatrics, Aristotle University, ‘Hippokratio’
Hospital, Thessaloniki, Greece
The prevalence of twinning and other multiple births is
increasing all over the world, due at least in part to fertility
enhancing medical therapies. Twins and multiplets are at a
higher risk for intrauterine and neonatal death than singletons
and are also at increased risk for neurologic morbidity,
including CP. In a partly retrospective, partly prospective
study during the year period 1984–2001, we identified 149
patients with CP who were the product of a twin pregnancy
(80 males, 69 females; age range 3–13 years). The incidence
of CP due to twinning in the whole CP population taking
care of during the same period (N ¼ 1128) was estimated to
be 13.9%. A total of 117 CP twins (78.5%) were of the same
sex as their co-twin, while only 32 (21.5%) were of the
opposite sex. Eight-six patients (57.7%) had caesarean
section; 68 patients (45.6%) were A twins, while the rest
81 patients (54.4%) were B twins. The vast majority of
patients were monozygotic twins (N ¼ 85; 57.0%)
compared to dizygotic (N ¼ 64; 43.0%). Forty-two twins
(28.2%) had a birth weight (BW) , 1500 g, 82 (55.0%)
between 1500 and 2500 g, and only 25 (16.8%) . 2500 g.
A total of 130 twins were preterm (,37 weeks) and 19 were
full-term (.37 weeks). Regarding the fate of the co-twin, it

458
Abstracts
was normal 68 cases (30 monozygotic, 38 dizygotic;
P , 0:05), the co-twin had CP in 40 cases (31 monozygotic,
nine dizygotic; P , 0:05), there was intrauterine death in 18
cases and death in the first 6 months of life in 23 cases.
Monozygotic twins had smaller mean BW compared to
dizygotic ones; the same was true for normal co-twins of
dizygotic twins compared to normal co-twins of monozygotic
ones (P , 0:05, respectively). Sixty-one patients had
spastic diplegia, 54 had tetraplegia, 26 had hemiplegia and
eight had dystonia. Additional impairments and disabilities
included mental retardation (47.7%), speech impairment
(69.1%), visual impairment (31.5%), hearing impairment
(9.4%), epilepsy (26.2%) and orthopedic problems
(81.9%). MRI of the brain demonstrated prenatal type findings
in 51% of the patients (porencephaly, PVL and combination),
perinatal type findings 36.2% (cystic or subcortical
leucomalacia, cortical atrophy, basal ganglia involvement
and combinations) and dysgenesis in 12.8% of the patients.
Monozygotic twins demonstrated more frequent prenatal
findings and less frequent infarcts compared to dizygotic
(P , 0:05, respectively). We conclude: (1) Twins of the
same sex seem to be at increased risk for CP compared to
those of opposite sex; (2) birth order is not associated with
CP; (3) monozygotic and SGA dizygotic twins are at
increased risk for CP; and (4) intrauterine death of a monozygotic
twin increases significantly the risk for CP in the
surviving co-twin.
FP-E
Nutrition
FP-E-001
Dietary intake and blood folate levels in Honduran
women of childbearing age
K.R. Holden, J.S. Collins, J.F. Greene, S. Hinkle, J.M.
Portillo, R.E. Stevenson
Greenwood Genetic Center, Mt Pleasant, USA
Neural tube defects (NTDs) are common birth defects
whose frequency appears to be reduced by maternal supplementation
and/or fortification of folic acid. Latin Americans
have a high incidence of NTDs, with Honduras having an
estimated NTD prevalence of 2.6 per 1000 live births. A
recent Center for Disease Control study demonstrated that
folic acid supplementation and/or fortification appears effective
in the US Hispanics to reduce the risk of NTD recurrence.
We surveyed the dietary intake of 231 Honduran women of
childbearing age using a 24-h dietary recall questionnaire in
inner city, town, and country areas. We also randomly
checked 24 blood folates in the surveyed population to
compare to the normal range for the US population. Normal
US recommended dietary allowance intake of folic acid
equal to 292.1 ^ 152.0 mcg was documented in Honduran
women in association with a low intake of many other essential
nutrients. There were also differences in nutrient intake in
city, town, and country areas. Serum (6.0 ^ 3.3 ng/ml) and
red blood cell (203.3 ^ 61.6 ng/ml) folate levels in all locations
were in the low normal range when compared to the presupplementation/pre-fortification
US population. Even when
considering the multiple socio-economic hurdles presented
by this developing country, our data support using an established
folic acid fortification public health initiative to
decrease the prevalence of NTDs in Honduras.
FP-E-002
Malnutrition increases dentate granule cell neurogenesis
in immature rats after status epilepticus
Y.-L. Wang, R.-P. Sun, G.-F. Lei, J.-W. Wang, B.-M. Li
Department of Pediatrics, Shandong University Qilu Hospital,
Jinan, China
Several experimental seizure inductions that produce
epilepsy as a consequence have been shown to be always
associated with the proliferation of dentate granule cells. To
investigate the effects of prolonged seizures on dentate granule
cell proliferation, and to study the associations among
malnutrition, granule cell proliferation and epilepsy, we
examined proliferating cells using bromodeoxyuridine
(BrdU) mitotic labeling combined with immunohistochemistry
for markers of immature granule neurons (TuJ1) and
mature granule neurons (calbindin). We maintained rat pups
on a starvation regimen from P2 to P22. The status epilepticus
was elicited by unilateral microinfusion of kainic acid
(1 mg) into the amygdula at P15. Rat pups were given BrdU
twice daily for 4 days beginning 3 days after status epilepticus
to label newly born cells. At P22, the rat pups were
killed and the brains were processed for dual-labeling
experiments. The results indicated that early malnutrition
did not modify seizure susceptibility and behavioral manifestations.
Although exposure to status epilepticus augumented
the expression of new cells in the dentate gyrus of
the immature rats, this expression was more pronounced in
the malnourished rat pups. Most BrdU-labeled cells coexpressed
neuro-specific markers (TuJ1 or calbindin). The
findings suggest that malnutrition followed by status epilepticus
can further increase granule cell proliferation, furthermore,
the vast majority of these mitotically active cells
differentiated into neurons in the dentate granule cell layer.
FP-E-003
Effect of iron deficiency on brain monoamine
neurotransmitters in rats
R.-M. Hu, M.-W. Wei, R.-P. Sun
Department of Pediatrics, Qilu Hospital of Shandong
University, Jinan, China
Objective: To identify the mechanism of iron deficiency on
learning and memory ability in brain. Methods: An iron deficient
diet (11.9 mg/kg) was used to make an animal model of

Abstracts 459
iron deficiency. The contents of iron in brain and liver were
determined by DCP-AES technique. An enzyme histochemical
method was used to test monoamine oxidase (MAO)
activity. Monoamine neurotransmitter and its metabolites
were determined by high performance liquid chromatography
with electrochemical detection (HPLC-ECD). Results:
The brain iron content(11.2 ^ 5.5 versus 20.7 ^ 6.5 mg/g
wet tissue, P , 0:001) and MAO activity were both
decreased in iron deficient non-anemic rats. The levels of
norepinephrine (NE) and 5-HT in cerebral cortex were
significantly higher than those of controls (269.2 ^ 46.9
versus 208.4 ^ 27.3 ng/g wet tissue, P , 0:05,
1568.8 ^ 234.8 versus 1194.1 ^ 163.4 ng/g wet tissue,
P , 0:01), while hydroxyindoleacetic acid (5-HIAA), a
metabolite of 5-HT in the hippocampus, was lower than
that of controls (2753.7 ^ 365.4 versus 3623.4 ^ 754.1 ng/
g wet tissue, P , 0:05). These changes were more significant
in anemic rats. Conclusion: The result suggested that the
abnormal metabolism of monoamine neurotransmitters in
brain may be a biochemical basis for learning and memory
defect caused by iron deficiency.
FP-E-004
Intracranial hemorrhage infarction due to late onset
Vitamin K deficiency-11 case studies
S.-Z. Xu, H.-H. Zhang, X.-X. Liu
Department of Pediatrics, Affiliated Hospital, Binzhou
Medical College, Binzhou, China
Objective: To summarize the clinical manifestations,
etiology, diagnosis and prognosis of 11 cases with intracranial
hemorrhage infarction which were diagnosed in our
hospital in the past 5 years. All cases were breast feeding,
seven cases had infantile hepatitis syndrome. These were
the common causes of the late onset vitamin K deficiency.
Among seven cases of infantile hepatitis syndrome, four
were CMV-IgG antibody positive. Prenatal or perinatal
CMV infection was an important cause of hepatitis
syndrome. The onset of Vitamin K deficiency in these
cases was between 31 and 70 days. The clinical manifestations
included convulsions, drowsiness, anorexia, irritability,
pallor, bulging of the anterior fontanelle, hypertonia and
external bleeding. Laboratory examination: coagulation
time .15 min. Twenty-four hours after vitamin K1 administration,
it became normal in all cases. Thrombocytosis was
found in eight cases (.350 £ 109/l). CT studies showed left
cerebral infarction after hemorrhage in nine cases, multiple
infarction after hemorrhage in two cases. Multiple intracranial
heamorrhage was seen in eight cases, mild hemorrhage
and quickly absorbed in three cases. Large cerebral infarctions
remained. The hemorrhage or hematoma was absorbed
in 2 weeks, but there were still low density areas on CT.
These areas enlarged in three cases 3 months later and encephalomalacia
formed. Typical clinical manifestations,
coagulation time test and CT are simple and reliable diagnostic
methods. The follow-up CT played an important role
in prognosis evaluation. Early administration of Vitamin K1
is effective in prevention this disease.
FP-F
Cytogenetics/Teratology
FP-F-001
Bilateral frontoparietal polymicrogyria
L. Sztriha, M. Nork
Department of Paediatrics, FMHS, UAE University and
Department of Radiology, Tawam Hospital, Al Ain, United
Arab Emirates
Polymicrogyria is a form of non-lissencephalic cortical
dysplasia with abnormal gyration characterized by narrow
and crowded gyri. According to a recent classification of the
malformations of cortical development, polymicrogyria is a
result of abnormal cortical organization. It can be either a
generalized or focal unilateral or bilateral lesion. Among the
rare, bilateral symmetrical forms several syndromes, such as
bilateral perisylvian polymicrogyria, bilateral parasagittal
parieto-occipital polymicrogyria and bilateral frontal polymicrogyria
have been recognized and characterized. Bilateral
polymicrogyria in other cortical areas seems to be even
more infrequent; only a few cases of large, bilateral frontoparietal
polymicrogyria have previously been described.
The clinical and radiological findings in three patients
with bilateral frontoparietal polymicrogyria and epilepsy
are presented. The patients had serious delay in mental
and motor development and seizures with epileptiform electroencephalographic
findings. The MRI revealed bilateral
abnormalities in the frontal and parietal lobes. The cortex
was thick with an irregular outer surface and multiple small
gyri and sulci were recognizable. The cortical-subcortical
junction showed a festoon-like appearance. The abnormal
cortex extended bilaterally from the frontal pole to the
parieto-occipital fissure medially and to the level of the
lateral cerebral fissure (fissure of Sylvius). The volume of
the underlying white matter was reduced, and the lateral
ventricles were moderately dilated. Bilateral frontoparietal
polymicrogyria might represent a variety of the congenital
bilateral symmetrical polymicrogyria syndromes in addition
to perisylvian, parasagittal parieto-occipital and frontal
polymicrogyria.
FP-F-002
The study of telomeric microaberrations in
chromosomes of children with undifferentiated forms of
mental retardation
S.G. Vorsanova, Y.B. Yurov, A.D. Kolotii, A.K. Beresheva,
I.V. Soloviev
Institute of Pediatrics and Children Surgery, Russian Ministry
of Health and National Research Center of Mental

460
Abstracts
Health, Russian Academy of Medical Sciences, Moscow,
Russia
Molecular-cytogenetic studies of chromosomal microanomalies
at subtelomeric chromosomal regions have been
conducted by two-step fluorescence in situ hybridization
(FISH) in 407 children with mental retardation and apparently
normal chromosome complements. A large set of
cosmid, BAC and PAC clones, containing telomeric
sequences was identified by FISH studies. Availability of
large-insertion PAC probes, which contain DNA sequences,
common for telomeric and, partially subtelomeric regions of
all human chromosomes allowed us to mark all telomeric
regions in one FISH study. Therefore, first FISH study for
each patient was performed with large-insertion PAC probe,
specifically marking telomeric and subtelomeric regions of
all human chromosomes. If some chromosome arm had
absence of telomeric hybridization signals, or the intensity
of hybridization signal was reduced in comparison to homologous
chromosome arm, we used second DNA probe,
which is strongly specific todefinite telomeric region of
individual chromosome. Microdeletions of chromosomes,
involving telomeric regions were detected in 20 (4.8%)
cases from 420 patients with severe and mild forms of
mental retardation and apparently normal karyotypes
revealed by classical banding techniques. Microdeletions
were detected in the following chromosomal loci: 4q35.2;
5p15.33 (two cases); 9q34.3; 10q26.3 (two cases); 11q25;
13q34; 16q24.3 (two cases); 18p11.32 (four cases); 18q23
(two cases); 21q22.2 (two cases) and 22q13.33 (two cases).
These data demonstrate that undifferentiated forms of
mental retardation could be associated with non-specific
telomeric micro-aberrations of different chromosomes.
FISH studies using specially developed panel of telomeric
DNA probes could be useful for identification of generally
undetermined forms of mental retardation.
FP-F-003
Bilateral perisylvian atrophy – a study of 11 cases from
Oman
R.A. Syed, S.M. Khusaiby
Department of Child Health, Royal Hospital, Muscat, Oman
Recent advances in neuroimaging have helped the clinician
to correlate clinical features with radiological findings
in various neurological disorders. This study describes 11
children with developmental delay with no known etiology
who showed bilateral perisylvian abnormalities in imaging
studies. Most of the patients had spastic weakness of the
limbs, microcephaly, severe developmental retardation
and seizures, as common symptoms. Speech and language
disorders along with masticatory and swallowing problems
were encountered in 85% of the patients. Three patients had
generalized dystonia with choreic movements mostly seen
in the facial and trunk muscles. Further studies are required
to evaluate the definite etiology.
FP-F-004
Pontocerebellar hypoplasia type 1: new leads for an
earlier diagnosis
M.S. Salman, S. Blaser, J.R. Buncic, C.A. Westall, E. Heon,
L. Becker
University of Toronto and Hospital for Sick Children,
Toronto, Canada
Background: Pontocerebellar hypoplasia (PCH) type 1 is
a rare disease characterized by PCH and anterior horn cell
degeneration. Death results from respiratory failure. The
oldest reported child died at the age of 26 months. Methods:
Case study of two siblings with PCH. The diagnosis was
made on autopsy after the death of the second sibling at 40
months from respiratory failure and the finding of anterior
horn cell degeneration on autopsy. The older sibling died at
14 months from pneumonia following a clinical course similar
to his sister. Results: Both siblings had significant global
developmental delay with initial axial and peripheral hypotonia.
Peripheral hypertonia with brisk reflexes developed
later. Extensive investigations in the second sibling ruled
out mitochondrial and other metabolic diseases. Genetic
testing for Friedreich’s ataxia, spinal muscular atrophy,
neuropathy/ataxia/retinitis pigmentosa (NARP), spinocerebellar
ataxia 1–3 and 6–8 gene abnormalities were negative.
Sleep study showed mixed central and obstructive sleep
apneas. The electroretinogram (ERG) showed abnormal
rod-cone response that was consistent with retinal dystrophy.
Conclusions: Our cases call for the expansion of the
phenotype for this disease. Survival may be prolonged with
emergent spasticity. The presence of abnormal ERG
response in infants with PCH may aid in the earlier diagnosis
of this disease, and provide new insights into its pathogenesis.
FP-F-005
The brain complications of immunodeficiency in
Nijmegen breakage syndrome
J. Wendorff, E. Hibner, K. Zeman, M. Piotrowicz
Research Institute, Polish Mother’s Memorial Hospital,
Lodz, Poland
Immunodeficiency is one of the most important symptoms
of Nijmegen breakage syndrome (NBS). Since the
description of the first patient approximately 70 cases
have been reported, mainly in Central Europe. The diagnostic
criteria are: characteristic phenotype with mental retardation,
humoral and cellular immunity disorders, increased
predisposition to cancer and chromosomal instability. The
most frequent clinically manifested NBS complications
result from predisposition to infections. The brain compli-

Abstracts 461
cations of immunodeficiency have not been reported up till
now. We present a 13-year-old girl with decreased levels of
immunoglobulins in all classes, and B and T lymphocytes,
in whom NBS was diagnosed on the basis of typical phenotypic
features and genetic examination (deletion 5 nt in gene
NBS 657–666 position). During 2 year follow-up three
different neurological syndromes were diagnosed: chronic
headache due to disturbance of CSF reabsorption, sinus
cavernosus thrombophlebitis and cerebellitis. The occurrence
of neurological symptoms was connected with lack
of proper immunosubstitution due to non-compliance. All
these neurological syndromes were clinically oligosymptomatic
or asymptomatic while the brain MRI showed considerable
involvement. The duration of neurological
complications was short and they disappeared after treatment
with immunoglobulins and steroids. Conclusion: The
occurrence of neurological signs and symptoms in NBS may
be related to immunodeficiency caused by lack of proper
immunosubstitution.
FP-F-006
Observation of some cases of Klippel-Trenaunay
syndrome
S.S. Shomansurov, Z.B. Rafikova, G.T. Hojaeva, S.R.
Rafikova
Department of Pediatric Neurology, Tashkent Institute of
Post-graduate Medical Education, Tashkent, Uzbekistan
Klippel-Trenaunay syndrome was first described in 1900.
Main clinical manifestations are asymmetric limb hypertrophies
and hemangiomas due to congenital malformations of
arteriovenous and lymphatic vessels. We observed 5 children
from age 2 months to 14 years, who underwent clinical and
neurological examination, brain ultrasonography (BUS),
computed axial tomography (CAT), and symptomatic treatment.
Follow-up lasted for 3 years. Clinical findings varied
from minimal to strongly pronounced. In two patients there
were no intracerebral hemangiomas. These patients developed
satisfactorily physically and mentally. Three patients
had cerebral hemangiomas and demonstrated developmental
retardation and secondary hydrocephalus, and required careful
management. BUS let us monitor forming and growth of
hydrocephalus relevant to location of intracerebral angiomas.
CAT showed enlargement of affected brain hemisphere
in two patients without signs of structural anomalies or
progress. The severity of the disease depended on craniocerebral
angiomatosis; therefore we identified clinical
variants of the syndrome with affected extremities only and
with affected extremities plus brain. Follow-up observation
and symptomatic management allowed us to achieve stabilization
of pathologic process and improvement of neurological
status in all children. So, progress of the disease of
Klippel-Trenaunay syndrome is determined first of all by
the severity of cerebral anomalies, and opportune symptomatic
therapy improves patients’ condition and prognosis.
FP-F-007
Neuronal migration anomalies in children: a study of 43
patients
W.-H. Xie, Y.-C. Chen, A.-D. Zheng, X.-J. Hou
Department of Pediatrics, First Affiliated of Fujian Medical
University, Fuzhou, China
The aim is to study the clinical and neuroimaging findings
of children with neuronal migration anomalies
(NMA). The medical records of 43 children with NMA
seen in our hospital during the past 12 years were
reviewed. There were 35 boys and 8 girls. Age ranged
from 2 months to 12 years. The major clinical presentations
of these patients were cerebral palsy (51.2%),
seizures (32.5%), and mental retardation (16.3%). To
study the characteristics of craniocerebral CT and MRI
findings in these migration anomalies, CT images of 43
patients with NMA were evaluated. Five patients were
studied by MRI. Twenty had schizencephaly (46.5%), 14
patients had lissencephaly and/or pachygria (32.6%). Five
had polymicrogyria (11.6%), four had heterotopias of gray
matter (9.3%). Twenty-six patients (60.5%) had other associated
brain anomalies, including absence of the septum
pellucidum in 14 cases, absence or agenesis of the corpus
callosum in eight cases, cyst of the septum pellucidum in
three cases, holoprosencephaly in two cases, and arachnoid
cyst in two cases. MRI is a more superior method for
diagnosing NMA than CT. It can clearly delineate the
detailed morphologic changes of the brain caused by
neuronal migration disorders as well as the other associated
anomalies.
FP-F-008
Dubowitz syndrome
B. Jocic-Jakubi, G. Jovanovic, V. Micic
Clinic of Mental Health and Child Neuropsychiatry,
Department of Child Neurology, University Hospital, Nis,
Yugoslavia
Dubowitz syndrome is an autosomal recessive disorder
characterized by small stature, peculiar face, infantile
eczema, microcephaly and mild mental retardation, originally
described by Dubowitz (1965). We report a 7-year-old
boy with postnatal growth retardation, characteristic facies,
micrognathia, short palpebral fissures and asymmetric
ptosis. Hypotonia, hyperextensible joints, clinodactyly of
fifth finger were present. Eczema, speech delay, submucous
cleft palate and delayed skeletal maturation were noted in
early childhood. His mother has craniofacial anomalies too,
but no intelligence abnormalities. In their family there
were mentally retarded persons and one sibling died at
birth with congenital malformation. The differential diagnosis
is discussed with respect to this very polymorphous
syndrome.

462
Abstracts
FP-F-009
One case of Xp21 contiguous gene deletion syndrome
H.-W. Ma a , J. Jing a , Z.-C. Wang a , L.-Y. Chen a , M. Matsuo b
a Laboratory of Genetics, The Second Clinical College,
China Medical University, Shenyang, China; b Division of
Genetics, International Center for Medical Research, Kobe
University School of Medicine, Kobe, Japan
We describe a Chinese boy previously misdiagnosed with
adrenoleukodystrophy who was subsequently diagnosed
with Xp21 contiguous gene deletion syndrome, which
included congenital adrenal hypoplasia (CAH), glycerol
kinase deficiency (GKD) and Duchenne muscular dystrophy
(DMD). His peripheral blood routine karyotyping was without
visible deletions. Molecular mapping in the Xp21 region
revealed that the deletion encompassed nearly the whole
region from a portion of 3 0 end of the DMD gene to a site
telomeric to the locus for X-linked CAH. The centromeric
startpoint of the deletion was localized to the 3 0 end of the
DMD gene, between exons 62 and 63, while the telomeric
deletion breakpoint was not determined in the present study.
However, no evidence for the deletion of the GKD gene
located between the DMD and the CAH was observed,
which might be fortuitously caused by its pseudogene. Interestingly,
the deletion of the marker DXS992 located
between the DMD and the GKD was not detected too,
which could have resulted from either the presence of a
separate double deletion or the mechanism of other complex
deletion within this region. The exact mapping of the deletion
could be confirmed by FISH and this study is underway
in our patient. These findings suggest that Xp21 contiguous
gene deletion syndrome should be further considered in any
patient with adrenal insufficiency in spite of the normal
karyotype. Analysis of molecular genetics will help in determining
the extent of the deletion.
FP-F-010
Kabuki syndrome (Niikawa-Kuroki): study of five
Spanish cases
S.M. Briceño-Cuadros, J. Campos-Castelló, E. Miravet-
Fuster, G. Bueno-Lozano, O. Pérez-Rodriguez
Hospital Clinico San Carlos, Madrid, Spain
Objectives: The Kabuki Syndrome, described by Niikawa
and Kuroki (1981) has an estimated prevalence of 1:32 000
live births. No biological marker is available yet. The diagnosis
is exclusively based on phenotypic features: facial
dysmorphism resembling the make-up used by kabuki
actors, fetal flesh on finger tips, dermatoglyphic abnormalities,
postnatal failure to thrive, and osseous malformations.
It is associated with variable frequency of ophthalmologic,
dental, cardiac, genitourinary, ENT and neurologic abnormalities.
The latter include mental and language delays,
epilepsy, migration defects and hydrocephalus. A hundred
cases have been reported in Japanese, European and North
American patients. We report five Spanish cases and a
review of the literature. Results: We present five children
(four males, one female) with a history of hypotonia, delay
attaining developmental milestones, mild cognitive impairment
and language with mostly phonologic and syntactical
problems and excentric prosody. Facial dysmorphic features
include arched eyebrows, eversion of external third of inferior
eyelid, enlarged ears, high and depressed nasal bridge,
high arched palate and fetal flesh on finger tips. Short height
and skeletal abnormalities are variable in frequency. Chromosomal,
hormonal, hematologic and metabolic tests were
normal. Imaging studies (CT, MRI) only showed a mild
atrophy in deep grey matter and cortex in two patients.
Conclusion: Kabuki syndrome is relatively easy to recognise
because of its dysmorphic features. It is a multiorgan
disorder that involves the central nervous system whose
most frequent clinical manifestations are mental and
language delay.
FP-F-011
The FG syndrome: report of a large Italian series
A. Battaglia
Stella Maris Scientific Research Institute Division of Child
Neurology and Psychiatry, University of Pisa, Pisa, Italy
FG syndrome was first described by Opitz and Kaveggia
in 1974, as a rare multiple congenital anomalies/mental
retardation (MCA/MR) syndrome occurring only in boys,
due to a recessive mutation on the X chromosome. Based on
reports of over 50 reported cases, FG syndrome is associated
with developmental delay (especially speech), hypotonia,
postnatal onset relative macrocephaly, prominent forehead,
frontal hair upsweep or whorls, ocular hypertelorism, thin
vermilion border of the upper lip, relatively short fingers
with broad thumbs and halluces, persistent fetal fingertip
pads, anal anomalies, and/or constipation. Major malformations
are rare, and include pyloric stenosis, anal agenesis,
cryptorchidism, hypospadias, inguinal hernias, and congenital
heart defects. Abnormal EEG findings and seizures
have been reported in almost 70% of patients. MRI studies
commonly show abnormalities of the corpus callosum associated
with variable dilatation of the lateral ventricles; while
less frequent are periventricular nodular heterotopias, mild
cerebellar defects, and reduced amounts of periventricular
white matter. Chiari I malformation seems to be a rather
frequent finding. The behavior phenotype appears to be
characterized by attention deficit/hyperactivity disorder,
and relatively less developed language, fine motor and
executive function skills, whereas visual-spatial abilities
seem to be a relative strength. At present, due to an
improved awareness, FG syndrome emerges as a rather
common MCA/MR syndrome, occurring in boys and girls,
presumed to be due to incompletely recessive mutations of
genes on chromosome X. However, in a few instances

Abstracts 463
inheritance from an affected father has been suspected. Two
or three candidate loci on chromosome X are already at
hand, making it possible the cloning of causal gene(s) in
the near future. We describe twenty patients, referred to our
Institute for investigation of developmental delay/mental
retardation, and diagnosed as FG syndrome. In all of them
diagnosis was confirmed by Dr Opitz. This is the first
reported Italian series of FG syndrome cases, with particular
emphasis on physical, neurological and developmental findings,
and natural history. Follow-up on all patients ranged
between 6 months and 7 years.
FP-F-012
Functional and genetic interactions between
doublecortin and lissencephaly-1 (LIS1) in neuronal
migration
T. Tanaka a , C. Paczkowski a,b , F.F. Serneo a , S. Sasaki c , M.J.
Gambello d , S. Hirotsune c , A. Wynshaw-Boris b,d , J.G.
Gleeson a,b
a Department of Neurosciences,
b Graduate Program in
Neurosciences, d Departments of Pediatrics and Medicine,
University of California, San Diego, USA; c Center for
Genome Medical Science, Saitama Medical School,
Saitama, Japan
Neurons destined for cerebral cortex must migrate
hundreds of cell-body distances to reach their final destination.
Neuronal migration is dependent upon doublecortin
(DCX) and lissencephaly-1 (LIS1), as humans with mutations
in either gene display defective migration. However,
very little is known about the molecular events underlying
neuronal migration and its disorders. Here we show that
LIS1 and DCX function on a common pathway in neuronal
migration. We adopted a cerebellar granule cell migration
assay system with retroviral transduction of either wild type
proteins or fluorescently-tagged proteins to study the effect
of gene overexpression and to determine the subcellular
localization of these proteins during cell migration in
vitro. To study the effect of loss of function of LIS1, we
utilized neurons from LIS1 deficient mice. LIS1 deficient
neurons displayed a dose dependent decrease in migration
distance. Overexpression of LIS1 or DCX in wild type (WT)
neurons resulted in increased neuronal migration distance.
DCX overexpression rescues the migration defect in LIS1
deficient neurons. These results suggest that LIS1 and DCX
have dosage-sensitive effects on neuronal migration in both
deficiency and overexpression.
FP-F-013
Angelman syndrome: a case report
M.H. Ortiz, E.C. Cutiongco, S.G.P. Perez
Neurodevelopmental Section, Child Neuroscience Division,
Philippine Children’s Medical Center, Quezon City, Philippines
Angelman syndrome (AS) is a neurological disorder associated
with severe developmental delay, speech impairment,
gait ataxia and characteristic behavior of inappropriate
laughing and smiling. It affects approximately 1 in
12 000–20 000 individuals and is caused by the loss of
maternally imprinted contribution in the 15q11–13 region
which can occur by at least five different known genetic
mechanisms. This is a case report of a 4.5-year-old Filipino
boy seen at the Philippine Children’s Medical Center. He
presented with feeding problems (2 months), developmental
delays (4 months), frequent smiling (9 months), seizures
(2.6 years), and sleep disturbance (4 years). Pertinent physical
examination revealed microcephaly (47 cm P , 2 Nellhaus),
wide mouth, wide-spaced teeth and prominent
mandible. Neurological examination showed an inappropriate
happy demeanor, fleeting eye contact, short attention
span, hyperactivity, monosyllabic utterances, drooling,
and mouthing. On gait testing, ataxia was noted with arms
abducted and elbows flexed. Deep tendon reflexes were
hyperactive. Diagnostic investigations revealed gastroesophageal
reflux on barium swallow; generalized and multifocal
epileptiform discharges and focal and generalized
slowing of the background activity on EEG; and normal
cranial MRI. Cytogenetic study revealed a normal karyotype
on routine high resolution G banding which was then
augmented with FISH technique using the probes small
nuclear ribonucleoprotein and D15S10 loci. On FISH analysis,
a deletion was detected on one of his chromosome 15
confirming the diagnosis of Angelman Syndrome. This may
represent the first reported genetically-confirmed AS in the
Philippines.
FP-F-014
Angelman syndrome in Malaysian children
M.-K. Thong
Department of Paediatrics, University of Malaya Medical
Centre, Kuala Lumpur, Malaysia
AS is a neurodevelopmental disorder characterized by
severe intellectual disability, movement or balance disorder,
microcephaly, speech impairment with absent or minimal
speech and seizures with characteristic electroencephalogram.
Earlier reports emphasized the typical behavioral
phenotype such as the ‘happy’ disposition, frequent laughter,
hand-flapping movements and hyperactivity. Consensus
for diagnostic criteria for AS included a normal antenatal
and birth history, normal head circumference at birth with
absence of major birth defects, on-going developmental
delay evident by 6–12 months of age with no regression,
normal laboratory investigations and absence of structural
brain abnormalities. Other associated clinical features
present in 20–80% of patients include flat occiput, deepset
eyes, prognathism, wide mouth with widely spaced
teeth, fair skin and hair, hyperreflexia and drooling. Diagnosis
is based on diagnostic criteria and usually difficult in

464
Abstracts
the first 2–3 years of life. Other conditions with similar
presentation include Rett syndrome, autism, Lennox-
Gastaut syndrome and cerebral palsy. Currently, molecular
diagnosis with DNA methylation study has improved the
accuracy of the diagnosis. However, most reports are
centred upon Caucasian children and there are very few
reports of Angelman syndrome in Asian children. We report
our series of Malaysian children of Malay, Chinese and
Indian ethnic background with Angelman syndrome.
FP-F-015
A case of Niikawa-Kuroki syndrome with chromosomal
abnormality 46, XY, add (22)(p11,2)
M. Sakurai a , H. Satoh a , I. Ichihashi a , K. Yamaguchi a ,T.
Takeuchi a , J. Furusho a , Y. Iikura a , K. Kumagai a,b
a Department of Pediatrics, School of Medicine, Showa
University, Tokyo; b Chisaki-Hanano-Sono, Institution for
Social Welfare Corporation, Japan
Niikawa-Kuroki syndrome (Kabuki make-up syndrome)
is characterized by unusual face, mental retardation, skeletal
anomalies, dermatoglyphic abnormalities, and postnatal
growth retardation. However no certain causal factor has
been identified in this syndrome. We report 1 year old boy
case of Niikawa-Kuroki syndrome with chromosomal
abnormality (46,XY, add (22)(p11,2)). He was the second
child of healthy parents. Facial anomalies, skeletal anomalies
and pulmonary artery stenosis were found at birth.
Cataract, mild mental retardation, growth retardation,
dermatoglyphic abnormalities, and dilatation of sylvian
fissure on cranial MRI are noted now. His parents have
no chromosomal abnormalities. Thirteen cases of chromosome
abnormalities in associated with Niikawa-Kuroki
syndrome have been reported. In summary, dominant
inheritance, ring (X) chromosomes and a severe phenotype,
Turner syndrome and pseudoautosomal dominant inheritance
are speculated. In our case, it is very interesting
that his chromatin abnormality is localized to the autosomal
chromatin area (p11,2).
FP-F-016
Dandy-Walker malformation, hypertrophic
cardiomyopathy and cranial anomalies in a newborn: a
new case of 3C syndrome
F. Fabbri, C. Galasso, A. Di Paolo, L. Cristina, F.
Silvestrini, P. Curatolo
Department of Pediatric Neurology, Tor Vergata University,
Rome, Italy
Ritscher-Schinzel syndrome or 3C (cranio-cerebellocardiac)
syndrome, characterized by congenital heart
malformation, cerebellar anomalies and cranial defects, is
a rare genetic syndrome only recently delineated. Until now
28 cases have been reported. Craniofacial findings include
cleft palate, ocular coloboma, prominent occiput and forehead,
hypertelorism, down-slanting palpebral fissures,
depressed nasal bridge and micrognathia. Cerebellar
anomalies including Dandy-Walker malformations
(DWM) or its variant are responsible for gross motor
delay associated with secondary hypotonia. Speech delays
are reported in surviving patients. We describe a baby born
at 37 weeks by Caesarean section, from healthy and nonconsanguineous
parents. At 24th week of pregnancy a
routine ultrasound scan detected DWM and cardiac defect.
Birth weight was 2980 g, length 48 cm, and head circumference
35 cm. The infant had no asphyxia at birth; Apgar
scores were 8 and 10 at 1 and 5 min, respectively. At birth
the baby was noted to have typical dysmorphic findings of
3C syndrome. Hepatosplenomegaly and bilateral cryptorchidism
were found. Neurological examination revealed
severe generalized hypotonia with poor spontaneous movements
and poor sucking reflex. Grasp reflexes, Moro and
other primitive reflexes were elicitable but weak. Karyotype
was normal (46,XY) and the 22q11.2 microdeletions (FISH)
were excluded. Cerebral MRI showed: a posterior fossa
cystic malformation with vermis hypoplasia and upward
displacement of the tentorium without evidence of hydrocephalus.
Echocardiogram and cardiac MRI revealed a
hypertrophic cardiomyopathy with secondary valvular
defects. This syndrome should be considered in the differential
diagnosis whenever a newborn is found to have the
DWM, or its variant phenotype, and craniofacial
dysmorphic findings.
FP-F-017
Visual and quantitative analysis of EEG in children with
a cavum of the septum pellucidum
M. Kacinski, A. Kubik, M. Dolik-Michno, G. Augustyn, L.
Bednarski
Department of Pediatric Neurology, Collegium Medicum,
Jagiellonian University, Krakow, Poland
Purpose: Persistent cavum of the septum pellucidum
(CSP) is an anomaly of the brain midline and is considered
a marker of brain dysgenesis. The aim of the project was to
determine whether the symmetry and synchrony of the
bilateral bioelectric activity of the brain is different in children
with and without CSP. Material: A group of 17 children
with a CSP and a control group matched for age and
sex were examined. The analysis included 136 pairs of
EEG curves from independent leads from above both the
hemispheres. Methods: A PL-EEG-24 Classic Campaign
Video option apparatus was used for recording the data.
Apart from visual analysis performed by a doctor, mathematical
analysis was used to estimate 7-s sections of the
EEG recording, Fourier transformation included. Results:
The results were shown in the form of topographic description,
symmetry, synchrony, character, frequency, amplitude,
shape of the pattern, physiological factors and

Abstracts 465
faulty elements. The results of the quantity estimation
included the correlation analysis of the signal, as well as
the analysis of the statistic factors and a description by
means of time-frequency analysis. The strength of the
signal was proved to be several times higher and the statistic
factors were twice higher in CSP children in comparison
to the controls; in addition the former displayed
bilateral asymmetry of the activity in terms of frequency
and amplitude. The differences frequently escaped the
visual analysis. Conclusions: It is necessary to use the
mathematical analysis of EEG in children with a cavum
of the septum pellucidum. This paper was supported by a
grant No 4 PO5E 124 18 of the State Committee of the
Scientific Research.
FP-F-018
Long-term efficacy of active congenital hydrocephalus
treatment in pediatric patients: results of 10-year followup
L.M. Kuzenlova, O.I. Maslova, V.M. Studenikin, E.I.
Stepakina
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific Center of Child Health (Russian Academy of
Medical Sciences), Moscow, Russia
Background: Therapy for children with active congenital
hydrocephalus remains a challenge both for pediatric
neurologists and neurosurgeons. Benefits of conservative
versus surgical treatment are still widely disputed. Goal:
Our intention was to compare the long-term efficacy of
various treatment strategies for patients with different
disease types. Methods: Forty pediatric patients with various
hydrocephalus types (originally aged 3 months–2 years)
were followed-up for a 10-year period. Every available
diagnostic procedure was incorporated. Conservative treatment
was used in 20% of cases, and neurosurgery was
performed in 80% of children (including ventriculo-atrial,
ventriculo-peritoneal, and lumbo-peritoneal shunting).
Results: Decrease of linear and planometric indices was
registered in all patients (as judged from CT and MRT
results). Symptomatic epilepsy (with focal sites localized
by shunting regions) was evident in 85% of surgical cases,
and decreased brain tissue perfusion present in the areas of
surgical shunting (100%). As for biochemical hydrocephalus
markers (levels of xanthine and hypoxanthine), these
two indices were significantly increased in lumbar and
ventricular liquor of patients with severe forms of hydrocephalus
(after conservative/surgical therapy, concentrations
of these two metabolites tended to decrease, but still
remained elevated, suggesting that metabolic and energetic
processes in brain tissue were only partially restored).
Conclusion: Efficacy of surgical and non-surgical treatment
protocols for hydrocephalus is still debatable and remains to
be established.
FP-F-019
Place of hydrocephalus in cerebral dysgenesis in infants
O.I. Maslova, V.M. Studenikin, S.Y. Ishkhanova, L.M.
Kuzenkova, E.I. Stepakina
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific Center of Child Health (Russian Academy of
Medical Sciences), Moscow, Russia
Background: Most congenital anomalies and malformations
of the central nervous system are known either to be
combined with or to induce formation of hydrocephalus,
which fact supplements heavily to clinical course and prognosis
of the disease. Goal: Aim of our study was to reveal
the incidence of hydrocephalus in cerebral dysgenesis
syndromes among a group of 1st year patients. Method:
Case histories of 112 pediatric patients with verified cerebral
dysgenesis (infants aged from 1 to 12 months, among
them 78 males and 34 females), hospitalized in our clinic in
1999–2001, were analyzed. Additional diagnostic methods
of neurovisualization (CT, MRI, and neurosonography,
SPECT) were utilized. Results: Excessive head circumference
(pathological) accretion was registered in 26.8% of
cases during the first 6 months of life (30 patients), considered
as an active hydrocephalus form in seven cases, and as
residual or passive hydrocephalus in the rest of patients (23
cases). Etiology of verified hydrocephalus cases varied from
various pathological conditions, such as congenital malformations
and intrauterine infections, to intraventricular
hemorrhages, etc. Conclusion: Our data suggest that hydrocephalic
cases should be identified during infancy. Active
forms of the disease require administration of acetazolamide,
while drugs of such kind are not routinely indicated
for patients with static hydrocephalus. No explanation can
be currently offered concerning the predominance of male
hydrocephalic cases over female ones, other than for genetic
mechanisms involved.
FP-F-020
Chinese fragile X syndrome
Y.-Z. Guo, J.-H. Chia, S.-P. Zhang, Y. Dan, S.-M. Zhao, S.-
Z. Wang
Department of Pediatrics PUMC Hospital, CAMS and
PUMC, Beijing, China
Objective: Fragile X syndrome is the most common
inherited mental retardation (MR) with an IQ level less
than 60. Studies have been done how to perform screening
and diagnosis of fragile X syndrome. Study design: Nine
clinical characteristics from special patient records of 190
boys and 18 female pediatrics for fragile X screening testing
were analyzed. The characteristics included mental
retardation, family history of mental retardation, elongated
face, large or prominent ears, attention deficit hyperactivity
disorder, autistic-like behavior, simain crease, macroorch-

466
Abstracts
idism and hyperextensible joints. The disease was screened
with PCR amplified to detect (CGG) n repeat sequence, and
diagnosed with PCR, Southern hybridization. Results:
Seven cases were diagnosed with fragile X syndrome by
Southern analysis on PCR product. Six boys have been
identified to carry fragile X full mutation and one mosaicism
that consists of a premutation (CGG repeat
size ¼ ,100) allele. Among the nine characteristics,
simian crease, macroorchidism, and hyperextensible joints
were eliminated, because of low frequency and statistical
insignificance. Using the remaining six-item criteria for
requesting fragile X testing, about more than 60% of this
test in our cases could have been eliminated clinically
without missing any positive cases. Thereby the proportion
of case with positive result improved from 3.4 to 8.8%.
Conclusion: Our simplified six-item clinical checklist
obviously increased the proportion of cases with positive
FRAXA. The PCR method is fast, simple and useful in
screening FRAXA.
FP-F-021
Place of hydrocephalus in the structure of cerebral
dysgenesis in infants
O.I. Maslova, V.M. Studenikin, S.Yu. Ishkhanova, L.M.
Kuzenkova, E.I. Stepakina
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific Center of Child Health (Russian Academy of
Medical Sciences), Moscow, Russia
Background: Most congenital anomalies and malformations
of the central nervous system are presently known
either to be combined with or to induce formation of
hydrocephalus. This fact supplements heavily clinical
course and prognosis of the disease. Goal: Aim of our
study was to reveal the incidence of hydrocephalus in the
structure of cerebral dysgenesis syndromes among the
group of 1st year patients. Methods: Case histories of
112 pediatric patients with verified cerebral dysgnenesis
(infants aged from 1 to 12 months; 78 males and 34
females), hospitalized in our clinic in 1999–2001, were
analyzed. Additional diagnostic methods of neurovisualization
(CT, MRT and neurosonography, SPET, etc.) were
utilized. Results: Excessive head circumference (pathological)
accretion was registered in 26.8% of cases during the
first 6 months of life (30 patients); active hydrocephalus in
seven cases and residual or passive hydrocephalus in 23
cases. The origin of hydrocephalus varied from various
pathological conditions, such as congenital malformations
and intrauterine infections, to intraventricular hemorrhages,
etc. Conclusion: Our data suggest that hydrocephalic cases
should be properly examined, as infants with active forms
of the disease require administration of acetazolamide,
while drugs of such kind are not routinely indicated for
patients with residual hydrocephalus. No explanation can
be currently offered concerning the predominance of male
hydrocephalic cases over female ones, except for genetic
mechanisms involved.
FP-G
Infection/Immunology
FP-G-001
Elevated cerebrospinal fluid levels of anti-CD 9
antibodies in patients with subacute sclerosing
panencephalitis
T. Shimizu a,b,f , T. Matsuishi b , R. Iwamoto e , K. Nihei h ,H.
Yoshioka c , H. Kato b , S. Ueda d , H. Hara f,g , T. Tabira f,g ,E.
Mekada a,e
a Institute of Life Science, b Department of Pediatrics, School
of Medicine, Kurume University, Kurume; c Department of
Pediatrics, Kyoto Prefectural University of Medicine,
Kyoto; Departments of d Neurovirology and e Cell Biology,
Research Institute for Microbial Diseases, Osaka University,
Osaka; f Division of Demyelinating Disease and Aging,
National Institute of Neuroscience, Kodaira;
g National
Institute of Longevity Sciences, Aichi, Japan; h National
Children’s Hospital
SSPE is a slowly progressive and highly lethal disease of
the central nervous system. Although the primary cause of
SSPE is persistent infection of neuronal and glial cells by
measles virus, the precise mechanism of the progression of
this disease has not yet been elucidated. CD9, a member of
the tetraspanin family, is expressed in myelin and other
nervous tissues, and this study found that significant
amounts of anti-CD9 antibodies were detected in the
CSF of all SSPE patients. Anti-CD9 antibodies were also
detected in the CSF of some cases of other neurological
disorders, but in these patients’ levels were lower than in
patients with SSPE. The level of anti-CD9 antibodies was
elevated and reached a peak that coincided with the
appearance of brain atrophy. Our findings appear to indicate
a new aspect of the etiology and progression of this
disease.
FP-G-002
Long-term follow up of the intelligence development of
Japanese encephalitis (JE) patients
B. Wang, D. Ding, Z. Hong
Institute of Neurology, Fu Dan University, Hua Shan Hospital,
Shanghai, China
Objective: To observe the impact of the JEV infection on
the intelligence development of JE patients. Methods:
Forty six Japanese encephalitis virus (JEV) serological
test positive encephalitis patients and 33 JEV serological
test negative encephalitis patients were followed up for 8–
26 years. Mini-mental state examination (MMSE) and
Wechsler’s Intelligence test were used for examination

Abstracts 467
for them as well as 46 healthy people who were 1:1
matched by 46 JE patients. Results: Seven (15.2%) JE
patients were diagnosed as mental retardation (FIQ , 70).
Among them, six patients’ MMSE scores were under 21.
Comparing the FIQ, VIQ, PIQ, and IQ sub-tests within
these three groups, all the scores of JE patients are lower
than those of other two control groups. Conclusion: JEV
infection during the childhood had severe impact to the
intelligence development. The intelligence development
of JE patients was worse than those of patients suffered
other encephalitis and normal controls.
FP-G-003
Long-term sequelae of Japanese encephalitis
D. Ding, Z. Hong, B. Zhou
Institute of Neurology, Fu Dan University, Hua Shan Hospital,
Shanghai, China
Objective: To measure the risk of long-term post-JE
illness-associated disability, and give the basis of estimating
the disease burden of JE. Methods: We carried out a
retrospective, controlled cohort study with participation of
neurologists-experts, to follow up patients suffering from
JE from 1973 to 1994. Two control groups, no-JE (JE
serological test negative) patients and healthy people,
were followed up as control groups. Physical and neurologic
examinations, IQ measurement and MMSE were used
for examination. Results: We found 29 [34%, (95% CI: 24–
44%)] JE patients (including the dead) with long-term
sequelae associated with JE among the 85 patients
followed up, six [8%, (95% CI: 2–14%)] no-JE patients
(including the dead) with long-term sequelae associated
with no-JE among the 73 patients followed up, and only
one [1%, (95% CI: 21–3%)] subject with IQ ¼ 82 in
normal control group. The proportion of long-term sequelae
of JE patients is larger than that of no-JE and normal
control group with the relative risk of 4.15 (95% CI: 1.83–
9.44) and 26.61 (95% CI: 3.71–190.76), respectively
(P , 0:00). Conclusion: The long-term post-JE illnessassociated
disability is very severe, and will give heavy
burden to the society and families.
FP-G-004
CSF b 2 -MG value for the diagnosis of convulsive
diseases
J.-K. Wei, H.-Z. Liu, Y. Tian, X.-Y. Li, Y.-C. Wang, X.-M.
Ma, P.-F. Wei
The Affiliated Hospital of Hebei Medical College for Continuing
Education, Baoding, China
Objective: Mild viral meningitis with convulsions is
similar to infectious toxic encephalopathy in general clinical
manifestations. It is difficult to make therapy scheme
and to predict prognosis. The objective of this paper is to
investigate the search differential diagnosis value of CSF
b 2 -microglobulin (b 2 -MG, X ^ S ng/ml) measuring. Methods:
CSF b 2 -MG was detected in cases with mild viral
meningitis with convulsions and in those with infectious
toxic encephalopathy with CSF b 2 -MG of non-infectious
non-hemorrhagic convulsions by radio-immunization using
the wide-range testing box provided by China Atomic
Scientific Research Institute. Each group included 38
cases. Result: CSF b 2 -MG in mild viral meningitis
(2484.01 ^ 357.57) was obviously higher than that in
infectious toxic encephalopathy (1043.78 ^ 140.73)
(P , 0:01) and the non-infectious non-hemorrhagic
convulsion group (972.12 ^ 102.03), but CSF b 2 -MG in
infectious toxic encephalopathy was not significantly
different from that in non-infectious non-hemorrhagic
convulsion group. Conclusions: CSF b 2 -MG testing is of
help to differentiate mild viral meningitis from infectious
toxic encephalopathy when their clinical symptoms are
similar.
FP-G-005
Effects of early compositive treatment with
immunoglobulin (IG) on serious CNS viral infection
J.-K. Wei, H.-Z. Liu, X.-F. Li, Y.-H. Xu, X. Shi, X.-R. Cao
The Affiliated Hospital of Hebei Medical College for Continuing
Education, Baoding, China
To explore the effects of compositive treatment with IG
in different disease stages on serious CNS viral Infection,
retrospective analysis was taken on 84 cases (1–13 years
old) of serious CNS viral infection. Among them seven
cases had herpes simplex, three cases EB, one case pox
viral infection. The treatment included IG (1.5–2 g/kg IV
divided into 3–5 days), combined with Ribavirin (R, 15
mg/kg daily, 10–14 days) and/or Acyclovir (A, 10–15 mg
daily, 10–14 days), Dexamethasone (0.5–1 mg/kg daily, 5–
7 days), Cerebrolysin (1 ml/kg daily, 10–14 days) and
other supportive therapies. Some cases were subjected to
EEG and CT scan tests. To compare the effect on two
groups, 50 cases of early compositive treatment group
(within 3 days of disease stage) and 34 cases of late
(Middle/Late, M/L) treatment group (after 3 days of
disease stage), we found early treatment is more effective
than later treatment, especially the group with compositive
treatment with IG and R 1 A showed the best result (Table
3).
Table 3
The effect on compositive treatment on meningitis with IG in two stages
Dead Low intelligence Epilepsy Cerebral palsy Recovery
Early 1 3 4 0 42
M/L 5 7 7 3 12

468
Abstracts
FP-G-006
Angiostrongyliasis cantonensis in children: 3 years
follow-up of two cases
X.-Y. Ye, H.-W. Hu, Z.-D. Lin, G.-Q. Li, Y.-L. Zhang
Neurology department, the Yu-Ying Children Hospital
affiliated to Wenzhou Medical College, Wenzhou, Zhejiang,
China
Objective: To confirm the prognosis and manifestations,
and the differences from adults, of Angiostrongyliasis
cantonensis in children. Methods: We analyzed the manifestations,
diagnosis and treatment in two children (a 4-
year-old boy and a 2-year-old girl) with Angiostrongyliasis
cantonensis infection. The two cases had been followed up
for 3 years. Results: The disease was characteristically
demonstrated with eosinophilic meningitis (boy) or eosinophilic
meningoradiculitis (girl). Symptoms of elevated
intracranal pressure and abnormal sensation was observed
in the 2nd week after the onset of the disease. Eosinophils of
peripheral blood and cerebrospinal fluid had been increased
for one month, 43 worms (42 larva) were collected from
CSF during the 1st week of hospitalization in the girl. It
was shown that the conduction was delayed in both patients
at upper brain stem on BAEP, meanwhile intracranial/spinal
CT scan or MRI was negative. After two courses (1 week
per month as a procedure) of the treatment with mannitol,
dexamethasone and Zental, the boy recovered, but the girl’s
CFS returned normal 4 months later after the treatment. The
boy was found without any abnormality associated with the
episode. The girl showed neurological bladder, although it
was gradually improved. Conclusion: In the patient with
Angiostrongyliasis cantonensis, eosinophilic meningitis
would result in complete recovery, but eosinophilic meningoradiculitis
may be followed by some sequelae. The
outcome may be associated with the loci of the disease,
but not with the age of patient.
FP-G-007
The influence of dexamethasone on TNF-a and IL-8
contents in brain tissue during edema induced by
lipopolysaccharide in rats
H.-L. Wang, J.-P. Lu
Department of Pediatrics, The First Affiliated Hospital,
Zhengzhou University, Zhengzhou, China
Objective: To study the influence of dexamethasone
(DXM) on TNF-a and IL-8 expression and the relation
between them in brain edema induced by lipopolysaccharide
(LPS) in rats. Methods: TIA and ELISA methods were
used to measure the contents of TNF-a and IL-8 in brain
tissue after brain edema induced by LPS in rats. Results:
The water content and Evans blue (EB) were increased
significantly in the brain tissue of rats in LPS group and
DXM group compared with those of the rats in NS group
(P , 0:05). The levels of TNF-a and IL-8 in brain tissue of
the rats in LPS group were higher than those in NS group
(P , 0:01). The levels of TNF-a and IL-8 in brain tissue in
DXM group were higher than those in NS group in earlier
time (P , 0:05). After 24 h, there was no significant difference
(P . 0:05) between them. In the LPS group, the water
content in brain tissue and EB content, TNF-a and water
content in brain tissue, IL-8 and water content in rain
tissue, IL-8 and EB content were all have positive correlation
(r were 0.537, 0.423, 0.437, 0.831, respectively,
P , 0:05). Conclusion: TNF-a and IL-8 could participate
in the onset and development of brain edema induced by
LPS. Glucocorticoid could inhibit the production of inflammatory
cytokines and attenuate inflammatory response and
tissue injury.
FP-G-008
The role of tumor necrosis factor-a and interlerlcukin-8
in the pathogenesis of brain edema induced by LPS in rat
J.-P. Lu, H.-L. Wang
Department of Pediatrics, The First Affiliated Hospital,
Zhengzhou University, Zhengzhou, China
Objective: To study the role of TNF-a and IL-8 in the
pathogenesis of brain edema induced by LPS in rats. Methods:
RIA and ELISA methods were used to measure the
contents of TNF-a and IL-8 in brain tissue after infectious
brain edema was induced by LPS in rats. Results: The
levels of TNF-a and IL-8 in brain tissue in the LPS
group were successively increased as compared to the
blank control group and NS group (P , 0:01) They continued
to be high until 48 h. In the LPS group, water content
in brain tissue and EB content, TNF-a and water content in
brain tissue, IL-8 and water content in brain tissue, IL-8
and EB content were all have positive correlation. (r was
0.537, 0.423, 0.437, 0.831, respectively, P , 0:05).
Conclusion: TNF-a and IL-8 could contribute to the
onset and development of inflammatory brain edema
induced by LPS and have closely relationship with brain
injury.
FP-G-009
Levels of insulin-like growth factors in cerebrospinal
fluid of children with meningitis
X.-P. Guo, G.-C. Xue, T.-X. Shi, W.-M. Wang
Department of Pediatrics, the First Affiliated Hospital of
Xinxiang Medical College, Weihui, Henan, China
The insulin-like growth factors, IGF-I and IGF-II, are
potent mitogenic and anabolic peptides. They play prominent
roles in the regulation of immunity and inflammation.
We used an immunoradiometric assay kit for measuring
CSF IGF-I and IGF-II in four groups of patients: bacterial
meningitis (7), tuberculous meningitis (5), viral meningitis
(21), and normal controls (15). Levels of CSF IGF-I in

Abstracts 469
patients with bacterial meningitis and tuberculous meningitis
were elevated in comparison with that in the control
subjects (P , 0:001 and P , 0:001, respectively), but the
levels of IGF-I in CSF of patients with viral meningitis
were not elevated (P ¼ 0:199). The patients with bacterial
meningitis or tuberculous meningitis had significantly
higher levels of CSF IGF-I than those with the viral meningitis
(P , 0:001 and P ¼ 0:001, respectively). CSF levels
of IGF-II in patients with bacterial meningitis were
elevated in comparison with levels in the control subjects
(P , 0:001) but the levels of IGF-II in CSF of patients
with tuberculous meningitis and viral meningitis were not
elevated (P ¼ 0:221 and P ¼ 0:512, respectively). The
patients with bacterial meningitis had significantly higher
levels of CSF IGF-II than those with tuberculous meningitis
and viral meningitis (P ¼ 0:050 and P ¼ 0:001, respectively).
These data suggested that IGF-I and IGF-II might
play an important roles in the pathophysiological process
of meningitis, especially bacterial meningitis.
FP-G-010
Levels of insulin-like growth factor binding protein-3 in
cerebrospinal fluid of children with meningitis of
different etiologies
G.-C. Xue, X.-P. Guo, T.-X. Shi, F.-L. Zhu, F.-Y. Wang, H.-
Y. Qian
Department of Pediatrics, the First Affiliated Hospital of
Xinxiang Medical College, Weihui, Henan, China
Objective: To observe changes of insulin-like growth
factor binding protein-3 (IGFBP-3) in the CSF of children
with viral meningitis, tuberculous meningitis, and partially
treated bacterial meningitis, and the relations between
IGFBP-3 and CSF inflammatory parameters. Methods:
CSF IGFBP-3 levels of these patients with meningitis and
of normal controls were tested by RIA. Results: In comparison
with normal controls, IGFBP-3 levels in CSF of children
with tuberculous meningitis and partially treated
bacterial meningitis were elevated significantly
(P , 0:001 and P , 0:001, respectively), but the levels of
IGFBP-3 in CSF of children with viral meningitis were not
elevated significantly (P ¼ 0:090). The levels of CSF
IGFBP-3 of patients with tuberculous meningitis were
higher than those of patients with partially treated bacterial
meningitis (P , 0:001), and the latter were higher than
those of patients with viral meningitis (P , 0:001). The
CSF IGFBP-3 levels were not significantly correlated with
the CSF leukocyte counts (P ¼ 0:241), CSF protein concentrations
(P ¼ 0:113), and CSF glucose concentrations
(P ¼ 0:320). Conclusion: These data suggested that
IGFBP-3 involved in pathophysiological process of meningitis
and it might be a useful index for differential diagnosis
of viral meningitis, tuberculous meningitis, and partially
treated bacterial meningitis.
FP-G-011
The clinical features and prognostic analysis of acute
myelitis
S.-J. Yu, W. Wang, X. Wang, X. Wu
Department of neurology, Harbin Children’s Hospital,
Harbin, China
We have done an overall analysis of 30 children with
acute myelitis and follow-up of ten cases. The results
showed that clinical features are diverse, and it is easily
misdiagnosed. Children under the age of 6 years were
more likely featured as the transverse lesion than adults,
and may get a relatively long spinal cord shock period and
serious prognosis. The earlier the application of gammaglobulin
and glucocorticoid hormone, the better the prognosis
was observed.
FP-G-012
Diagnosis and treatment of 86 cases with cerebral
malaria
M.-L. Bao, C. Ma, Y. Chen
Inner Mongolia Medical University, Hohhot, China
Objective: To investigate the diagnosis and treatment of
cerebral malaria (CM). Materials and methods: Eight six
cerebral malaria children, male 54, female 32, aged 2
months–14 years. Malaria parasites were examined in
blood experimentally. Quinine and other medicine were
given. Results: The rate of malaria parasites was 100%.
After treatment 53 cases were obviously improved on the
1st day and 25 cases on the 2nd day. eight children died.
Conclusion: The early diagnosis of cerebral malaria was
very important. Mortality may be reduced if the medicine
was given timely. The key of the treatment was eliminating
malaria parasites, lowering down intracranial pressure,
controlling high fever, convulsion, and protecting comatose
patients.
FP-G-013
The relationship of neuroimages and clinical status at
different stages of tuberculous meningitis in children
G.-Q. Li, H.-W. Hu, X.-Y. Ye, Z.-D. Lin, J.-W. Zhao, D.-K.
He
Yuying Children Hospital, Wenzhou Medical College,
Wenzhou, Zhejiang, China
Objective: To investigate the relationship of CT and MRI
at different stages of tuberculous meningitis in children and
the clinical status. Methods: Ninety one cases of tuberculous
meningitis at different stages were studied by CT, MRI and
the clinical status. Results: Sixty seven cases were examined
by CT, and 58 cases were abnormal; the abnormality rate
was 86.6%. Thirty eight cases were examined by MRI, and

470
Abstracts
35 cases were abnormal; the abnormality rate was 92.1%.
Changes of CT were similar to those of MRI: changes of
cisterns and sulci or slightly low density of parenchyma in
the early stage; encephaledema and hydrocephalus in the
middle stage; and low, mixed, or high density in parenchyma
and oppressed, occluded, or adhered sulci in the late
stage. Almost recovered patients had slight changes in CT or
MRI. The patients with poor prognosis had obvious changes
in CT or MRI. Conclusion: We thought that CT and MRI
were useful for clinical evaluation of the degree and prognosis
of TM.
FP-G-014
The change of immune function and serial observation
on IL-2R expression of PHA-activated PBL in 20
patients with viral encephalitis
Y.-Y. Liang a , S.-G. Li b , Q.-H. Kong c , X.-J. Yan c
a Children’s Hospital of Shanxi Province, b Department of
Paediatrics, Shanxi Medical University, Taiyuan, China
Objective: The aim of study is to evaluate the significance
of T-lymphocyte subsets, IL-2R, NK cells in peripheral
blood, serial observation on IL-2R expression of PHA-activated
(0, 24, 48 and 72 h) PBL in patients with viral encephalitis
in acute and convalescence stages, with a group of
normal children as controls. Methods: The 20 patients with
viral encephalitis and normal controls were recruited into
the study using the immunofluoresent antibody test (IFAT)
method. The results revealed that the percentages of the
CD8, NK, IL-2R, and B cells in patients with viral encephalitis
are obviously increased compared with those of the
control group, but the expression of IL-2R obviously
decreased in patients with viral encephalitis at different
times (0, 24, 48 and 72 h). The percentages of the NK, Il-
2R, CD8, B cells and CD4/CD8 in patients with viral encephalitis
of the convalescence stage were clearly higher than
those in the acute stage. Conclusion: The result suggested
that the measurement of immune function and observation
of IL-2R expression may be regarded as a valuable judge
index in the evaluation of therapy and the prognosis in the
patients with viral encephalitis.
FP-G-015
A study of nitric oxide in serum and cerebrospinal fluid
in children with viral encephalitis
S.-G. Li a , Y.-Y. Liang b , Q.-H. Kong c , X.-J. Yan c
a Department of Paediatrics,
b Shanxi Medical University,
and c Children’s Hospital of Shanxi Province, Taiyuan,
China
Objective: The aim of study is to evaluate the significance
of NO in serum and cerebrospinal fluid in children with viral
encephalitis. Methods: Twenty patients with viral encephalitis
and 20 normal controls were recruited into the study.
The levels of NO in serum and cerebrospinal fluid were
determined by spectrophotometry. Result: The result
revealed the level of serum NO in patients with viral encephalitis
was higher than that the normal control (P , 0:01).
The NO level of the serum in patients of acute stage was
clearly higher than that of convalescence stage. The level of
serum NO showed positive relation with the level of cerebrospinal
fluid (r ¼ 0:489, P , 0:01). Conclusion: The
result suggests that the NO level is associated with the
onset, progress and severity in patients with viral encephalitis.
FP-G-016
The change of C-type natriuretic peptide and
neurotensin in rabbit brain injury induced by endotoxin
Y.-C. Zhang, D.-H. Tang, Y.-M. Zhang, L. Xu, L.-Q. Chen
Shanghai Children’s Hospital, Shanghai, China
Objective: To study the change and significance of C-type
natriuretic peptide (CNP) and neurotension (NTin brain
injury induced by endotoxin in rabbits. Methods: One
hundred mg/kg endotoxin was intraventricully injected in
30 New Zealand white rabbits and the same volume of
normal saline was injected in five rabbits as controls. CNP
and NT concentrations were detected by radioimmunoassay
in variable periods in the plasma, CSF and brain tissue
(hippocampus area), and brain water content were measured
by the dry method. Results: The plasma, CSF and brain
tissue CNP concentrations after 3–6 h endotoxin injection
were significantly higher than those of the control group or
before injection (P , 0:05 or P , 0:01). Then, the CNP
altered to decrease in the following periods and the lower
levels emerged at 12–24 h after endotoxin injection. The NT
content were decreased and the lower levels also emerged at
12–24 h after endotoxin injection (P , 0:05 or P , 0:01).
Brain water content was significantly higher (P , 0:05 or
P , 0:01) and reached the peak level at 24 h after injection.
Conclusions: The changes of CNP and NT were related to
brain injury and brain edema induced by endotoxin. The
early high CNP expression may be one of the protective
mechanism.
FP-G-017
The clinical analysis of eight cases in children colti virus
encephalitis
H.-H. Zhu, C.-F. Yan
Guangzhou Children’s Hospital, Guangzhou, China
The study was designed to examine all double serums of 54
children patients, suffering from clinically diagnosed viral
encephalitis from 1999 till 2001. Their serums were checked
to detect pathogens with ELISA provided by Institute of
Virology of Chinese Preventing Medical Academy of
Sciences. Among them, in eight cases IgM anti-colti virus

Abstracts 471
antibodies were positive; males six, females two, the youngest
child 7 months old, and the oldest 10 years old. The
months they got the disease: one in May, one in September,
and in June, July, August, each of them has two cases. All of
them suffered sickness with acuteonset, and all had symptoms
of upper respiratory infection for 1–7 days before they
showed neurological symptoms. All eight cases had fever at
38–39.58C, five of them had consciousness disturbance of
various degrees. Brain CT: showed disseminating low
density foci in one case, and brain atrophy in three cases.
No patient died, five cases recovered; one case developed
secondary epilepsy, one case mild left hemiparesis, one
case disturbance of speech and intelligence. Colti virus is a
new category of Reoviridae, which was denominated by
International Virology Classifying Committee in Berlin in
1990. This virus has been isolated in America, Germany,
France, Indonesia, and also in Beijing, Hainan Province,
Yunnan Province and Gansu Province. In the 54 cases for
this study with double serum testing with ELISA, eight cases
were found to be positive for anti-colti virus IgM antibody.
Today, ELISA is the most widely used examination technique,
which can be applied to checking virus antibodies. This
group of eight cases got the diseases during May–September,
most of them during June–August. Three cases developed
sequelae although there was no fatal case. The colti virus is
one of the pathogens of the summer-autumn children’s viral
encephalitis in the Guangzhou area, and it has severe clinical
symptoms and poor prognosis. It is important for the clinical
staff to pay more attention and confirm the diagnosis.
FP-G-018
Observing evoked potentials of virus encephalitis in 16
children
Y.-L. Zhu, J. Yang, H. Cai, W.-M. Yang, G.-Z. Xu
Capital Institute of Pediatrics, Beijing, China
This paper is discussing whether the evoke potentials
could serve as an evaluation marker of virus encephalitis
causing brain damage. By assaying the evoked potentials at
the acute phase, recovery phase and sequela phase of 16
children the final diagnosis of virus encephalitis, we demonstrated
that in clinically severe virus encephalitis abnormal
evoked potentials were elicited at all clinical stages studied.
We therefore concluded that evoked potential is well correlated
with the prognosis of encephalitis resulting in brain
damage.
FP-G-019
Cerebellar hypoplasia with in utero parvovirus infection
D.L. MacGregor, E.L. Ford-Jones, S. MacPhee, E.N. Kelly,
S. Blaser
The Hospital for Sick Children, Toronto, Ont., Canada
Background: In utero parvovirus infection is associated
with feline cerebellar hypoplasia – kittens so affected
demonstrate dysmetric head movements and abnormal
behavior. In mice and rats, parvovirus has been found to
be teratogenic and similarly to cause cerebellar hypoplasia
with ataxia. Human fetal parvovirus (B19) infection causes
non-immune hydrops and fetal anemia. Autopsy studies
previously reported confirmed hydrops but no other malformations.
However, there are case reports of CNS abnormalities
including ventriculomegaly, periventricular
calcifications, arthrogryposis and neurodevelopmental
disorders. Case report: An 8 month-old girl was referred
for assessment of dysconjugate eye movements and developmental
delay. The pregnancy was complicated by gestational
diabetes mellitus and hypertension. At 28 weeks, fetal
hydrops was diagnosed requiring treatment with in utero
transfusions. A positive culture for parvovirus B19 was
obtained. Delivery was at 30 weeks by Cesarean section
for breech presentation. Neurological examination showed
poor visual responses and wandering eye movements. There
was optic nerve atrophy and underdevelopment of the macular
area. Motor examination demonstrated hypotonia – but
no tremors or ataxia. MRI – showed extensive periventricular
calcification and severe volume loss in the posterior
fossa – including the pons, anterior medulla and cerebellar
hemispheres without sparing of the vermis. Discussion: This
case represents the first human report of cerebellar hypoplasia
with parvovirus infection. P antigens (blood group P
system) – the binding sites for parvovirus, are located on
vascular endothelium and erythroid precursors – suggesting
that vasculitic thrombosis may be a mechanism for brain
injury with in utero parvovirus infection.
FP-G-020
Inborn syphilis – its diagnostics and problems of
treatment
A.A. Ovchinnikova, S.E. Goulyaeva, N.V. Michailichenko,
S.A. Goulyaev
Vladivostok State Medical University, Vladivostok, Russia
Peculiarities of the nervous system prenatal pathology of
198 children born from the mothers infected with syphilis
were studied. Ninety seven children took a course of standard
specific therapy and 101 did not take it. The control
group represented children born from 365 mothers infected
through sex relation, of non-syphilitic etiology. It was found
that infant syphilis developed in children born from the
mothers having a second relapse and early latent syphilis.
In the early neonatal period the following triad was
observed: symptoms of generalized infectious intoxication;
intrauterine growth retardation; and signs of central nervous
system irritation. Abnormalities of skin and mucous
membrane were not specific, and they were fragmentary
and transient. Skeletal system abnormalities were revealed
in 15% of the cases. Blood serological tests were not informative.
Among the infants without treatment at full dosage,

472
Abstracts
signs of the central nervous system disturbance were found
in 80% by the end of the 1st month of life. Generalized brain
symptoms were transformed into meningitis and meningoencephalitis.
In cases of treatment according to the traditional
scheme, 30% of the children got delayed meningitis
and meningoencephalitis at 2 or 3 months of life. The diagnosis
was confirmed by serological tests in the cerebrospinal
fluid. The most precise changes were seen by NSG of the
nervous tissue. This study showed that there is no guarantee
for full recovery in children with syphilis by using the traditional
therapy scheme of treatment. Our result points out the
necessity of diagnosing children as ‘inborn syphilis’ at the
very early stages of life in cases with specific clinical
changes (triad) and also the necessity of early treatment in
full dosage, so that the drugs will pass through the blood–
brain barrier in sufficient amount.
FP-G-021
Intercellular adhesion molecule-1 (ICAM-1) mRNA
changes and drug effects in the brain of mice with herpes
simplex encephalitis
L.-Y. Zhu a ,M.Yi b , B. Wei a , B.-M. Wu b
a Pediatric Department, PLA, 202 Hospital, and b Pediatric
Department, The Second Clinical College, China Medical
University, Shenyang, China
Objective: To investigate the ICAM-1 mRNA changes in
the brain of mice with herpes simplex encephalitis (HSE),
and in the brain after the treatment with acyclovir (ACV)
or ACV 1 dexamethasone (DEX). Methods: (1) The HSE
mice models were established by HSV-1 injection into the
cerebral ventricle; (2) ICAM-1 mRNA expression was
detected by the half quantitative RT-PCR method; and
(3) The changes in the brain cells of mice were observed
under transmission electron microscopy. Results: (1) In the
control group, the ICAM-1 mRNA expression was very
low but detectable. In the HSE group, the expression
increased gradually, reached the highest point on the day
4, and then decreased gradually. There was no difference in
ICAM-1 mRNA expression between HSE on day 1 and the
control group. ICAM-1 mRNA expression of HSE 2 day
group was slightly higher than that of the control group
(t ¼ 2:46, P , 0:05). (2) ICAM-1 mRNA expression was
decreased significantly after ACV and ACV 1 DEX treatment.
ICAM-1 mRNA expression of HSE 3 day group was
significantly higher than that of ACV 3 day group
(t ¼ 4:02, P , 0:01). No difference was seen in the
ICAM-1 mRNA expression between ACV 3 day group
and ACV 1 DEX 3 day group. (3) The edema of ACV 1
DEX treated mice brain vessels were attenuated. Conclusions:
ICAM-1 was involved in pathological process of
HSE inflammatory reaction. Treating HSE mice with
ACV or ACV 1 DEX could decrease ICAM-1 gene
expression. ACV 1 DEX might be necessary in treating
HSE in the early phase.
FP-G-022
The effect of dexamethasone therapy on the changes of
nitric oxide in children with bacterial meningitis
F. Gao, J.-F. Lu, Q.-X. Shui, Z.-Z. Xia, B.-L. Zhou
Department of Neurology, Children’s Hospital, Zhejiang
University Medical College, Hangzhou, China
Objective: To investigate the effect of dexamethasone
therapy on the changes of nitric oxide in children with
bacterial meningitis. Methods: The concentrations of the
end product of nitric oxide (nitrite) and its precursor (larginine)
in CSF of 26 cases of bacterial meningitis were
detected with Griess method and an amino acid analyzer,
and 31 children without any neurologic diseases formed the
control group. Results: (1) The CSF nitrite level of bacterial
meningitis before the treatment of antibiotic drug with or
without dexamethasone was significantly higher than that of
the control group (P , 0:01). The concentrations of l-arginine
were lower in patients than in controls (P , 0:05). (2)
Among the 26 patients, 16 cases were treated with antibiotic
drug only, and the other ten cases were treated with pulsed
dexamethasone. Dexamethasone exerted a significant effect
on the end product of nitric oxide (P , 0:05). CSF nitrite in
children with bacterial meningitis declined to the normal
range after the treatment of antibiotic drug with or without
dexamethasone. (3) CSF levels of l-arginine were significantly
lower in patients with antibiotic drug only than those
with pulsed dexamethasone (P , 0:05). Conclusion: Dexamethasone
therapy might decrease the neuropathologic
damage of patients with bacterial meningitis caused by the
overproduction of nitric oxide partly.
FP-G-023
The protective effect and its mechanism of pretreatment
with curcumin on infectious brain edema in rats
F. Luo, R. Huang, Y.-J. Yang
Department of Pediatrics, XiangYa Hospital, Central South
University, Changsha, China
Objectives: To study the protective effect and its mechanism
of pretreatment with curcumin against infectious brain
edema in rats. Methods: SD rats weighing 210 ^ 27 g were
randomly divided into five groups (1) Normal control group
(NS); (2) Infectious brain edema (PB); (3) DMSO control
group; (4) HS pretreatment group; and (5 curcumin pretreatment
group (CUR). The water content (WC), Na 1 and K 1
content in the brain tissue were measured. The content of
MDA and SOD were assessed by chromatometry. The levels
of TNF-a and IL-1b were detected by ELISA. The HSP70
expression was examined by Western blot analysis. Results:
The results were shown: (1) the contents of water and Na 1 ,
MDA, TNF-a and IL-1b were significantly increased in PB
group than in NS group (P , 0:01 or P , 0:05; compared
with PB group, the WC, the content of Na 1 , MDA, TNF-a

Abstracts 473
and IL-1b were significantly decreased in HS and CUR
groups P , 0:01 or P , 0:05; and (2) the content of SOD
was significantly decreased in PB group than in NS group
(P , 0:05). Compared with PB group, the content of SOD
was significantly increased in HS and CUR group
(P , 0:05); (3) Western blot analysis showed that the band
density areas of HS, CUR and PB groups were more
increased than NS and DMSO group, especially in CUR
group. Conclusion: There is a protective effect against infectious
brain edema in rats of pretreatment with curcumin. The
effect might be associated with antioxidant, inhibiting the
activity of cytokines and inducing expression of HSP70.
FP-G-24
Study of enteroviral central nervous system infection by
polymerase chain reaction in China
Z.-B. Chen, Y.-S. Dong, X.-W. Fan
Department of Pediatrics, The Qingdao Haici Hospital of
Qingdao University, Qingdao, China
Objective: The aim of the present study was to evaluate the
diagnostic potential of previously published enterovirus
(EV) PCR assay to detect EV in CSF samples from children
with a diagnosis of aseptic meningitis and to investigate the
patient characteristics and epidemic status in China. Methods:
EV RNA was detected in 187 CSF samples and serum
and/or urine samples of partial patients by PCR and developed
viral culture technique. Results: PCR was positive in all
62 CSF specimens with positive viral culture as well (100%).
In addition 93 (74%) of 125 CSF samples with negative viral
cultures had positive PCR. In 21 cases of these 93 patients,
viral culture from other sites (serum and/or urine) was positive.
The positive rate of CSF PCR based on clinical diagnosis
in patients with meningitis of negative bacterial culture
results was 83%, considerably higher than the sensitivity of
CSF virus culture (33%). This PCR results are available
within 4 h, whereas the viral culture of CSF requires 4.6
days for a cytopathic effect to develop. Echovirus 6 and
echovirus 30 in this study caused twice summer outbreak
in different regions of China. The clinical characteristics of
155 patients with EV meningitis are different in different age
groups. Conclusion: EV infection is the most common cause
of aseptic meningitis in China. The PCR assay was rapid,
sensitive and specific for the diagnosis of EV meningitis
and showed the potential for PCR tests to shorten hospitalization
and reduce the use of antibiotics.
FP-G-025
Clinical characteristics and follow-up studies in 22 cases
of herpes simplex encephalitis in children
F. Fang
Beijing Children’s Hospital, Beijing, China
Objective: HSE was one of the severe forms of acute viral
encephalitis in children, with high mortality and morbidity.
We analyzed the clinical characteristics and the prognosis of
herpes simplex encephalitis in children. Method: Twentytwo
patients with HSE, 14 males and eight females, were
included in our study. The ages were between 3 months
and 13 years. Eighteen patients were treated with acyclovir,
15 mg/kg per day for 14 days. Fifteen of 22 patients were
followed up 6 months–6 years, including intellectual ability
assessment, CT examination, etc. Diagnosis of HSE was
based on positive anti-HSV IgM in CSF, or fourfold increase
in anti-HSV IgG titres in the two different CSF samples, or
ratio of serum to CSF anti-HSV antibody titers ,20 by
ELISA; or using PCR to detect HSV-DNA in CSF. Results:
Fever was found in 22 patients (100%), altered consciousness
in 18 patients (81%, coma in 12 patients. The Glasgow Coma
scale scores were ,4 in three cases, 5–7infive cases, 8–9in
four cases. Seizures were found in 16 (72%), vomiting in 13
(59%), headache in ten (45%), personality change in eight
(36%). Cranial nerve paresis in ten (45%), hemiparesis in
seven (31%). Cranial CT and/or MRI showed abnormalities
in 19 of 22 patients. Temporal and frontal lobes were affected
in 12 patients. Cerebral hemorrhages in seven cases. Five of
15 patients recovered during follow-up, three cases were
mildly disabled, five cases moderately disabled, two cases
severely disabled. Conclusions: HSE was classified as mild
and diffuse encephalitis. The early consciousness and
personality changes are clinical characteristics of HSE. Definite
diagnosis of HSE was based on virological diagnostic
tests in CSF. The duration of illness prior to treatment, the
Glasgow Coma scale score and lesions of the brain are important
to prognosis.
FP-G-026
The clinical characteristics and the treatments of brain
abscess in children
J. Xiao
Beijing Children Hospital, Beijing, China
Objective: To summarize the clinical characteristics and
discuss the treatments of brain abscess in children. Methods:
We summarized the clinical characteristics in 37 patients
with brain abscess diagnosed by CT or MRI. Twentythree
of them were followed up from 3 months to 5 years.
Results: The causes of brain abscess in 78% of patients were
obscure. The seizures as the first symptom were presented in
20 patients (54%). Twenty-four patients (65%) were without
fevers, and 18 patients (49%) with headache. Thirty
patients were treated with antibiotics through intravenous
injections for 3–6 weeks. Eleven patients recovered completely,
12 had improvement and one died, and total efficiency
was 77%; seven patients received surgery, one recovered,
three got better. Conclusions: Clinical symptoms of brain
abscess in most children were not specific; we always
needed to do the diagnostic neuroimaging examinations to
make the diagnosis earlier. The conservation treatments

474
Abstracts
with antibiotics under the surveillance of CT or MRI were
the main therapy.
FP-G-027
Clinical research of treatment with ganciclovir on the
virus encephalitis of children
R. Huang, G.-Y. Zhang, Y.-J. Yang, L. Liang, Y. Yu
Department of Pediatrics, Xiangya Hospital of Center South
University, Changsha, China
Objective: To observe the clinical therapeutic effect of
ganciclovir in the children with virus encephalitis. Method:
Ninety eight patients with virus encephalitis were
randomly divided into the virazole treatment group (52
cases) and the ganciclovir treatment group (46 cases). Clinical
performance and therapeutic effect were compared.
Results: The mean time of headache, vomiting, seizures
and the recovering of consciousness was shorter than the
virazole treatment group. The duration of fever was
shorter, but there was no statistical difference. Conclusion:
Ganciclovir showed an obvious effect for the treatment of
children with virus encephalitis.
FP-G-028
Clinical analysis in 37 cases of brain abscess in children
X.-H. Yu, Y.-J. Yang, L.-Z. Cao
Department of Pediatrics, Xiangya Hospital, Central South
University, Changsha, China
Objective: To clarify the clinical characteristics and
discuss the causes of misdiagnosis and the treatments of
brain abscess in children. Method: The clinical characteristics
were analyzed in 32 patients with brain abscess diagnosed
by CT or MRI. Results: In the 32 patients, the causes
of brain abscess in ten patients (31.2%) were obscure; ten
patients (31.2%) were otogenic; four patients (12.5%) were
traumatic; eight patients (25%) were hematogenous. There
are only ten patients with typical clinical symptoms of
fever, headache and local signs of the nervous system. A
total of 68.8% patients had no significant clinical manifestations,
so that brain abscess was easily misdiagnosed.
Conclusions: The causes of brain abscess were obscure
or otogenic; most clinical symptoms of brain abscess in
children were not specific. The surgical treatment is the
main therapy.
FP-G-029
Twenty one cases of childhood vestibular neuritis
C.-J. Liu, X.-T. Song, G.-F. Zhao, B.-F. Xu
Yishui Central Hospital, Yishui, Shandong, China
We report 21 cases of childhood vestibular neuritis (M/F
13/8, age 9–13 years). Before onset, there were upper
respiratory tract infection (n ¼ 16), nasosinusitis (n ¼ 1),
and cases with unknown cause (n ¼ 4). Major finding:
vertigo of sudden onset and remission after 7 days; five
cases recovered in 10 days. The concomitant symptoms:
nausea, vomiting, and horizontal nystagmus. No headache,
deafness or tinitus. Cold water test: unilaterally positive in
15 cases and bilaterally positive in six cases. Treatment:
dexamethasone was IV dripped first, then prednisolone,
antibiotics and vitamins were used. The water-electrolyte
imbalance was corrected by transfusion. Completely recovery
was achieved in 16 patients, and hearing loss was
recovered in five patients with persistent mild dizziness.
The time of hospitalization was 10–31 days. No recurrence
has been reported after 2 years. This disease is idiopathic,
but the virus infection theory is approved and no specific
treatment is available. The dexamethasone, antibiotics, and
vitamins can be used. It has the tendency of self-remission.
The recovery was correlated to the age. The younger the
child was, the more rapidly recovery was achieved. The
patients in this group were all children who recovered
rapidly with no sequelae.
FP-G-030
Rhythmic fast activity on EEG in a patient with Mollaret
meningitis
J. Arita
Department of Pediatrics, The Jikei University School of
Medicine, Tokyo, Japan
Mollaret meningitis is a very rare disease of unknown
etiology, characterized by repeated aseptic meningitis with
transient neurological manifestations and quick recovery.
The patient, a 16-year-old boy, developed acute encephalitis
with complete recovery from age 13 to 16 every year.
The symptoms at the onset were loss of consciousness,
generalized tonic clonic seizure and pyramidal signs.
First admission to our hospital was at age 15. EEG at the
early stage showed diffuse and low voltage rhythmic fast
activity. He recovered his consciousness after a week. A
recent episode of the attack occurred at age 16. He was
admitted to our hospital last year because of high fever of
398C. Right after admission, he developed generalized
tonic clonic seizure and consciousness disturbance with
the alternating pattern of deep coma and delirium. Cerebrospinal
fluid during the early stage revealed mild leukocytic
pleocytosis, elevation of protein and high level of IL-
6, although brain CT and MRI showed no positive findings.
EEG showed intermittent diffuse middle voltage rhythmic
fast activity. After 5 days of admission, his neurological
symptoms completely disappeared, and the EEG changed
into normal. This is the first report of rhythmic fast activity
on EEG in Mollaret meningitis. These clinical pictures
suggest that this illness could cause a brainstem dysfunction.

Abstracts 475
FP-G-031
Laboratory findings in children with bacterial
meningitis
N.-C. Chiu, H. Chi, C.-S. Ho, E.-Y. Shen
Department of Pediatrics, Mackay Memorial Hospital,
Taipei, Chinese Taipei
Laboratory data from 239 episodes of culture-proved
bacterial meningitis were collected during the period from
1984 to 2000. The most common causative agent was group
B streptococcus (GBS, 19.7%), followed by Streptococcus
pneumoniae (15.9%), Escherichia coli (13.8%), Hemophilus
influenzae (7.5%), and Enterobacter cloacae (5.4%).
The major pathogens in patients less than 1-month-old
were GBS and E. coli, in patients aged between 1 month
and 1 year were GBS, S. pneumoniae and E. coli, and in
patients older than 1 year were S. pneumoniae and H. influenzae.
During the study period, cases of S. pneumoniae
meningitis increased strikingly since 1991. More than
1000 leukocytes/mm 3 in CSF was found in 42.4% patients.
Protein level higher than 200 mg/dl in CSF was found in
53.6%. Normal CSF leukocyte counts, protein levels,
glucose levels or CSF to plasma glucose ratio were found
in 9.6, 5.0, 15.9 and 12.2% in the cases examined. However,
only one patient had all four factors within normal range.
Blood cultures yielded the same bacteria as the CSF cultures
were recorded in 61.2% cases. Hyponatremia (serum
sodium ,130 mEq/l) was found in 19.1% patients. High
mortality rates were found in patients with CSF less than
five leukocytes/mm 3 (40.0%), CSF glucose less than 20 mg/
dl (22.1%), CSF protein more than 250 mg/dl (30.2%), and
serum sodium less than 130 mEq/l (34.8%).
FP-G-032
Long-term follow-up of childhood tuberculous
meningitis
J.F. Schoeman a , J. Wait d , M. Burger b , F. van Zyl c ,G.
Fertig a , A.J. van Rensburg a , P. Springer a , P.R. Donald a
Departments of a Paediatrics and Child Health, b Physiotherapy,
and c Audiology, Tygerberg Children’s Hospital and
Faculty of Health Sciences; and d Department of Psychology,
University of Stellenbosch, Tygerberg, South Africa
Introduction: The morbidity of tuberculous meningitis
(TBM) remains high in spite of modern antituberculosis
treatment. Methods: The long-term outcome of 76 children
with TBM, who received modern antituberculosis drugs and
active normalization of raised intracranial pressure, was
determined. The median age on admission was 29.5 months
and on follow-up 9 years. Intelligence, motor function, vision
and hearing were assessed by a team of health professionals,
while functionality was also assessed by means of a quality of
life questionnaire. Results: Only 20% of children were functionally
completely normal on follow-up. Main areas of functional
deficit were cognitive impairment (80%), poor
scholastic progress (43%) and emotional disturbance
(40%). Twenty-five per cent of children had evidence of
motor impairment, but all could walk and only five children
(6% of total) were unable to run. One child was blind but no
child had sensori-neural deafness. Conclusions: Older longterm
follow-up studies on TBM found that 21% of survivors
had moderate to severe hearing loss and 12% were deaf. The
improved auditory outcome in our study is most readily
explained by the exclusion of streptomycin from the treatment
regimen. The multiple other handicaps documented in
this study, however, have serious implications for social,
academic and career prospects, especially since most children
come from socially deprived backgrounds. A high index
of suspicion of TBM in communities as ours where tuberculosis
is highly prevalent will help to diminish the morbidity
associated with this devastating disease.
FP-G-033
Clinical features of multiple sclerosis (MS) in children
L.-L. Tian
Xuanwu Hospital of the Capital University of the Medical
Sciences, Beijing, China
Objectives: To obtain clinical features for the early diagnosis
of MS among patients with neurological diseases.
Patients and methods: Clinical data of five patients with
MS hospitalized at pediatrics were analyzed and collected
retrospectively. Results: Age at onset of the five patients vary
from 7 to 10 years (mean 9.4 years). Visual symptoms were
reported in the five patients and were the first manifestation in
four of them. Seizure occurred in one patient. A 10-year-old
boy had developed central diabetes insipidus for 3 years
before visual symptom occurred. Some other clinical
features were found in these five patients, such as hypesthesia,
paresthesia, limb weakness and so on. Brain MRI
disclosed subcortical lesions in four of them. Conclusion:
Visual acuity decrease is a clinical characteristic of MS in
children. Some other symptoms and signs may be found
either. They must be emphasized. Brain MRI and visual
evoked potentials (VEPs) should be recorded as soon as
possible in order to obtain more data for early diagnosis.
FP-G-034
Communication problem and neurological aspect of
cytomegalovirus infection in children
Sunartini
Department of Pediatrics, Gadjah Mada University, School
of Medicine, Yogyakarta, Indonesia
Cytomegalovirus (CMV) infections or inclusion diseases
in children, can affect many organs, especially the central
nervous system. Clinical manifestations range from slight to
severe and often fatal. Recently, the incidence of CMV

476
Abstracts
infections in infants and children has been increasing. This
research is a case study, with an analytical descriptive
approach. Subjects and methods: Children with abnormal
psychomotor development, speech or communication disorder
were suspected to have toxoplasmosis, other agents,
rubella, cytomegalovirus, herpes simplex (TORCH) infection,
and admitted to Dr Sardjito Hospital. Routine blood
and serological tests were carried out at least once. USG,
brain CT Scan, EEG and other supporting examinations also
were done based on clinical indicators. Results: The serological
tests of almost 50% of cases were positive for CMV. A
majority was diagnosed as active infections of CMV. Some
cases, particularly with acute infections, and latent infections
suggesting the possibility of reactivation were treated
with ganciclovir. Improvement of psychomotor development,
communication, and clinical course of the disease
varied greatly and continues to be observed, along with
drug side effects. Treatment with ganciclovir remains
controversial. The drug is difficult to obtain and is very
expensive.
FP-G-035
Neurologic and neuropsychologic characteristics of
early disseminated Lyme disease in children
A.L. Belman, M. Milazzo, T. Preston, P.K. Coyle, R.
Grimson, L. Hyman
School of Medicine, State University of New York, Stony
Brook, Stony Brook, NY, USA
To identify neurologic and neuropsycholgic features of
early disseminated Lyme disease (LD) in children, we
enrolled 37 children (22 boys, eight girls, ages 6–17
years) with CDC defined LD in a prospective study.
Controls were 25 healthy children (14 boys, 11 girls, ages
6–17 years) from the same LD endemic geographic area.
None had evidence of LD or Bb exposure. Protocol: structured
neurologic history, examination; laboratory studies;
standardized neuropsychologic and behavioral assessments.
Presenting LD syndromes: facial nerve palsy (FNP) (17),
lymphocytic meningitis (LM) (9), radiculoneuritis (1),
multifocal EM (MEM) (4), and arthritis (3). The most
frequent self-reported symptoms were fatigue/listlessness
(84%), headache (81%), neck pain (72%), mood disturbance
(70%), and sleep problems (62%). Disrupted/lost school
days were reported for 56% of the cases; 85% reported
missed activities/sports. CSF findings: " wbc/mm 3 (76
%), " protein (52%), and intrathecal anti-Bb production
(37%). Of the 24 children with CSF pleocytosis, 13 had
FNP, seven had LM, three had MEM, one had radiculoneuritis.
Of the 17 children with FNP, 75% had " wbc/mm 3 .
Behavioral problems (mood disturbances) were significantly
more frequent in cases than controls. Psychometric
evaluation showed no difference between the two groups.
Conclusions: Headache, fatigue, and sleep problems are
frequent in early disseminated LD; a high rate of morbidity
results in disrupted school days and activities; CSF abnormalities
are common, even in children without frank meningeal
signs; FNP is often associated with an occult
meningitis; disturbance of mood is common; and cognitive
function is not affected. Supported in part by NINDS (NIH)
P01-NS34092; NGCRC: M01-RR10710-02.
FP-G-036
Diagnosis of acute disseminated encephalomyelitis and
multiple sclerosis in childhood and their differential
diagnosis
W.-C. Zhang, H.-S. Wu, T.-C. Liu
Beijing Children’s Hospital, Beijing, China
Objective: Acute disseminated encephalomyelitis
(ADEM) and MS are both common demyelinating diseases
of the CNS. This paper was to study the clinical and lab test
features of ADEM and MS so as to improve clinical and
differential diagnosis. Methods: The clinical features and
experimental results were analyzed in 34 cases with
ADEM and 17 cases with MS compatible with the established
criteria. Results: ADEM cases included 19 boys and
15 girls, and MS cases seven boys and ten girls. Frequency
of certain clinical features (ADEM%; MS%) were helpful to
differentiate between ADEM and MS, such as fever (64.7
and 29.4%), headache (58.8 and 23.5%), consciousness
change (64.7 and 5.9%) including coma (17.6 and 0%);
and seizures (35.3 and 0%). ADEM often produced widespread
CNS disturbance, but MS usually presented monosymptomatic
syndrome, such as six cases of myelopathy,
five hemiplegia, and 33 optic neuritis, etc, It was impossible
to make a certain distinction on CSF immunological examinations.
Cranial MRI revealed thalamic involvement
(ADEM 32.4%; MS 0%); EMG examination showed
peripheral nerve damage in ADEM whereas not any in
MS. Conclusion: It is suggested that ADEM be considered
in the patients presenting previous history of an infection or
vaccination, widespread CNS disturbance and alteration of
consciousness or seizures in particular, and multifocal white
matter lesion on MRI especially with thalamic involvement,
and that MS be considered with onset of monosymptomatic
syndrome, at least two non-associated lesions in the white
matter, and relapses and remissions.
FP-G-037
Long term outcome of streptococcal movement
disorders
K.G. Walker, J. Wilmshurst
Neurology/Cardiology Units, School of Child and Adolescent
Health, University of Cape Town, Red Cross Children’s
Hospital, Cape Town, South Africa
Post streptococcal conditions such as rheumatic carditis,
Sydenhams chorea (SC), PANDAS and other movement

Abstracts 477
disorders remain major public health problems in South
Africa. Whilst the ideal goal is primary prevention, some
measure of secondary prevention has been attained using
prophylactic penicillin but too many children’s lives are
ruined by long term complications and morbidity. A retrospective
study of 40 cases attending the Rheumatic Fever
Clinic and/or neurology clinics over the past 10 years has
been undertaken in order to direct attention to possible
means of preventing complications but also to emphasize
the urgency of addressing primary prevention and penicillin
prophylaxis. We are discharging children to the same
conditions from which they come! Forty patients with
movement disorders and a past infection with b-haemolytic
streptococcus were identified (22 males:18 females). Average
age of presentation was 8 years. Twenty-one children
are severely socio-economically deprived and 19 moderately
deprived. Pharmacological interventions, results and
side effects have been reviewed. The natural history, long
term morbidity and association with penicillin prophylaxis
and compliance have been addressed. Three patients initially
diagnosed with SC have now been diagnosed as
having Tourette’s syndrome. This study emphasized the
high morbidity of neuropsychiatric disorders in post-streptococcal
movement disorders. Possible links between SC,
PANDAS and Tourette’s syndrome will be discussed.
FP-G-038
Misdiagnosis analysis of 38 children with tuberculous
meningitis
Z.-C. Wang, X.-T. Cai
Department of Pediatrics, Longgang Central Hospital,
Shenzhen, China
Object: To analyse the causes of misdiagnosis of tuberculous
meningitis and to explore prevention methods. Methods:
A total of 186 cases of tuberculous meningitis were
treated from January 1, 1996 to December 31, 2000. We
analysed 38 cases which were misdiagnosed as other
diseases initially. Results: The misdiagnosis rate is 20.4%.
Thirty eight cases were misdiagnosed initially as the following
diseases: viral encephalitis (seven cases), purulent
meningitis (six cases), bronchopneumonia (five cases),
epidemic encephalitis B (four cases), acute upper respiratory
infection with febrile convulsion (four cases), acute
toxic encephalopathy (three cases), typhoid (two cases),
septicemia (two cases), and others (five cases). The main
causes of misdiagnosis included atypical early clinical
manifestations, atypical changes in CSF and the appropriate
using drugs, which may disturb the natural course. Conclusion:
Improving the knowledge of tuberculous meningitis,
serially monitoring the radiography of chest and CSF
features of suspected cases and taking a necessary diagnostic
therapy early can effectively decrease the occurrence of
misdiagnosis.
FP-G-039
Recurrent cerebellar ataxia with central nervous system
(CNS) demyelination: a particular multiple sclerosis
(MS) form in children?
C. Triki, F. Kammoun, Z. Mnif, F. Choyakh, I. Feki, M.S.
Kéchaou, C. Mhiri
Department of neurology, CHU H. Bourguiba, Sfax, Tunisia
Demyelinating CNS diseases include MS, optic neuritis,
acute transverse myelitis, acute disseminated encephalomyelitis
and some rare conditions such as Schilder disease.
MS is rare in children under 10 years and uncommon in
adolescents. We report two patients. A 9 years old girl
and a 15 years old boy presented with subacute cerebellar
ataxia following non-specific infections. Clinical examination
showed cerebellar ataxia, vestibular disturbances and a
pyramidal syndrome in the lower limbs. Eye examination
was normal. CSF was normal: pleocytosis was absent and
oligoclonal banding was present only in one case. Lactic
acid was normal. Evoked potential studies showed an
increase in the latency of the P100 potential of the visual
evoked response and increase of latency of somatosensory
and auditory evoked responses in the two patients. Cerebellar
MRI showed several areas of increased signal on T-2
weighted sequences located in the cerebellum, brainstem,
cervical spinal cord and hemispheric white matter. Basal
ganglia were normal. A diagnosis of acute disseminated
encephalomyelitis was made and these patients were treated
by high-dose intravenous corticosteroids followed by oral
therapy. The outcome was good, in fact, the cerebellar
ataxia disappeared. But, when the dose of corticosteroids
was under 20 mg/day, cerebellar ataxia reappeared. The
diagnosis of MS was suspected, but absence of any other
symptoms continues. After 4 years cerebral MRI showed the
same lesions. These cases show the difficulty of MS diagnosis
in children.
FP-G-040
Early cerebral sonographic abnormalities in a case of
vertically transmitted HIV-1
R.O. Jorge
Hospital G Fricke-Facultad de Medicina Universidad de
Valparaíso-Alvarez, Viña del Mar, Chile
Previously Bode and Rudin (1995) reported echogenic
stripes in the basal ganglia identified in a 4 months infant
with human immunodeficiency virus. This was attributed to
a calcifying arteriopathy. These authors postulated that
cerebral sonography performed in early infancy might
allow identification of HIV encephalopathy even before its
clinical manifestation. We had a control patient whose
parents are both affected with AIDS. During a short period
between the second and third month of age, progressive and
severe developmental abnormalities, acquired microce-

478
Abstracts
phaly, hypotonia and finally quadriplegia were observed. In
this period CMV, toxoplasma, varicella zoster, and other
bacterial, fungal and parasitic infections of CNS were
ruled out. A transfontanelle sonographic examination (US)
was performed in the newborn period and described as
normal. At the age of 1 month a second brain US showed
an increase of distance in which the pericallosal branches of
the anterior cerebral artery were easy followed. Its course
was evident in almost all its extension in the parasagittal
views without moving the transducer. This was associated
with an increase of the pulse ratio in the pericallosal and in
the cortical branches of middle cerebral arteries. The hipoechogenicity
surrounding these vessels was increased. A
brain CT at this stage of disease was considered normal.
Progressive symmetric frontal ventriculomegaly was
evidenced by ultrasounds in the period between 2 and 4
months. A second CT at the age of 4 months showed a
severe frontal and cerebellar atrophy, white matter hypodensity
and multiple calcifications. We postulate that the early
vessels sonographic findings express the increase of sulks
space due to initial cortex atrophy. This findings could help
in the early suspicion of HIV encephalopathy, even before
ultrasound calcifications and clinical regression becomes
evident.
FP-G-041
Multiple sclerosis in children
V.N. Yefimenko
Medical university, Donetsk, Ukraine
Twenty five children with the authentic diagnosis of MS
according to C. Poser et al. (1983) are under our observation.
In the study group the number of female persons predominates
(16 girls and nine boys). The age of onset was 4–15
years, the peak age being 12–13 years. The first symptoms
in eight children (32%) appeared prior to the age of 10. The
earliest onset was noted in a 4-year-old girl. A monosymptomatic
onset was present in 16 children (64%), a polysymptomatic
onset in 9 (36%). The initial symptoms were
retrobulbar neuritis of the optic nerve, ataxia, isolated
disturbance of sensation, facial nerve neuropathy, dyskinesia.
A broad picture of MS developed by the age of 2
months–9 years from the onset, 21 within the first 2 years
(84%). At the onset of the disease a remittent course with
frequent exacerbations, twice to four times a year, prevailed.
Follow-up in these children was studied during 2–12 years
(7.1 years the average). A remittent course persisted in 17
children; in seven patients the course had a secondary
progressive character. A primary progressive course was
noted in one case. At the stage of the first MS manifestations,
MRI abnormalities were revealed in 58.3% of cases,
an increase up to 77% was seen at 5 years. Immunologic
studies during exacerbation showed a decrease in the
content of CD3 1 , CD4 1 , CD8 1 – cells of blood and
increase in the level of circulating immune complexes. The
number of CD8 1 – cells increased and the ratio of CD4/
CD8 decreased when reaching the stage of remission.
Immunogenetic studies of class I HLA-antigens demonstrated
an increase in HLA-B12 incidence in patients in
comparison with controls (P , 0:025; RR ¼ 6.29).
FP-G-042
Brainstem encephalitis and acute disseminated
encephalomyelitis following mumps
F.M. Aynaci a , A. Ayvaz a , A. Ahmetoglu b
Karadeniz Technical University, Faculty of Medicine,
a Department of Child Neurology and b Radiology, Trabzon,
Turkey
A previously healthy 4 years 3 month old female developed
brainstem encephalitis with clinical manifestations
including fever, decreased level of consciousness, left facial
and abducens paralysis, and midriasis of the left eye 1 week
after bilateral swelling of the parotis and submandibular
regions. Twenty days after remission of encephalitis,
marked ataxia, dysarthria and fever became apparent. MRI
revealed multiple hyperintense lesions which were
increased in size when compared with MRI at admission.
High dose MPZ therapy was started and followed with
decreased MPZ doses for 2 months. Two weeks after beginning
MPZ, ataxic symptoms resolved. Within 48 days, she
was able to walk with support. Today, now in her 8th month
of illness she has no neurological deficit.
FP-G-043
Reverse Shapiro’s syndrome – an unusual cause of fever
of unknown origin
K.-L. Lin, H.-S. Wang
Division of Pediatric Neurology, Chang-Gung Children’s
Hospital, Taoyuan, Chinese Taipei
Shapiro et al. first described two cases of recurrent spontaneous
hypothermia associated with agenesis of the corpus
callosum (Shapiro’s syndrome). Here we report a girl
presenting with fever of unknown of origin who had
complete agenesis of the corpus callosum. The same condition
had been reported by Hirayama et al. called ‘reverse
Shapiro’s syndrome’. This 9-month-old girl had had the
symptom of periodic hyperthermia since the age of 7
months. On examination, she had mild hypotonia with
delayed developmental milestones. No other neurological
or physical abnormalities were noted besides the callosal
agenesis. Investigations for her prolonged fever including
complete blood cell counts, peripheral blood smear, serum
electrolyte levels, urinalysis, renal function, liver enzyme
levels, thyroid function, immunologic work-up (C-reactive
protein, rheumatoid factor), as well as radiology were
normal. Thus, hidden infections, collagen vascular disease,
hematologic diseases, malignancy, and thyroid dysfunction

Abstracts 479
were carefully excluded as the cause of her periodic
hyperthermia. Whole body Gallium-67 scan revealed no
active inflammatory focus. Hormonal studies were normal.
Her electroencephalogram was normal. According to the
observation of previous literature, we suggest that periodic
hyperthermia in this girl is caused by the dopaminergic
denervation of the hypothalamic thermoregulatory center.
We tried a dopamine agonist (levodopa with carbidopa) in
this girl but failed to control the hyperthermia.
FP-G-044
Neonatal bacterial meningitis in southern Taiwan:
epidemiologic trends and prognostic factors
C.-J. Chang
Department of Pediatric Neurology, Chang Gung Memorial
Hospital (Kaohsiung), Kaohsiung Hsien, Chinese Taipei
In two investigative phases over a 16-year study period
(1986–2001), 60 neonatal patients (under 28 days) were
studied. Normal and mildly abnormal were considered
good outcome. Group B streptococci were the most
common causative pathogens, accounting for about 32%
of the episodes. Escherichia (E) coli, the second common
pathogen, was more frequently noted in the second time
period. Moreover, 73% of E. coli strains and 10% of
Group B streptococci isolates were resistant to ampicillin.
The overall mortality rate was 10%. However, if those with
poor outcomes were considered, 38% would be considered
treatment failures. Significant prognostic factors included
the presence of seizures, thrombocytopenia, high CSF
protein concentration, and low CSF glucose concentration.
Initial empirical antibiotics with third-generation cephalosporins
should be considered for the majority of meningitis
cases resulting from infection with Gram-negative bacilli
and streptococcal species. Despite the availability of
newer and more potent antibiotics, the outcome of neonatal
bacterial meningitis remains unsatisfactory. There has been
increasing incidence for the emergence of resistant strains
presenting a therapeutic challenge in resent years. Early
diagnosis and choice of appropriate antibiotics according
to in vitro antimicrobial susceptibility and epidemiologic
trends are essential to maximize the potential for survival.
FP-G-045
The use of intravenous immunoglobulin in subacute
sclerosing panencephalitis: a retrospective cohort study
M.B. Lukban, B.C. Chua, A.M. Salonga, M.L. Bolanos,
B.C. Sanchez
Section of Pediatric Neurology, Departments of Neurosciences
and Pediatrics, University of the Philippines-Philippine
General Hospital, Manila, Philippines
In the search for a treatment for SSPE, Gurer et al. in 1996
reported the successful use of IVIg in SSPE. A retrospective
cohort study was done to compare the clinical outcome of
SSPE patients who received IVIg to those who did not
(control group). The clinical stages before the initiation of
IVIg treatment and serially after from 1 to 36 months were
noted. For patients in the control group, their clinical
outcome was followed from the approximate time of treatment
in the IVIg group (mean ¼ 7 months of illness). Clinical
outcome parameters noted were improvement in the
clinical staging, stabilization of the illness, and deterioration
in staging. There were 33 patients in the IVIg group with a
mean age of 9.8 years. The control group had 26 patients
with a mean age of 10.1 years. Among patients in stages 1
and 2 of the disease, 31% (5/16) improved within 1 until 6
months of the treatment as compared to 0% (0/12) in the
control group. Among patients in stages 3 and 4 of the
disease, 12% (2/17) in the IVIg group improved within 1
month of the treatment however, this decreased to 6% (1/17)
by 6 months from treatment whereas none improved in the
control group. The use of IVIg in SSPE may produce a
transient improvement in the clinical staging of the disease
for up to 6 months among patients in the earlier stages of the
disease.
FP-G-046
Risk factors for the development of subacute sclerosing
panencephalitis among pediatric patients in the
Philippine General Hospital
B.C. Chua, A.M. Salonga, M.B. Lukban, B.C. Sanchez
Section of Pediatric Neurology, Departments of Neurosciences
and Pediatrics, University of the Philippines-Philippine
General Hospital, Manila, Philippines
SSPE is a late complication of measles infection the incidenceofwhichappearstobeincreasinginthePhilippines.The
risk factors for the development of SSPE still remain undetermined.
The medical records of the pediatric patients diagnosed
with SSPE in the Philippine General Hospital from
July 1998 to June 2001 were reviewed to determine the risk
factors for the development of SSPE. The subjects for the
control group are children without SSPE seen in the outpatient
follow-up clinic unrelated to the SSPE subjects. Information
on sex, race, family size, birth order, socio-economic status,
residence, educational attainment of parents, medical history,
nutritional status and circumstances surrounding the initial
measles infection of the patients were obtained. The Chisquare
test was used to analyze the relationship between
these factors and the development of SSPE. Thirty-four
patients were included in the SSPE group and 29 patients in
the control group, with a male to female ration of 1.43:1 in the
SSPE group and 1:1.6 in the control group. Among the factors
analyzed, children who contracted measles at less than 24
months of age have a statistically significant increased risk
of developing SSPE ( £ 2 value-8.94, P ¼ 0.001). Male sex, a
history of a sibling who contracted measles at the same time as
the patient did, and the absence of measles immunization can

480
Abstracts
beconsideredasriskfactors,however,thevaluesarrivedatdid
not reach statistical significance. This may be due to the small
sample size.
FP-G-047
Levels of IL1, IL6, and IL8 in serum in viral encephalitis
in children
J. Zhang
Department of Neurology Hunan Children’s Hospital,
Hunan, China
Objectives: Viral encephalitis (VE) is one of the most
frequent infectious diseases of the central nervous system
in children. The prognosis is closely associated with the
severity of the infection. In order to identify the related pathogens
of the infection, we tested the levels of cytokines IL1,
IL6, and IL8 in serum of the patients with viral encephalitis.
Methods: Twenty three patients admitted to the neurology
department of our hospital were randomly selected, as well as
30 normal healthy children. We took blood samples from all
participants early in the morning before they took any food.
ELISA was used to test the levels of IL2, IL6, and IL 8 in
serum. Results: IL1, IL6, and IL8 in the VE patients were
significantly higher than those in the normal controls
(P , 0:001). Conclusion: The higher levels of IL1, IL6,
and IL8 in serum from children with VE indicated that
viral infection and following brain injury may activate
microglial cells to produce IL1. IL1 may stimulate microglial
cells or astrocytes to express IL6 and IL8. All this cytokine
activation may induce secondary neural cell injury and make
more severe functional defects of the central nervous system.
FP-G-048
Effect of heat shock response on brain tissue in rats with
infectious brain edema
D.-A. Mao
The Second Xiang-Ya Hospital of Central South University,
Changsha, China
Objective: To explore the effect of heat shock response on
brain tissue in rats with infectious brain edema. Methods:
Sprague–Dawley (SD) rats were randomly divided into
three groups: control group, normal saline treated rats (NS)
and infectious brain edema (PB) group. The rats were administered
pertussis bacilli (PB, 0.2 ml/kg) injected into left
ICA without pretreatment. In the pretreatment with heat
shock (HS 1 PB) group, the rats were pretreated with heat
shock before being injected with PB into left ICA. The rats
were killed at 8, 24 h after injected PB or NS, respectively.
The WC, cations of Na 1 and K 1 in the brain tissue were
measured and the morphological changes were studied by
electron microscopy. Results: The WC and Na 1 content
were significantly decreased in the HS 1 PB group
compared to the PB group (P , 0:01). The K 1 content in
HS 1 PB group was significantly higher than those in the
PB group at all time points except for 24 h (P , 0:01). The
electron microscope revealed that neurons were severely
swollen and the nuclei were slight pyknotic in PB group at
8 h, the processes of astroglial cells were moderately swollen,
mitochondria of neurons were enlarged in HS 1 PB group at
8 h. Dark cells and broken astroglial cells characterized cells
of PB group at 24 h. Dark cells and light cells, processes of
astroglial cells of perivascular region were severely swollen
and neuronal cells widened in HS 1 PB group at 24 h.
Conclusions: Heat stress response had a neuroprotective
effect on the infectious brain edema in rats, the effect may
be associated with the reduction of WC, Na 1 content and
enhancement of K 1 content.
FP-G-049
Clinical evaluation of high dosage intravenous
immunoglobulin on treatment of acute paralysis in
children
S.-H. Guo, R.-M. Hu, R.-P. Sun
Department of Pediatrics, Qilu Hospital of Shandong
University, Jinan, China
Objective: To evaluate the clinical effects of high dosage
intravenous immunoglobulin on treatment of acute paralysis
in children. Methods: 72 cases of admitted acutely paralyzed
children were randomly divided into two groups. There were
42 cases in the experimental group, which were given high
dosage intravenous immunoglobulin (400 mg/kg per day for
5 days). There were 30 cases in the control group, which were
given regular treatment. We compared the recovery period of
muscle force and the duration of hospitalization in the two
groups. Result: The recovery period of muscle force of the
experimental group was shorter than the control group
(3.18 ^ 1.38 versus 12.94 ^ 5.19 days P , 0:01). The average
duration of hospitalization of the experimental group was
shorter than that of the control group (14.45 ^ 4.18 versus
21.49 ^ 5.83 days; P , 0:01). The experimental group had
excellent results during an average of 3 months follow-up.
The validity rate in the experimental group was higher than
that of the control group (95.2 versus 86.6%). Conclusion:
Application of high dosage intravenous immunoglobulin in
acutely paralyzed children can increase cure rate and shorten
the average duration of hospitalization. IVIG should be
broadly used in acute paralyzed children.
FP-G-050
Clinical evaluation of the role of ancillary investigations
in the diagnosis of cerebral cysticercosis in 51 patients
G.-L. Li a , D.-Y. Zhou a , X. Yan b
a Harbin Children’s Hospital, Harbin, and b No. 203 Hospital
of P.L.A, Qiqiha’er, China
Objective: To evaluate ancillary investigations in the

Abstracts 481
diagnosis of patients with cerebral cysticercosis. Method:
We reviewed and analyzed ancillary investigations including
cysticercosis skin test (N: 38), cranial MRI (N: 13),
cranial CT scan (N: 45) and strengthened cranial CT scan
(N: 42) in 51 patients diagnosed with cerebral cysticercosis.
Results: Thirty eight cases had the cysticercosis test and 20
of them were positive, with the sensitivity rate 52.6% and
specificity rate 100%. The sensitivity and specificity of
cranial MRI/CT scan and strengthened cranial CT scan to
the low density areas in the brain were 84.6, 44.4 and 100%,
respectively. Our data showed that cranial CT scan had
poorer specificity in the diagnosis of cerebral cysticercosis
but strengthened cranial CT scan was much better in showing
low-density areas of cerebral cysticercosis. Conclusion:
Strengthened cranial CT scan may be used as a good method
in the diagnosis of cerebral cysticercosis.
FP-G-051
Study on the damage to peripheral nerves induced by
Campylobacter jejuni exotoxin
L.-S. Xie, F.-C. Cai
Department of Neurology, Children’s Hospital, Chongqing
University of Medical Sciences, Chongqing, China
To explore the pathogenesis of the damage to peripheral
nerves induced by C. jejuni exotoxin (CJT), the CJT was
extracted from Penner’s serotype 19 of the bacterium and
injected perineurally and intravenously in Wistar rats,
respectively. The animals were sacrificed and their sciatic
nerves were examined for teased fibers, transverse section
with toluidine blue stain and electron microscopy; sections
of sciatic nerves in either normal rats or humans were preincubated
with CJT and the pathological sciatic nerves
induced by CJT were obtained for observation of the binding
capability of CJT with peripheral nerves by streptavidinbiotin-peroxidase
complex (SABC) and FITC-immunofluorescent
methods, and nucleic acid hybridization techniques
for detection of TNF-a mRNA expression in
pathological sciatic nerves samples. The results showed:
(1) pathological changes were identified in 76.8% of teased
fibers after perineural injection, but only 9.6% fibers were
damaged in the control group (P , 0:01). The fiber damage
(19.5%) was found on the 3rd day after CJT intravenous
injection, and abnormalities still in 15.5% on the 14th
day. However, no abnormal changes were demonstrated in
the control group, and in the groups with the injection of
anti-CJT sera and the supernatants of peripheral blood
mononuclear cells (PBMCs) (P . 0:05). (2) Positive
combination of CJT was found predominantly in the sciatic
nerves of normal rats or humans incubated with CJT and
also in the pathological sciatic nerves induced by CJT.
Conclusion: CJT could remarkably damage the peripheral
nerves in rats, but the immunological pathogenicity of CJT
could not be demonstrated in the nerves of rats after immunization
with CJT.
FP-G-052
Levels of b-EP in serum and cerebrospinal fluid and
changes in erythrocytes immunological function in
children with nervous system infectious diseases
Y. Chen, X.-H. Wang, Q.-Y. Meng
Department of Pediatrics. The First Affiliated Hospital of
Jiamusi University, Jiamusi, China
Objective: To study the levels of b-EP in serum and
cerebrospinal fluid and the changes in erythrocytes immunological
function in children with nervous system infectious
diseases. Methods: Fifty-six children with nervous
system infectious diseases (observation group) and 44
aged-matched children with no nervous diseases (control
group) were selected. Their levels of b-EP in serum and
cerebrospinal fluid, ECR1 and their nitrate reductase
(NtrR) were assayed. Results: The levels of b-EP in serum
in the observation group were higher than in the control
group (P , 0:001). The levels of b-EP in cerebrospinal
fluid in the observation group were higher than that in the
control group too. Compared with the control group, the
quantity of ECR1 showed no obvious changes (P . 0:05)
and NTRR decreased in the observation group (P , 0:001).
The more severe the disease condition was, the higher the
levels of b-EP in serum and cerebrospinal fluid were, and
the lower the NTRR was. Conclusion: b-EP had dual regulation
of erythrocytes immunological function, and both of
them related to the disease condition. Because the levels of
b-EP in serum and cerebrospinal fluid rose in children with
nervous system infectious diseases, their erythrocyte immunological
function was suppressed.
FP-G-053
A case of streptococcus acidominimus meningitis
Q. Zhen
Department of Pediatrics, Anhui Provincial Corps Hospital,
Hefei, China
A 2-year-old boy had chopsticks poked into his pharynx,
which left a small hole in his pharynx. Next day, he had a
fever, cough and whoop with a convulsion. White blood cells
were increased. Chest X-ray showed bronchopneumonia.
Fever, cough and whoop disappeared after treatment by penicillin
and ampicillin for 20 days. Chest X-ray was normal.
However, the child became agitated and irritable. Hypertonia
was found in his right limbs. He was unable to stand and hold.
Five days later, fever relapsed. The patient received penicillin,
ampicillin and chloromycetin intravenous infusion for 10
days. Symptoms had not remitted. Therefore, he was transferred
to our hospital. Temperature was 39.18C. No neck
stiffness was found. Right Babinski sign was positive.
Peripheral WBC was 23.5 £ 10 9 /l, with neutrophils (N)
87%, lymphocytes (L) 10%, eosinophils (E) 1%, monocytes
(M) 2%. CSF was turbid, with the WBC 4050 £ 10 6 /l, includ-

482
Abstracts
ing N 95%, L 5%. In CSF, the protein was 540 mg/l, glucose
0.8 mmol/l, chloride 120 mmol/l. Streptococcus acidominimus
were found in the CSF culture. The child was treated
with ceftriaxone 1.0/day, dexamethasone 2.5 mg/day intravenously.
He was better from 2 days after treatment. The
temperature declined to 37.48C after 4 days treatment. But
2 days later, the temperature was up to 398C. He was irritable
again. The parents of child gave up treatment. Streptococcus
acidominimus belongs to the group of Streptococcus viridans.
It is a Gram 1ve coccus. It resides in respiratory tract
and enteric tract of human being. Streptococcus acidominimus
is a low-virulent pathogen. It is seldom that Streptococcus
acidominimus causes meningitis.
FP-G-054
A case of cryptococcosis accompanying ascariasis of the
biliary tract
X.-D. Wang
Department of Pediatrics, The Chinese People’s Armed
Police Forces Hospital, China
We treated a patient with cryptococcosis accompanying
ascariasis of the biliary tract in June, 1996. A boy, 6 years
old, came from the countryside of Henan province. His chief
complaints were abdominal pain and jaundice for 70 days,
and headache, vomiting, cough and fever for 40 days. Physical
examination found a large head circumference, about 58
cm, a 1 cm long skin lesion on the top of his head and a
setting-sun eye sign. The left eye had strabismus. His neck
was stiff. The liver was palpable at 4 cm below the costal
margin. Deep tenderness was found in the right upper quadrant
of the abdomen. The muscle strength of limbs was
grade IV. Knee and ankle reflexes were weak, Kernig’s
and Brudzinski’s sign were positive. Laboratory examination:
cryptococcus was found in CSF and secretion of skin
lesion. Cranial CT showed a low density lesion. Ultrasonogram
showed ascariasis of the biliary tract. Treatment:
amphotericin B associated with 5-flucytocin was used to
treat cryptococcosis. One month later, his general condition
was obviously better. The setting-sun sign disappeared. The
skin lesion and cough were cured. His neck was flexible.
Cryptococcus had not been found in CSF for three consecutive
examinations. Cranial CT demonstrated that the low
density lesion disappeared completely.
FP-G-055
Outbreak of Coxsackie virus B meningitis in Xuzhou
district of China
B.-Q. Yuan, H. Cheng, A.-H. Guo, M. Lu, T.-Y. Xie, L.
Pang, S.-G. Lu
Department of Pediatrics, The Affiliated Hospital of Xuzhou
Medical College, Xuzhou, China
An outbreak of aseptic meningitis due to Coxsackie virus
B occurred in the Xuzhou district, north of Jiangsu in China,
during May–August 2001, with 262 cases fitting the clinical
case definition. Medical files were reviewed and a standard
questionnaire administered. Viral PCR including Coxsackie
virus B, echovirus, encephalitis B virus and adenoviruses
were performed on cerebrospinal fluid. Meanwhile indirect
immunofluorescence assays were made on serum. Coxsackie
virus B was isolated in 39 of 75 (52%) cerebrospinal fluid
specimens with RT-PCR and 69.8% of 254 cases in serum.
The clinical presentation and laboratory findings were typical
of viral meningitis. Cases were aged 28 days to 49 years;
90% of patients were aged around 3 years. Headache was
present in 20%, photophobia in 10%, vomiting in 40%,
fever in 80%, and neck stiffness in 36.8%. A total of 10%
of cases had seizures, and 84.5% of cases had abnormal EEG.
One case died and other patients got well. In spite of temporal
clustering, the mode of transmission in this outbreak
remained speculative. Respiratory transmission was possibly
responsible for this outbreak of Coxsackie virus B meningitis.
FP-G-056
A clinical values of NSE, NO/NOS changes in
cerebrospinal fluid in patients with various nervous
system diseases
Z. Huang, Q. Chen, C.-F. Zai
Children’s Hospital, Chongqing University of Medical
Sciences Chongqing, China
Objective: To determine both NSE and NO/NOS in CSF.
We also investigated their regular changing pattern, their
relationship, their influence on the originating, developing
and recovering of various nervous system diseases. Their
clinical values were illustrated. Methods: A total of 115
cases with nervous system diseases including 18 GBS, 30
epilepsy, 28 bacterial meningitis and 40 acute viral encephalitis
were studied. Twenty age-matched children with
normal neurologic development were taken as the control
group. NSE and NO/NOS levels in CSF were studied by
ELISA enzymo-immunoassay and Griess chemical colorimetric
methods, respctively. Results: (1) NSE, NO/NOS
levels in CSF were significantly higher in all disease groups
(5.309 ^ 0.59 , 11.91 ^ 4.28 ng/ml, 2.45 ^ 0.58 , 6.89
^ 0.89 mmol/ml, 4.44 ^ 0.89 , 12.60 ^ 2.10 mmol/ml
per min, respectively) than those of the control group
(4.11 ^ 0.431 ng/ml, 1.90 ^ 0.41 mmol/ml, 2.82 ^ 0.65
mmol/ml per min). (2) The CSF-NSE levels were at highest
level in the bacterial meningitis group (11.91 ^ 4.28 ng/ml),
and NO/NOS levels in CSF were at highest level in the
epilepsy group (6.89 ^ 0.89 mmol/ml, 12.6 ^ 2.10 mmol/
ml per min). (3) NSE to NO, NSE to NOS and NO to
NOS in CSF changes were markedly correlated
(r ¼ 0:57 , 0:78, P , 0:05 , 0:01) in all groups of
subject. Conclusion: (1) NSE, NO/NOS levels in CSF
were elevated to varying degrees in patients with nervous

Abstracts 483
system diseases. These illustrated the definite neuronal and
glial cell damage in these diseases, including GBS, epilepsy,
bacterial meningitis and acute viral encephalitis. (2) As the
biological marker of living neurons, NSE and NO/NOS CSF
changes correlated (r ¼ 0:57 , 0:78, P , 0:05 , 0:01).
That indicated that they all play an important role in
diseases’ originating and developing course. (3) NSE levels
in bacterial meningitis and epilepsy were highest, associated
with the highest levels of NO/NOS. This implied the most
serious neuronal and glial cell damage, NO/NOS might
make this damage more serious by their cytotoxic effects.
In GBS, mainly due to immunological damage of axon and
myelin of peripheral nerves, the neuronal damage was not
serious, so NSE levels were elevated mildly, as were NO/
NOS levels.
FP-G-057
A child with acute pandysautonomia: recovery after two
courses of intravenous high-dose immunoglobulin
therapy
M. Ishitobi, K. Haginoya, T. Kitamura, M. Munakata, H.
Yokoyama, K. Iinuma
Department of Pediatrics, Tohoku University School of
Medicine, Miyagi, Japan
Acute pandysautonomia is a rare disorder, characterized
by acute dysfunction of the autonomic nervous system with
history of preceding infection. It is recently recognized as a
neuroimmunologic disorder like Guillain-Barre syndrome,
and is occasionally treated with IVIG. Here we report a
previously healthy 11 year-old boy, who suffered from
severe orthostatic hypotension after a febrile episode.
Serial examination of the orthostatic test and coefficient
of variation in the R-R intervals showed complete recovery
after two courses of IVIG, which were given 3 months
from the onset. One or more courses of IVIG should be
considered to treat this disorder without spontaneous
remission.
FP-G-058
Availability of cerebrospinal fluid ferritin for early
diagnosis of bacterial meningitis in children
Y.-O. Kim, Y.-J. Woo, M.-H. Youm, E.-Y. Kim
Department of Pediatrics, Chonnam University Hospital,
Kwangju, Korea
Purpose: To determine the normal CSF ferritin level
according to age and the cut-off value for early diagnosis
of bacterial meningitis. Methods: 203 children (27 weeks
gestation to 16 years old) were classified into four groups:
non-meningitis (73), aseptic meningitis (76), bacterial
meningitis (26), and bacterial meningitis suspected group
(28). WBC, protein, glucose and ferritin in CSF were
analyzed in each group. Results: CSF ferritin level in the
bacterial meningitis group was significantly higher than in
the non-meningitis group or the aseptic meningitis group.
The CSF ferritin had positive correlation with WBC and
protein in CSF, but negative with CSF glucose
(P , 0:01). The CSF ferritin decreased up to 1 year old
but was not related to age over 1 year in the non-meningitis
group. For early diagnosis of bacterial meningitis, 15.58 ng/
ml was considered as the appropriate cut-off value of CSF
ferritin with a sensitivity of 96.2% and a specificity of
96.6% in this study. Conclusion: The normal CSF ferritin
level according to age was determined and it showed
decreasing levels up to 1 year old including the premature.
CSF ferritin level of 15.58 ng/ml was considered as an
appropriate cut-off value for discriminating bacterial meningitis.
FP-G-059
Enterovirus 71 infection with severe neurological
complications
M.A. Nolan, M. Lahra, M. Craig, P.C. Prager, W.D.
Rawlinson, G.D. Williams, P.I. Andrews
Sydney Children’s Hospital, Sydney, Australia
Enterovirus 71 (EV71) is a picornavirus. It is usually
associated with hand, foot and mouth disease, a mild,
self-limiting febrile illness. Neurologic complications are
infrequent but include aseptic meningitis, acute flaccid
paralysis (AFP) and brainstem encephalitis (BSE). A
distinctive syndrome due to invasive EV71 infection in
infants and young children has emerged. It is characterised
by acute neurogenic pulmonary edema (PE), acute respiratory
failure, BSE, AFP and characteristic MRI signal
abnormalities in pons, medulla and spinal cord, and is
almost universally fatal. We describe seven patients with
this distinctive syndrome who survived the acute illness.
The acute cardiopulmonary crisis was managed with
mechanical ventilation, aggressive after load reduction
and moderate inotrope support. Other acute therapies
included pleconaril (anti-enteroviral agent, unproven activity
against EV71; n ¼ 4), high-dose intravenous steroids
(n ¼ 4) and IVIG (n ¼ 3). Variable neurological improvement
occurred within the first 2 months. The six patients,
who have survived at least 18 months, have severe, residual,
motor dysfunction, including flaccid paresis of one or
more limbs, cranial nerve palsies and ventilatory failure,
with preserved cognition and sensation. Residual motor
deficits are consistent with persistent MRI signal abnormalities
in the respiratory centre and other regions of the
brainstem, phrenic nerve nuclei, and anterior grey matter
of the spinal cord. Clinical improvement after the first 12
months has been limited, and four require ongoing ventilatory
support. At least three epidemics of EV71 infection
associated with this syndrome have occurred in the last 5
years, and there is great need for effective immunisation
and specific therapy.

484
Abstracts
FP-G-060
Spastic paraparesis and encephalpathy associated with
HTLV-1 infection in Argentinian seronegative children
L. Czornyj, A. Mangano, L. Sen, N. Fejerman
Hospital de Pediatria ‘J.P. Garrahan’, Buenos Aires,
Argentina
Human T-cell leukemia virus-1 (HTLV)-1 has been associated
with T cell-leukemia and myelopathy/tropical spastic
paraparesis. There have been some reports of adult patients
with spastic paraparesis (SP) and asymptomatic individuals
carrying detectable tax sequences in PBMC but were
HTLV-1 seronegative. We studied ten children (six girls
and four boys) three with SP and seven with encephalopathy
(E), who were referred for diagnosis of HTLV-1 infection
by serologic and molecular assays. They had an average age
of 43.9 months (ranging from 3 months to 16 years). Detection
of antibodies to gag and env HTLV-1/2 was accomplished
by gelatine particle agglutination and Western
blotting test. HTLV tax and pol sequences were investigated
by nested PCR amplification from PBMC, and then
subtyped by RFLP for HTLV-1 and HTLV-2.All children
were HTLV-1/2 seronegative. HTLV-1 tax sequence was
present in the ten patients studied while the pol fragment
was absent in the seven cases checked. Although preliminary,
the presence of tax sequences in the absence of pol
sequences and without detectable antibodies to HTLV-1
gag and env in children with neurological disorders, may
suggest an HTLV-1 infection with a defective provirus.
FP-G-061
Multiple sclerosis in children: a case series
M.A.M. Prudencio, M.H. Ortiz, E.L. Avendaño
Child Neuroscience Division, Philippine Children’s Medical
Center, Quezon City, Philippines
MS is a chronic relapsing, remitting disease characterized
by neurologic symptoms referable to lesions disseminated
throughout the neuraxis with a propensity for spontaneous
improvement. This disease is rare among infants and children
with an incidence of 2.7%. It is even rare in children
less than 10 years of age. Three boys aged 4–9 years and one
girl aged 15 years were diagnosed as having MS at the
Philippine Children’s Medical Center (PCMC) within a 3
year period (1996–1999). All presented with multiple
neurologic deficits occurring with a relapsing and remitting
course. Neurodiagnostic evaluations included the following:
CSF IgG was elevated in two/four cases (50%) and abnormal
evoked responses (visual evoked response, somatosensory
evoked response, brainstem auditory evoked response)
were noted in three/four cases (75%). Cranial MRI done in
all patients showed presence of nodular lesions with
increased T2 signals involving different areas of the white
matter. Improvements were observed with steroid administration.
FP-G-062
Recurrent purulent meningitis in Thai children
S. Chiemchanya, A. Visudtibhan, J. Patrungsri,
P. Visudhiphan
Division of Neurology, Department of Pediatrics, Faculty of
Medicine, Ramathibodi Hospital, Mahidol University,
Bangkok, Thailand
Background: Recurrent purulent meningitis in children
was rare and the diagnosis for the causes remained problematic.
There were few reports concerning this illness in
children. Objective: To study the causes of recurrent purulent
meningitis in children. Method: A retrospective study
was conducted by reviewing medical records of all pediatric
patients who had purulent meningitis admitted at the
Department of Pediatrics, Ramathibodi Hospital between
January 1, 1977 and December 31, 1997. Result: Nineteen
children were included in this study. There were 13 patients
who had more than two episodes of purulent meningitis
before presenting for evaluation. Seventeen children had
anatomical defects, which were congenital CSF fistula (11
children) and acquired diseases causing leakage of CSF (six
children). Those with congenital fistula comprised five children
with inner ear anomaly, five children with meningoceles,
and one child with cribriform plate defect. Among
those with acquired diseases, five had traumatic CSF fistula
and the other had chronic otitis media. There were two
children who did not have either demonstrable anatomical
defect or immune deficiency disease. Streptococcus pneumoniae
was the most common causative microorganism.
During follow up evaluation ranging from 1 to 10 years,
one child with temporal bone defect had recurrent meningitis.
However, after second surgical treatment, there was no
recurrence. Conclusion: Anatomical defect was the most
common cause of recurrent purulent meningitis observed
in this study. Complete investigation to exclude any congenital
defect is recommended in children with recurrent purulent
meningitis.
FP-G-063
Herpes simplex encephalitis in pediatrics diagnosed by
CSF polymerase chain reaction: a report of three cases
and review of literature
A.L.F. Luat
Child Neuroscience Division, Philippine Children’s Medical
Center, Quezon City, Philippines
HSE is an acute, devastating infection of the central
nervous system that causes significant morbidity and
mortality. The clinical presentation of HSE includes a
syndrome of acute onset characterized by fever, headache,

Abstracts 485
seizures, focal neurological signs and impaired consciousness,
however, cases of atypical HSE have also been
reported. Early diagnosis of HSE is important because it
is the only viral infection of the central nervous system for
which treatment has been proven effective in rigorous clinical
trials. The diagnosis, however, is difficult because of
the protean clinical manifestations and the inability to
isolate virus from the cerebrospinal fluid. Both the detection
of intrathecal HSV antibody and HSV antigen are
retrospective means of establishing the diagnosis. Brain
biopsy used to be the only means of making a definitive
diagnosis of HSE. The development of PCR as a diagnostic
procedure may contribute to the early diagnosis of HSE
and, therefore, early initiation of treatment and better therapeutic
outcome. We report three cases of pediatric HSE
diagnosed by CSF PCR and treated with Acyclovir. The
use of PCR may uncover a wider clinical spectrum of HSE
than has been previously recognized.
FP-G-064
Acute severe pediatric central demyelination
E. Shahar
Child Neurology Unit, Rambam Medical Center, Haifa,
Israel
The different clinical presentations, medical therapy and
current outcome are reported in a series of thirteen children
diagnosed with acute severe central demyelination
(ASCDM), namely acute disseminated encephalomyelitis
(ADEM) or spinal cord multi-focal myelopathy (SMM):
seven males and six females presenting at age of 2.5–14
years (mean: 6.6). Four children developed ADEM,
presenting with deteriorating alertness and developing
coma along with cranial nerves and brainstem involvement,
brisk tendon reflexes and extensor plantar responses.
Seven children developed SMM with flaccid pyramidal
weakness of limbs, mainly the lower limbs, of whom
seven became bed-ridden, along with bladder and bowel
incontinence, without apparent change in alertness. All
ADEM patients were treated with high-dose methylprednisolone
(HDMP), of whom three completely recovered
within 5–7 days. An additional child, with combined
myelo-radiculoneuropathy (MRN), also treated with
IVIG, failed both therapies and remains handicapped. Of
the seven bed-ridden SMM patients, six were initially treated
with HDMP of whom five completely recovered within
2–9 days (range: 5.83). The seventh patient with West Nyle
Fever MRN, was also given IVIG but remains handicapped.
Another patient with SMM initially failed oral
steroids and subsequently fully recovered with IVIG. Overall,
eight of eleven children with ASCDM, given HDMP
completely recovered. Therefore, HDMP or IVIG, either
given separately or even combined in protracted cases,
seems to be efficacious in severe debilitating pediatriconset
ASCDM with negligible adverse-effects.
FP-G-065
Bacterial meningitis during the season of flu epidemic
Z. Milenkovic, K. Karovski, N. Pop-Jordanova, V.
Markovski, P. Stojovska, R. Naumovski
Clinic of infectious diseases, Faculty of medicine, Skopje,
Macedonia
In an open, randomized, controlled study on 118 patients
with purulent meningitis (PM) treated at Clinic of infectious
diseases in Skopje during the season of flu epidemic (test
group, tg) in the last 5-year (1997–2002) period, some
biochemical, clinical and microbiological characteristics
have been analysed. The results obtained were compared
with the findings of 186 patients with PM treated outside
that period (control group, cg). The etiology of disease was
confirmed by bacterial isolation from CSF in 55.6% of
cases. The most frequent etiological agent in both groups
was Str. pneumoniae. Biochemical and microbiological
findings: lower initial CSF WBC count (898 ^ 788
(87.7%) versus 1.699 ^ 1.008 (59.3%) £ 10 6 /l) with relatively
higher CSF protein level (1.86 ^ 1.36 (73.1%) versus
2.06 ^ 1.46 (70.9%) g/l) and higher percentage of presence
of parameters of bacteriaemia (19.5 versus 16.7%), sepsis
and septic shock (37.3 versus 32.8%), in tg were found.
Clinical parameters: higher frequency of initially disturbed
state of consciousness (32.2 versus 30.6%) and transitory
neurological deficit (11.9 versus 8.6%), in the tg were found.
Contrary to our expectations based on the biochemical, clinical
and microbiological findings cited, the mortality rate
was lower in tg (10.2 versus 13.4%).
FP-G-066
Subacute sclerosing panencephalitis (SSPE): summary
of the California experience
M. Philippart
Brain Research Institute, UCLA, Los Angeles, USA
Measles vaccination almost eliminated SSPE, which still
presents challenges of diagnosis, treatment and pathophysiology.
The natural course has been distorted towards the
most severe cases, leading to early death. SSPE was
confirmed in 26/29 cases evaluated since 1979. Six cases
were born in California, two of them among four born in
1989 during the 400-case Riverside epidemic; two in other
States; 14 in Central America; one each in Belgium, Brazil,
Canada, and the Philippines. Two died within 6 months.
Fourteen with onset from 1965 to 2000 are still alive. One
survives 25 years after onset without treatment. One with a
Rasmussen presentation seems cured after a left hemispherectomy
in 1989. Five received Inosiplex only: one, in stage 4,
did not benefit but survived 8 years; one lived 20 years after a
recovery to stage 1, lasting 10 years; one was lost to followup
(F/U); one, who recovered to stage 2 for 8 years, is still
alive 15 years after onset. One with no benefit from recom-

486
Abstracts
binant interferon has been 15 years in stage 4. Fifteen were
treated with partially purified human interferon: four (stage
4) did not benefit, two of them were lost to F/U; three died
within 2 years of onset; three stabilized (9–15 years); five
improved: two from stage 2 (6 months, 4 years); two from
stage 3 (2 and 6 years), the last, who 6 months after onset sank
to stage 4, had the quickest biological response, IgG synthesis
rate/day falling from 59.6 to 6.8 mg (normal ,3.3).
FP-G-067
Atypical form of subacute sclerosing panencephalitis
related to vaccine measles virus
F.N. Arita, S. Rosemberg
Santa Casa of Sao Paulo School of Medicine, Department of
Pediatrics, Neuropediatrics Division, Sao Paulo, Brazil
SSPE is a rare progressive inflammatory disease of the
CNS caused by persistent mutant measles virus infection
whose onset occurs several years after the primary infection
in early life. Its occurrence fell dramatically after introduction
of measles vaccine. However, a few vaccinal cases have
been reported. It is estimated that this occurs at a rate of 0.5–1
cases per million of applied doses of vaccin. We report three
patients who had been vaccinated after the measles
epidemics of 1997 in Sao Paulo State and presented an
unusually early onset and rapid progressive course of the
disease. At onset, the ages of the patients were 2 years, 2
years 2 months, and 3 years, and all had been vaccinated,
respectively, at 14 months, 16 months, and 2 years 2 months
prior the onset. This third patient, however, had had measles
infection at age of 9 months, 1 month before immunization.
In all cases, the first symptoms consisted of frequent falls and
periodic myoclonic jerks occurring every 5 or 10 s. The EEG
showed typical periodic complexes. CSF protein electrophoresis
showed levels of g-globulins above 30 mg.% and
measles virus-specific antibodies were detected. The clinical
course was characterized by a dramatically rapid progressive
psycho-motor deterioration. In a few weeks all patients were
bedridden and unresponsive to external stimuli. One patient
died at 4 years 8 months of age and the others, aged 4 years 7
months and 5 years 3 months, are alive in a vegetative state.
These cases demonstrate that measles vaccine virus may
provoke a special clinical form of SSPE in which the incubation
period is short and the clinical course acute.
FP-G-068
Subacute sclerosing panencephalitis presenting with
hemiparesis: a case report
H. Ozyurek, A. Degerliyurt, G. Turanl
Hacettepe University, Department of Pediatric Neurology,
Ankara, Turkey
Subacute sclerosing panencephalitis (SSPE) is a slow
virus infection of the central nervous system characterized
by myoclonic seizures, involuntary movements, and mental
deterioration. SSPE is diagnosed with strikingly elevated
levels of measles antibodies in the serum and CSF in addition
to the clinical picture. Epidemiologic studies revealed
that the age at onset of SSPE (7.6–5 years) decreased especially
after 1995 in Turkey. In childhood, SSPE rarely
present with focal neurologic signs. A 14-month-old girl
was admitted because of intermittent fever and lethargy of
2 months duration, followed by left-sided hemiparesis. Her
past medical and family histories were unremarkable. Her
motor and mental development was normal. There was no
history of measles. During follow-up, firstly partial than
generalized seizures developed in the patient. In cranial
magnetic resonance, on T2-weighted images there was
hyperintensity of frontoparietal cortex bilaterally and
white matter especially in the right hemisphere. Electroencephalogram
showed periodic paroxysmal activity characterized
by suppression burst that did not disappear with
diazepam. There was delay in the latencies of visual evoked
potentials. Electroretinogram was normal. Urine sample for
organic acids and lysosomal screening was negative. The
lactic and pyruvic acid concentrations were normal in CSF.
Serology for rubella and herpes type I was negative.
Elevated measles antibody titers were detected in both
serum and CSF by complement fixation (1/512 and 1/16,
respectively). The patient was diagnosed with SSPE on
laboratory findings. In summary, symptoms of SSPE may
present as early as infancy like in Sour patient. Although
focal neurological signs are mostly seen in adults with
SSPE, they rarely may be a component of SSPE in childhood.
Thus one should also consider SSPE in infants with
focal neurological signs.
FP-G-069
Long-term follow-up of a patient with subacute
sclerosing panencephalitis successfully treated with
intrathecal interferon alpha
M. Miyazaki a,b , M. Nishimura a , Y. Toda a , T. Saijo a ,K.
Mori a , Y. Kuroda a
a Department of Pediatrics, Tokushima University School of
Medicine, Tokushima; b Miyoshi Medical Clinic, Kagawa,
Japan
We previously reported that a patient with SSPE was
successfully treated with intrathecal interferon alpha (IFNa)
and his pronounced improvement was observed in a
dose-dependent manner (Ann Neurol 1991). To clarify
long-term effects of IFN-a in SSPE, we followed up the
patient for 17 years with neuroradiological and physiological
examinations. He was diagnosed with SSPE at age 8
years and treated weekly or fortnightly with 1.0 to
3.0 £ 106 IU IFN-a by intrathecal route. IFN-a therapy
induced remission of SSPE and he maintained a higher
quality of life for approximately 8 years compared with
other SSPE patients. At age 16 years, he gradually devel-

Abstracts 487
oped muscle rigidity, gait disturbance and difficulty in
speech. Increasing the dose of IFN-a to 5 £ 106 IU and
changing to IFN-a brought no remarkable benefit. Currently
he is a malnourished 25-year-old male with akinetic mutism
who barely responds to loud sounds and strong painful
stimulation. The electroencephalogram shows diffused low
voltage fast activity without a-rhythm, and brain CT scan
discloses severe brain atrophy in the cerebellum and brainstem,
as well as linear high density along the circle of Willis.
These results may indicate that IFN-a-induced remission is
most likely to be temporary, even if an SSPE patient shows
an excellent initial response to the treatment. To improve
the long-term prognosis of SSPE patients, more effective
therapy, such as a combination of IFN-a with novel agents,
still needs to be developed.
FP-G-070
CXCL10 production from cytomegalovirus-stimulated
human microglia: regulation by interleukin-10
S. Hu, M.C.-J. Cheeran, S.L. Yager, P.K. Peterson, J.R.
Lokensgard
Neuroimmunology Laboratory, Minneapolis Medical
Research Foundation; and the University of Minnesota
Medical School, Minneapolis, USA
Microglial cells orchestrate multi-cellular immune
responses to viral infections of the central nervous system
and synchronize various immune functions through a regulated
network of cytokines and chemokines. Recruitment of
T lymphocytes to local sites of infection is critical for resolution
of cytomegalovirus disease. In the present study, we
investigated the production of an alpha-chemokine
CXCL10 (interferon-gamma inducible protein-10, IP-10)
in response to CMV infection of glial cells and the regulation
of its production by IL-10. We found that human microglial
cells, but not astrocytes, produced CXCL10 in
response to CMV, reaching maximal levels at 48–72 postinfection.
Expression of CXCL10 mRNA in CMV-stimulated
microglia was not dependent on secondary protein
synthesis but did require replication competent virus. Activation
of NF-kappa B and p38 MAP kinase in microglial
cells was observed in response to stimulation with CMV and
inhibitors of this activation decreased viral-induced
CXCL10 production. Exogenous CXCL10 itself had no
effect on CMV replication in permissive astrocytes,
however, supernatants from CMV stimulated microglial
cells increased chemotaxis of activated T-cells, which
could be partially suppressed by anti-CXCL10 antibodies.
Anti-inflammatory cytokines appear to play a critical role in
preventing excessive CNS inflammation including T-cell
accumulation. Treatment of microglial cells with IL-10
was found to inhibit CXCL10 production at the level of
mRNA transcription and was associated with decreased
CMV-induced NF-kappa B activation. These studies
suggest that microglial cell production of CXCL10 plays a
major role in recruitment of T-cells to foci of CMV infection
and that IL-10 functions to prevent excess inflammation.
FP-G-071
Reactivation of HHV-6 infection in chronic
encephalopathy
C. Arpino a,c , L. Lopez a , G. Anzidei b , M.P. Camporiondo b ,
D. Poveromo c , P. Curatolo c
a Eugenio Litta Rehabilitation Center for Developmental
Disabilities; b IRCCS Infectious Diseases L. Spallanzani;
and c Pediatric Neurology Unit, Tor Vergata University,
Rome, Italy
We present a case of a 13 year-old girl with chronic
encephalopathy, whose recrudescence of symptoms was
associated with the reactivation of HHV-6 infection both
at the systemic level and at the level of the CNS. Since
the age of 3 years, she had suffered from frequent epileptic
seizures resistant to treatment, and at the age of 8 years she
began to present cognitive deterioration. At the time of our
evaluation, the girl showed intermittent episodes of delirium
and epileptic seizures. Her karyotype, blood and urine
screening for metabolic diseases, optic fundi, motor and
sensory conduction velocity studies, immunoglobulins and
T lymphocyte subsets, were normal. Interictal EEG showed
absence of normal background activity, and a prominent
slow delta activity bilaterally in the anterior leads. MRI
imaging showed mild, non-specific, diffuse cortical atrophy
prevailing in the frontal regions. CSF examination for DNA
sequences of several viral agents (i.e. Cytomegalovirus,
HHV-6, herpes simplex 1 and 2, Epstein-Barr virus, varicella
zoster virus) by nested polymerase chain reaction
revealed HHV-6 sequences, which were also detected in
peripheral blood mononuclear cells. HHV-6 is a neurotropic
virus; up to now, demyelination and seizures or status
epilepticus have been observed in primary disease (i.e.
exanthema subitum) or in immunocompromized children.
Our report suggests that human Herpesvirus-6 (HHV-6)
reactivation, at the CNS level, may occur also in immunocompetent
hosts with severe neurological disease. It remains
to be defined whether, in our patient, viral reactivation was
the mere consequence of illness-related stress or a factor
contributing to the recrudescence of CNS disease.
FP-G-072
Experimental study of endotoxic brain edema in infant
rats and its treatment
M. Li, F.-C. Cai
Department of Neurology, College of Pediatrics, Chongqing
Medical University, Chongqing, China
Glucocorticoids are considered as effective drugs in reducing
blood–brain barrier (BBB) permeability. IVIG is

488
Abstracts
frequently used to cure neurological disease, but its role and
potential mechanism in brain edema has not been reported.
In this study, the infant rat endotoxic brain edema was
produced by intraperitoneal administration of 10 mg/kg of
LPS, and the brain water and EB content, intracellular free
Ca 21 and CaM expression were observed. The results
showed that brain water and EB content were remarkably
increased in endotoxemic rats compared with controls. Electron
microscopy studies revealed that brain microvesicular
endothelial cells had more endocytotic vesicles, degeneration
and basal membrane disrupted and the space around
brain capillaries was enlarged. The increase of brain intracellular
free Ca 21 and up-regulation of cellular CaM expression
were coincident with increase of EB and water content
caused by LPS. Administration of DEX and IVIG could
markedly decrease brain water and EB content. These
results suggested that LPS increases the intracellular free
Ca 21 and active CaM-dependent function via the Ca 21 /
CaM pathway, which might relate to the increased BBB
permeability and brain edema formation. The two drugs
have the ability to reduce the BBB permeability and alleviate
brain edema via reducing the intracellular free Ca 21 ,
inhibiting the expression of CaM, and blocking the Ca 21 /
CaM signal pathway. But IVIG failed to reduce the intracellular
free Ca 21 , which suggests that other factors may be
involved in regulating the intracellular free Ca 21 .
FP-G-073
Neurological outcome of children born to
antiphospholipid antibody-positive mothers
B.M. Tabarki, S. Boudhir, N. Salem, T. Mahjoub, A.
Trabelsi, M. Yacoub, H. Selmi, A.S. Essoussi
Farhat Hached Hospital, Sousse, Tunisia
Objective: To evaluate the neurological features and
outcome of children born to antiphospholipid antibodypositive
mothers treated with calcium heparin and/or aspirin
during pregnancy. Design: Retrospective data collection
and prospective pediatric review of patients seen at Farhat
Hached hospital between 1997 and 2001. Results: Seventythree
children, with ages ranging from 10 months to 5 years,
born to mothers with antiphospholipid antibodies were
reviewed. Four patients (5%) died because of prematurity
and/or nosocomial infection. Sixty-one (84%) patients were
normal on neurodevelopmental and physical examination at
pediatric review. Eight patients (11%) showed neurological
abnormalities: severe intellectual and motor dysfunctions
associated with epilepsy (four patients), mild mental retardation
(two patients), hemiplegia (one patient), and epilepsy
(one patient). Neuroradiological findings were: diffuse cerebral
ischemia (four patients), middle cerebral artery infarction
(one patient), sinovenous thrombosis (one patient),
CNS malformation (one patient), and normal MRI in one
patient. Four of the eight patients had positive antiphospholipid
antibodies. Neurologic complications were not related
to prematurity, growth restriction or other diseases. Conclusion:
These data indicate that children born to antiphospholipid
antibody positive mothers are at increased of CNS
diseases, especially cerebrovascular disorders.
FP-G-074
Epidemiological and clinical features of 63 cases with
subacute sclerosing panencephalitis diagnosed in 4 years
Ö.F. Aydin, N. Şenbil, M. Kara, Y.K.Y. Gürer
Department of Pediatric Neurology, Dr. Sami Ulus Children’s
Hospital, Ankara, Turkey
SSPE is an inflammatory neurodegenerative disease
caused by persistence of measles virus. Although its
frequency is declining because of widespread measles vaccination,
it is still prevalent in areas where measles is endemic.
We reviewed 63 patients diagnosed with SSPE over a period
of 4 years (1998–2001). The diagnosis is based on the characteristic
clinical and electroencephalographic findings and
demonstration of an elevated antibody titer against measles in
the plasma and the cerebrospinal fluid. Fifty-one of the
patients were males (81%) and 12 were females (19%);
male/female ratio was 4.25. The mean age of onset of SSPE
was 68.29 ^ 29.87 months and follow-up duration was
9.85 ^ 7.49 months. The initial symptoms noticed by
families were unsteady gait/stumbling (26.9%), head drops
(20.6%), frequently falling (15.8%) and jerks (11.1%). Head
trauma was the precipitating factor in 14 cases (25.9%). Fiftythree
patients had had measles and 20 of 43 patients questioned,
hadreceivedimmunization. Themeanage ofinfection
was 15.68 ^ 11.6 months, mean latent period was
51.8 ^ 28.34 months. Mean time between first symptoms
and admission was 11.4 ^ 14.87 weeks. Major complaints
on admission were slurred speech (41%), frequent falling
(38%) and unsteady gait/stumbling (34%). Examination on
admission revealed myoclonic jerks in 53% of patients and
they were mostly at clinical stage 3 A (24.5%) and 3B
(33.3%). Thirteen of the 49 patients (26.5%) that could be
followed up died. Conclusion: it is necessary to re-evaluate
the immunization programme in preventing in SSPE, which is
a complication of an infection preventable by immunization.
FP-G-075
Evaluation of the treatment results of 63 cases with
SSPE diagnosed between the years 1998–2001
Ö.F. Aydin, N. Şenbil, Ö. Eǧritas¨, Y.K.Y. Gürer
Department of Pediatric Neurology, Dr. Sami Ulus Children’s
Hospital, Ankara, Turkey
Sixty three patients diagnosed as SSPE between the
years 1998–2001 were treated with oral isoprinosine (100
mg/kg per day), subcutaneous a-interferon 2a (Roferon-A,
Roche) 10 million U/m 2 /three times a week and oral lamivudin
(10 mg/kg per day). Patients that received treatment

Abstracts 489
according to this protocol for at least 6 months (19
patients) have been compared with 13 patients controls
that did not or could not take the treatment. On the basis
of neurological deficit index, clinical stage and the average
of ages, there was no statistical difference between the
treatment and control groups. There was no difference
between the groups on their final evaluation for neurological
deficit index and clinical stages. There was no statistical
difference between the exitus ratios of treatment and
control groups, 3 and 6, respectively. Remission ratios
between the treatment and control groups were 7/19 and
0/13, respectively and were statistically significant
(P ¼ 0:036). Survival time was significant in the treatment
group (P ¼ 0:01). The findings that remission ratios were
different in the treatment group compared with the control
group and also different from the 5% spontaneous remission
rate reported in previous studies, and that the survival
periods were remarkably different, has made us think this
treatment protocol is effective.
FP-G-076
Enteroviral meningitis in Northern Jordan: prevalence,
association of clinical presentation, laboratory findings,
and serotypes involved
A. Abdallah, M. Meqdam, M. Kbaloussi
Princess Rahma Teaching Hospital, Irbid, Jordan
During the summer-autumn of 1999,a total of 390 CSF
specimens from infants and children less than 15 years of
age, suspected of having meningitis, and admitted to Princess
Rahma Hospital. Northern Jordan, were studied to determine:
the incidence of enteroviral meningitis, serotypes
involved, clinical presentation, and laboratory data (shell
vial culture assay and fluorescent assay (FA) were used).
Most cases occurred in children of 1 year of age (46.9%),
and males represented 71.9% of the total of diagnosed enteroviral
meningitis. The dominant symptoms were fever,
vomiting, and headache. Clinical presentation did not distinguish
between children with and without enteroviral meningitis.
Enteroviruses were isolated from 32 (8.2%) cases.
Coxsackie virus B types 2, 4 and 5 were isolated from 15
(46.9%) cases, and ECHO virus 9 was the most commonly
identified serotype (31.3%). The analysis of bacterial and
viral CSF cultures revealed 2 (6.2%) out of 32 patients
with mixed viral/bacterial meningitis. The virus isolation
rate was directly proportional to the number of leukocytes
in CSF. However, enteroviral isolation was directly achieved
in four (12.5%) out of 32 CSF specimens without pleocytosis.
Leukocyte differential count revealed that 71.4% of the cases
showed polymorphonuclear cell predominance. Duration of
hospitalization ranged from 1 to 25 days with a mean of 7
days. The majority (88.9%) of enterovirus-infected patients
were treated with at least one type of antibiotic. According to
these data, we conclude that enteroviruses are frequent in our
area and this indicates the importance of enteroviruses as
common and important pathogens. Moreover, clinical
presentation and CSF cytochemical findings in patients
with enteroviral meningitis, closely resemble other bacterial
and viral infections, and viral culture is a leading tool,
together with promising advanced molecular techniques
such as the PCR, for the diagnosis of enteroviral meningitis.
FP-G-077
Host defenses in children with low birth weight against
pertussis, diphtheria and tetanus
N.G. Kim, D.I. Makhmudova, L.D. Mullaeva
Scientific Research Institute of Pediatrics, Tashkent, Uzbekistan
From 5 to 10% of infants with low birth weight, on the
basis of adequate weight increase and achievement of body
mass 2500 g, received the same vaccines at the same terms
as the full-term infants. However, the degree of physiological
maturation of newborn at the moment of birth is the
factor influencing the adequate immune response to
diphtheria and tetanus toxoids and pertussis vaccine
(DTTPV). The purpose of the investigation was to determine
degree of postvaccine immunity against diphtheria,
pertussis and tetanus in children with low-birth-weight.
Investigation was carried out on 40 healthy children and
51 infants with birth weight ,/ ¼ 2500 g and receiving
the scheme of PTTP vaccination. The children with lowbirth-weight
were divided into three groups: (1) premature
newborns with body weight correlated to age of gestation
(31–34 weeks, body weight 1600–2400 g) N ¼ 18; (2)
premature newborns with body mass lower than expected
for gestational age (30–35 weeks, body mass 1300–1500 g)
N ¼ 18. It was established that postvaccine response in
premature infants against diphtheria, pertussis and tetanus
was characterized by low level of immune production.
Premature infants with birth weight lower than expected
for gestational age failed to induce immunity against pertussis
in 60% of cases. Full-term infants with hypotrophic type
of intrauterine growth and development retardation failed to
produce defensive titres of antibodies against tetanus and
diphtheria in every 3-d case and against tetanus in 66.6% of
cases. Thus, efficacy of DTTP vaccination in children with
low birth weight was correlated to the degree of physiological
development of infant at the moment of its birth.
FP-G-078
Factors influencing the formation of postvaccine
immunity in children with damage of the central nervous
system
Z.A. Kushnaeva, D.I. Makhmudova, G.K. Salimova
Scientific Research Institute of Pediatrics, Tashkent, Uzbekistan
Objective: to study the effect of degree of severity and

490
Abstracts
type of clinical syndrome on the formation of postvaccine
antibodies in children with damage to the CNS. Investigation
was undertaken of 45 children with perinatal damage
of the CNS. Studied children were divided into the following
groups: group I with mild degree of disease (n ¼ 16);
group II moderate degree (n ¼ 20) and group III severe
degree (n ¼ 9); and according to clinical syndrome: ICH
(n ¼ 15) and vegetative visceral dysfunction (VVDF)
(n ¼ 10). Analysis of serological data in children with
mild degree of lesion were found 1.9 times more frequently
as compared to control group (P , 0:05). In children with
moderate degree of damage, negative results were registered
in 40% of cases. Frequency of low titres in children
from this group was 1.9 times more frequent (P , 0:05).
The frequency of moderate and high titres in children of
group II was 3.5 and 2.2, respectively (P , 0:05). In the
third group of children seronegative results against
diphtheria were 6.7 times more frequent than in children
of the control group (P , 0:05). The frequency of moderate
titres was 4.7 times higher in healthy children than in
children with severe degree of damage. The titres of antibodies
against diphtheria in the studied groups were as
follows: group I, 62.4%, group 2, 25%, group 3, 11.2%.
In children with ICH seronegative results with vaccination
were eight times more frequent. The frequency of moderate
antibody titres in this group was four times lower than in
control group (P , 0:01). In VVDF seronegative results
against diphtheria were 7.5 times more frequent
(P , 0:01). The frequency of moderate titres in children
of the main group was higher than 5.3 times (P , 0:01).
The high titres in children against diphtheria were 2.2
times lower in children of the main group (P , 0:05).
Host defenses against diphtheria in ICH were 13.3% and
VVDF 20%. Thus, severity and type of clinical syndrome
of children with damage of the CNS influence the results of
immunization against diphtheria. This category of children
should undergo study of the presence of protective
diphtheria antibodies after vaccination. It is necessary to
develop a scheme to prepare this category of children for
vaccination.
FP-G-079
Clinical analysis of 23 children with cryptococcal
meningitis
L.-Z. Zhang
Department of Pediatrics, Heze Municipal Hospital, Heze,
China
The mean age of 23 children with cryptococcal meningitis
was 4.3, 16 of them were misdiagnosed. The rate of
misdiagnosis was 69.6%. A total of 30.4% were diagnosed
correctly initially. Symptoms included fever, headache,
vomiting, visual loss, convulsions and swelling of the spleen
and liver. The principal changes in CSF were as in tuberculous
meningitis. The level of CSF glucose and chloride
decreased. Cryptococcus neoformans can be found by ink
stain technique. Low density changes were found in head
CT. It is suggested that in order to prevent misdiagnosis an
attempt is made to detect the pathogen. The prognosis is
related to the intracranial pressure, delay in correct diagnosis
and severity of inflammation.
FP-G-080
Clinical features of 25 cases of children with viral
encephalitis
B.-Z. Jia
General Hospital of Yangquan Coalmine Group Yangquan,
China
Objective: To explore the clinical feature of pediatric
viral encephalitis to enable earlier diagnosis. Methods:
The complete data of 25 cases of children with viral encephalitis
in the last 2 years were analyzed. Results: The rate
of common symptoms is: fever (96%), convulsions (64%),
vomiting (52%), disturbance of consciousness (44%), headache
(36%). The rate of rarer symptoms is: thrill (16%),
paralysis (12%), supranuclear paralysis (8%), dizziness
(8%), salivation (4%). Before admission into the hospital,
the median of common symptoms is: headache (2.25),
vomiting (2.1), fever (1.96), convulsions (1.57), disturbance
of consciousness (1). The rate of positive pathological signs
is: Babinski’s sign (28%), stiff neck (20%), Kernig’s sign
(4%). Conclusion: In clinical work, special attention should
be paid to the common symptoms of viral encephalitis and
high vigilance over a few symptoms. In the case of headache
and vomiting, with or without fever in the sick children,
viral encephalitis is possible.
FP-G-081
The study of nitric oxide tumor necrosis factor and
interleukin-8 in serum and cerebrospinal fluid in
children patients with infection of central nervous
system
H.-Y. Wang, Y. Yuan, B.-L. Sun
Department of Pediatrics, the First Affiliated Hospital of
Jiamusi University, Jiamusi, China
In this study we evaluated the levels of serum and CSF
NO, NOS, TNF and IL-8. The NO, NOS, TNF, IL-8 in 18
cases of viral meningitis (VM), 14 cases of tuberculous
meningitis (TM), 16 cases of purulent meningitis (PM)
and 20 control cases (CG) were determined by colorimetric
method and radioimmunoassay. Results: (1) The levels of
NO, NOS, TNF in serum and CSF with VM, TM and PM
were significantly higher than those in CG (P , 0:01).
Moreover, the concentrations of NO, NOS, TNF with PM
were significantly higher in CSF than those in patients with
VM and TM (P , 0:01). (2) The levels of IL-8 in serum and
CSF with TM and PM were significantly higher than those

Abstracts 491
in CG. While the concentration of IL-8 with PM was significantly
higher than those with TM, there was no obvious
difference between VM and CG (P , 0:01). All of these
suggested that: (1) NO, NOS, TNF, IL-8 all participated
in the pathogenesis of central nervous system infection.
(2) The determination of NO, TNF and IL-8 of serum and
CSF can be a discriminating index among VM, TM and PM.
FP-G-082
Clinical analysis of 68 cases with mumps encephalitis
W.-Y. Sun, Y.-B. Yan
People’s Hospital of Liaocheng, Liaocheng, China
Objective: The aim of the study was to investigate the
epidemiological characteristics and clinical effects of
mumps encephalitis. Methods: Sixty-eight cases of mumps
encephalitis were studied It is a common complication of
epidemic parotitis and has a high incidence rate. Forty-eight
of the 68 cases occurred from May to September (70.6%).
The patients were aged from 7 to 12 years. All patients were
treated by antivirals and replacement of fluid and electrolyte
losses. Mannitol and corticoid was only used in some
patients who had intracranial hypertension. All patients
were cured. Conclusion: Mumps encephalitis mainly
occurred in non-epidemic seasons and the pediatric age
group. Prognosis is good.
FP-G-083
One versus 2 years of antiepileptic therapy in children
with single small enhancing CT lesions
P.D. Singhi a , J. Dinakaran a , N. Khandelwal b , S.C. Singhi a
a Departments of Pediatrics and b Radiodiagnosis, Post
Graduate Institute of Medical Education and Research,
Chandigarh, India
Objective: The duration of antiepileptic drug (AED) therapy
in children with seizures due to single small enhancing
CT lesions (SSECTL) is controversial. We sought to determine
whether there is any difference in the rate of seizure
recurrence after 1 versus 2 years of AED therapy and to
identify the factors predictive of seizure recurrence. Methods:
A total of 115 consecutive children with seizures and
SSECTL were randomly assigned into two groups. Group A
received AED(s) for 1 year and Group B for 2 years seizure
free interval. CT scan and EEG were done prior to AED
withdrawal and children were followed up for seizure recurrence
for at least 1 year. Association between seizure recurrence
and clinical and CT characteristics was analyzed.
Results: Groups A and B consisted of 55 and 51 children;
(nine lost to follow up). There were 61 boys, 45 girls; mean
age 9.33 years. Most (93%) had focal seizures-36%
complex partial, 22% simple partial, 35% partial with
secondary generalization, 21% had status epilepticus. The
two groups were comparable in clinical, EEG and CT characteristics.
CT scan and EEG prior to AED withdrawal were
abnormal in 44 and 33%, respectively. Six children, three
from each group had seizure recurrence. Significant association
was found between seizure recurrence and abnormal
CT (persistence/calcification of lesion) and abnormal EEG
prior to AED withdrawal (P , 0:01). The relative risk of
seizure recurrence in a child with abnormal CT and EEG
prior to AED withdrawal was 26.2 (95% confidence interval
3.3–210.2, P ¼ 0:0003). No association was found between
seizure recurrence and any of the other variables. Conclusions:
There was no difference in seizure recurrence after 1
versus 2 years of AED therapy. Combination of persistent/
calcified CT lesion and abnormal EEG prior to AED withdrawal
was the best predictor of seizure recurrence.
FP-G-084
Bacteraemia during the purulent meningitis
K. Karovski, Z. Milenkovic, N. Pop-Jordanova, A.
Bogdanovska
Clinic of infectious diseases, Faculty of medicine, Skopje,
Macedonia
In an open, randomized, controlled study of 304 patients
with PM treated at Clinic of infectious diseases in Skopje,
the frequency and the influence of bacteraemia on the
outcome of the disease, were investigated. The etiology of
the disease was confirmed by bacterial isolation from the
CSF in 55.6%, with S. pneumoniae (58%), N. meningitidis
(20.7%) and H. influenzae (7.7%), being the most frequent
agents. Fatal outcome in 12.2% of patients, and audiological
sequelae in 8.7% out of survivors occurred. In 17.8% of
patients, the etiological agent was isolated from the blood,
as well; namely, in 43.2% of patients with fatal outcome,
and in 14.2% of survivors. Clinical and laboratory-biochemical
features for sepsis and septic shock were recognized in
34.5% of patients; 70.3% out of patients with fatal outcome,
and in 29.6% of survivors. Out of the laboratory-biochemical
parameters, the increased levels of: ESR, WBC and
granulocytosis in 72.4, 57.2 and 44.7% of patients; as well
as, the decreased levels of WBC and neutropenia in 16.1 and
9.2%, respectively, were found. Increased levels of bilirubinaemia
and enzim activity of aminotranspherases were
registered in 24.3 and 20.6% of survivors, that is in 48.6
and 45.9% of patients with fatal outcome. It was concluded
that sepsis and septic shock lead to dramatic worseness of
the outcome in patients with PM.
FP-G-085
Neurological and psychological aspects of
encephalopathy due to tick born infections of the CNS
K. Gustaw a , K. Bel⁄towska b , M.M. Studzińska c
a Outpatients Neurological Department, Institute of Agricultural
Medicine, Lublin, Poland; b Department of Public
Health, Institute of Agricultural Medicine, Lublin, Poland;

492
Abstracts
c Department of Medical Sociology, Institute of Sociology,
UMCS, Lublin, Poland
The purpose of this study was to delineate the differences
distant neurological and neuropsychological effects of tickborne
encephalitis in children. The group of 11 patients
(four boys and seven girls in the age from 11–15 years)
was included to the study. Every patient had suffered from
encephalitis due to viral infection after bit of the tick and
was properly treated. Medical history, physical examination
and a series of neuropsychological experimental tests based
on WAIS-R, BDI, BENTON-BENDER, DUM were
performed. In the clinical history 27.3% of patients
complained of headaches, 18.1% of the patients of subjective
memory distortion. The neurological examination
showed that. 36.4% of the patients experienced weakness
of the muscles and irritability. Moreover in 72% of the cases
dysmethria, 45% nystagmus and in 54% positive Romberg
sign were found. A total of 45.4% of the patients showed
asymmetry in deep tendon reflexes (DTR’s) and in 45% of
the cases slight muscular strength abnormalities. Besides, in
18% of the cases disturbances in the sensory responses were
noticed. In 18% abnormalities in the function of the muscles
innervated by cranial nerves were examined. Psychological
tests indicated that 72% of the patients had problems in
thinking process and experienced memory impairment
45% of patients had significant organic damage in the
central nervous system. The results of this study suggested
the existence of long lasting consequences of tick born encephalitis,
which can significantly influence the quality of life
of the young patients.
FP-G-086
Conservatively treated multiple brain abscess
Z. Liptai, Z. Meszner, K. Kalocsai, P. Barsi
Pediatric Neurology, Saint Laszlo Hospital, Budapest,
Hungary
Authors report the case of a 17-year-old girl in whom
severe headache and fever developed 8 days after tonsillectomy.
Two days later slurred speech, adynamia and somnolence
occurred. Cranial CT and MRI revealed a 1.2 cm-large
mass in the right parieto-occipital region, with marked ringshaped
contrast-enhancement and wide perifocal edema. An
enhancement of the ipsi- and contralateral ventricular wall
and meninges could be visualized, too. Routine CSF findings
corresponded to massive purulent meningitis. The size
and localization of the abscess did not allow neurosurgical
approach. According to the susceptibility of the cultured
microaerophilic Streptococcus, meropenem was administered
and symptomatic treatment was added. This resulted
in a rapid clinical improvement. Neuroimaging studies 4
weeks later, however, showed marked progression of the
abscess with spreading both inside the right and toward
the contralateral, left hemisphere. Radiological improvement
started on oral cloramphenicol treatment, but severe
adverse effects (depression, anorexia, malnutrition, dehydration,
bone-marrow hypoplasia) made rehospitalization
and a change in antibiotic therapy (reinstitution of meropenem)
necessary, as well as the administration of antidepressant
medication. The patient has become free of complaints
and MR images have shown a slow but unequivocal
improvement. The CSF has been near-normalized. No
relapse occurred after discontinuation of the 3.5-month lasting
antibiotic therapy. Authors will discuss diagnostic and
therapeutic problems related to this case and run through the
scarce experience on similar cases in the literature.
FP-G-087
Postencephalitic parkinsonism in a 7-year-old boy
K.Y. Chae, J.H. Lee
Department of Pediatrics, College of Medicine, Pochon
CHA University, Sungnam, Korea
We experienced a 7-year-old boy who developed an
akinetic-rigid Parkinson syndrome following encephalitis.
On admission, he had convulsive status epilepticus with
prolonged postictal confusion state. EEG showed diffuse
high amplitude of slow wave discharges on the background
consistent with encephalopathy. The initial brain MRI
revealed focal high signal intensities on right hippocampal
area. Viral markers in CSF were all negative. On the fifth
hospital day, he developed lead pipe rigidity in all four
extremities but improved cognition. He progressed to bradykinesia,
blepharospasm, slow tongue movement with dysarthria.
Subsequent brain MRI showed no different findings
with the previous one. Methylprednisolone pulse therapy,
amantidine and levodopa were introduced, and thereafter
symptoms of parkinsonism (hypokinesia, blepharospasm,
dysarthria, and lead pipe rigidity) in all four extremities
partially improved. We suggest that steroid and oral amantidine
with dopaminergic therapy will be worthy in the
management of acute stage of postencephalitic parkinsonism.
FP-G-088
Clinical feature of 25 children with viral encephalitis
B.-Z. Jia
General Hospital of Yangquan Coalmine Group Yangquan,
Shanxi Province, China
Objective: To explore clinical feature of pediatric viral
encephalitis for earlier diagnosis. Methods: The complete
data of 25 children with viral encephalitis in the last 2
years were analyzed. Results: The rate of common symptoms
was: fever (96%), convulsion (64%), vomiting (52%),
disturbance of consciousness (44%), and headache (36%).
The rate of a few symptoms was: thrill (16%), paralysis
(12%), supranuclear paralysis (8%), dizziness (8%), and

Abstracts 493
salivation (4%). Before admission to the hospital, the
median of common symptoms was: headache (2.25), vomiting
(2.1), fever (1.96), convulsion (1.57), disturbance of
consciousness (1). The rate of positive pathologic sign
was: Babinski’s sign (28%), tonic neck (20%), Kernig’s
sign (4%). Conclusion: In clinical work, special care should
be taken to common symptoms of viral encephalitis and
high vigilance be made to a few symptoms. In case of headache,
vomiting and/or no fever, to the sick children perhaps
the viral encephalitis is possible.
FP-G-089
A study on nitric oxide tumor necrosis factor and
interleukin-8 in serum and cerebrospinal fluid in
children with infection of the central nervous system
H.-Y. Wang, Y. Yuan, B.-L Sun
Department of Pediatrics, the First Affiliated Hospital of
Jiamusi University, Jiamusi, China
In this study we evaluated the levels of serum and CSF
NO, NOS, TNF and IL-8. The NO, NOS, TNF, IL-8 in 18
cases of viral meningitis (VM), 14 cases of tuberculous
meningitis (TM), 16 cases of purulent meningitis (PM)
and 20 cases of control group (CG) were determined by
colorimetric methods and redioimmunoassay. The results
showed that: (1) the levels of NO, NOS, TNF in serum
and CSF with VM, TM and PM were significantly higher
than those in CG (P , 0:01). Moreover the concentrations
of NO, NOS, TNF in patients with PM were significantly
higher in CSF than those in patients with VM and TM
(P , 0:01). (2) The levels of IL-8 in serum and CSF with
TM and PM were significantly higher than those in CG. The
concentration of IL-8 with PM was significantly higher than
those with TM. There was no obvious difference between
VM and CG (P , 0:01). These results suggested that: (1)
NO, NOS, TNF, IL-8 all participated in the pathogenesis of
the central nervous system infection. (2) Determination of
NO, TNF and IL-8 in serum and CSF can be a discriminating
index among VM, TM and PM.
FP-G-090
Clinical analysis of 23 children with cryptococcal
meningitis
L.-Z. Zhang
Department of Pediatrics, Heze Municipal Hospital, Heze,
Shandong, China
The mean age of 23 children with cryptococcal meningitis
was 4.3; 16 of them were misdiagnosis. The rate of misdiagnosis
was 69.6%; 30.4% was diagnosed with cryptococcal
meningitis at first. Symptoms included fever, headache,
vomiting, visual loss, convulsion and hepatosplenomegaly.
Remarkable changes in the CSF were observed in tuberculous
meningitis. The levels of CSF glucose and chloride
decreased. Cryptococcus neoformans can be found by an
ink stain technique. Low density lesions were found by
head CT. It is suggested that misdiagnosis is prevented by
detecting the pathogenic agent. The prognosis is related to
the intracranial pressure, stage of misdiagnosis and severity
of inflammation.
FP-H-001
Benign infantile seizures
FP-H
Seizure Disorders
H.-Y. Li, S.-J. Ling, Y.-L. Cui
Xi’an Children’s Hospital, Xi’an, China
Objective: To determine the characteristics of benign
infantile seizures. Methods: A prospective study of 13
cases and a retrospective study of 44 cases of benign infantile
seizures were carried out in 1989–2001. Results: Among
the 57 cases of benign infantile seizures that had a normal
development, there were 35 males and 22 females. The age
of onset was from 50 days to 2.5 years. The clinical manifestations
included tonic-clonic seizure (44), motion arrest,
staring with or without smile or lip cyanosis (10), and partial
seizure (3). Fifty-three patients had the pretreatment
seizures frequency of two to 17 times, four had .40 times
and 28 had seizures occurred in clusters. A family history of
epilepsy or Fc was present in three. Interictal EEGs showed
general epileptiform discharge in eight, and focal epileptiform
discharges in five, and the remaining were normal.
Cranial CT scans performed in 44 were normal. Phenobarbital
(PB), carbamazepine (CBZ) and valproate (VPA) were
effective in 25, 18 and eight cases respectively. Five patients
received a drug combination of PB with CBZ or VPA and
one took no AEDs at all. No seizure occurred after adding
AEDs in 37 and seizures recurred because of early withdrawal
of AEDs or insufficient dosage in 20. All patients
were controlled between the age of 8 months and 5.5 years
and remained seizure-free for 8 months to 12 years (.4
years in 23). AEDs were discontinued after seizure-free
period of 1–5 years in 31, among them 14 were AEDsfree
for 3–10 years. Conclusion: The benign infantile
seizures are not uncommon and can be easily controlled
with PB or CBZ or VPA with favorable prognosis.
FP-H-002
P 300 test of benign focal epilepsy with centrotemporal
spike in children
Z.-D. Lin, H.-L. Wang, X.-Y. Ye, G.-Q. Li, H.-W. Hu
Department of Neuropediatrics, Yuying Children’s Hospital
Affiliated to the Wenzhou Medical College, Wenzhou,
Zhejiang, China
Objective:P 300 test was done in benign focal epilepsy with

494
Abstracts
centrotemporal spike to evaluate its cognition function.
Methods: 22 benign focal epileptic patients who had never
been treated with anti-epileptic drugs were studied and
compared with 25 healthy controls. Auditory event related
potential P 300 peak latency and amplitude were regarded as
the indicator. Results: No significant differences of P 300 peak
latency and amplitude were found in epileptic patients from
those in controls (P . 0:05), and no significant differences of
P 300 peak latency and amplitude were found in the subjects of
different age or gender (P . 0:05). Conclusion: It is
suggested that there is no severe cognition function abnormality
in benign focal epilepsy with centrotemporal spike in
children, especially in those without anti-epileptic therapy.
FP-H-003
Diagnostic value of video-EEG in children with
paroxysmal disorders
Z.-D. Lin, H.-W. Hu, G.-Q. Li, X.-Y. Ye, Y.-L. Zhang
The Department of Pediatric Neurology, The Yuying Children’s
Hospital affiliated to the Wenzhou Medical College,
Wenzhou, Zhejiang, China
To evaluate the diagnostic value of video-EEG in children
with non-epileptic seizure, 52 children suspected to
have or weeded out the possibility of epilepsy were examined
with video-EEG. Fifty-two cases were monitored to
have ‘seizure’ attacks, 35 of whom were diagnosed as
non-epileptic seizure including ten children with nocturnal
muscle clonic, six sleeping disorders, five habitual tics, two
hysteria, four non-epileptic paroxysmal tonic, four behavior
abnormality, two headache or stomachache attack, one
pertussoid attack and one infantile Moro reflex respectively.
While 17 of them were diagnosed as epileptic seizure,
consisting of four with generalized tonic-clonic seizures,
four tonic seizures, three clonic seizures, two localized
motor seizures and other three complex partial seizures,
myoclonic seizures and infantile spasms respectively. The
results indicated the video-EEG is not only the most sensitive
way to differentiate the seizure characteristics and
patterns but also the most effective way in differentiation
of epileptic seizures from the non-epileptic seizures, and is
of value in the classification of the epilepsy and epileptic
syndromes. But the major disadvantage is that in the period
of monitoring with the video-EEG, the children’s daily
activities must be restricted in some area, and is the best
choice in the children with frequent seizures.
FP-H-004
Hyponatremia is a predictor for recurrence of febrile
seizure
I. Tan
University of Santo Tomas, Manila, Philippines
FS even when recurrent have relatively no adverse
effects. However, witnessing a FS is a very scary event.
Predicting and treating FS recurrences will tremendously
allay family anxiety. Risk factors for a first FS and recurrent
FS in future febrile illnesses have been identified.
However, there is scanty information regarding risk factors
for FS recurrence in the same febrile illness. Thus, the
identification of possible risk factors for FS recurrence
within the same febrile illness is important. Low serum
sodium following a FS was found to be a risk factor for a
FS recurrence within the same febrile illness. To affirm or
negate this, 77 children with febrile seizures were prospectively
studied. Serum sodium determination was
performed on all of them upon presentation in the emergency
room. Forty-one of the children (53.25%) had low
serum sodium levels (,135 mmol/l, with a mean of
131.07 mmol/l). The mean serum sodium for the normonatremic
children was 137.58 mmol/l. Twenty-five children
(32.46%) experienced febrile seizure recurrence in
the same febrile illness. Twenty of these children with
FS recurrence were hyponatremic (mean serum sodium
was 129.75 mmol/l) as compared to only five from the
group with normal serum sodium level (P value ,0.01,
chi-squared test). Relative risk for febrile seizure recurrence
in the same illness was 3.5. Our study showed an
increased risk for febrile seizure recurrence in the same
febrile illness in children with hyponatremia, which was
statistically significant.
FP-H-005
The relationship between benign childhood epilepsy with
centrotemporal spikes and febrile convulsions
L. Yin, L.-H. Cao, J.-H. Wu, B.-D. Pang, Y. Zhang
Department of pediatrics, Maternity and Children Hospital,
Tangshan, Hebei, China
Objective: To explore the relation between benign childhood
epilepsy with centrotemporal spikes (BCECT) and
febrile convulsions. To study the possible etiology of
BCECT. Methods: Twenty-six patients, age 1–6 years
old, with typical BCECTs were investigated about the
family history of epilepsy and febrile convulsions. They
were examined with MRI or CT. A control group consisted
of age-matched randomly selected healthy children.
Results: The CT or MRI of the 26 patients was negative.
No hippocampal abnormality was found in the patients.
Only one patient had a history of febrile convulsions. Positive
history of febrile convulsions in the study group is
higher than that in the control group (0:025 , P , 0:05).
There is no difference in the positive epilepsy family
histories between the two groups (P . 0:05). Conclusion:
BCECTs and febrile convulsions are linked closely,
though BCECTs do not certainly derive from febrile
convulsions. Both disorders may have a related genetic
background.

Abstracts 495
FP-H-006
Lateralizing value of ictal version and limbs posturing in
partial seizure in children
L. Yin, K.-X. Luo, J.-H. Wu
Department of pediatrics, Maternity and Children Hospital,
Tangshan, Hebei, China
Objective: To evaluate the lateralizing value of ictal
dystonic posturing of limbs and version of mouth, head
and eyes in partial seizure in children. Methods: By questioning
the parents who have seen the seizure and review the
data of Video-EEG in 48 patients. Results: In the patients
with partial seizure, version of mouth and eyes occurred in
45.83 and 54.17%, respectively and were generally contralateral
to the ictal focus. The version of head were of no
lateralizing significance, dystonic posturing of limbs were
contralateral to the seizure focus (r ¼ 20:908). Conclusion:
The dystonic posturing and version of mouth and eyes have
lateralizing value in partial seizure.
FP-H-007
The value of video-EEG evaluation in clinical diagnosis
L.-H. Cao, J.-H. Wu, B.-D. Pang, Y. Liu, Y. Zhang
Department of pediatrics, Maternity and Children Hospital,
Tangshan, Hebei, China
Objectives: To evaluate the value of video-EEG in the
differential diagnosis of patients with epilepsy or nonepileptic
paroxysmal disorders. Methods: A total of 726
consecutive cases were investigated by VEEG during the
period from August, 1999 to December, 2001, the EEG
patterns and clinical seizure activities were reviewed by
pediatric neurologists. Results: (1) Among the 726 cases,
VEEG patterns were abnormal in 542 cases (74.66%), of
whom, 425 cases had epileptic discharges (78.41% of
abnormal findings) and non-specific changes in 117 cases
(16.12% of abnormal findings). Of these 726 cases, there
were 505 cases that had had previous routine EEG evaluation,
of whom 91 cases (18.02%) had non-specific abnormalities,
and 42 cases (8.31%) had epileptic discharges. There
were significant differences between the two methods. (2) In
the 425 cases with epileptic discharges, the foci were in
frontal in 12, central in 27, frontal-temporal in 50, parietal
in 24, occipital in 15, centro-temporal in 150, diffused
changes in 85, hypsarrhythmia in 20 cases. The epileptic
discharges were only positive during sleep in 238 cases,
during alert state in 52 cases and during both states in 135
cases. (3) After the VEEG evaluation, the paroxysmal disorders
in 187 cases were diagnosed as non-epileptic and in 493
cases were epileptic. Of the latter group, 150 cases had
BECTs, 143 generalized tonic-clonic seizure, 34 complexpartial
seizures, 44 secondarily developed generalized
seizures from partial epileptic activity, 36 myoclonic
epilepsy, 23 infantile spasms, nine Lennox-Gastaut
syndrome, 12 absences, and 15 atonic attacks. (4) The
epileptic discharges and clinical seizures in 227 cases had
been shown simultaneously in VEEG, but only in two cases
during routine EEG investigation. Conclusions: The video-
EEG investigation of patients with clinical paroxysmal
disorders is much more conclusive than routine EEG.
FP-H-008
Diazepam and aspirin-dl-lysine therapy in febrile
seizures: report of 108 cases
W.-Y. Wang, B.-B. Wu, M.-L. Liu
Department of Pediatrics, Affiliated hospital of Medical
college of Chinese People’s Armed Police Forces, Tianjin,
China
Objective: To explore the best method of treating child
with febrile seizures in a clinic work. Methods: Children
with febrile seizures had their body placed properly, their
respiratory tract kept unobstructed, oxygen inspiration therapy
and suction, vein transfusion pathway established
rapidly, and IV diazepam and IV aspirin-dl-lysine infusion.
After control of seizures, we analyzed their possible etiologies
again. IV diazepam and aspirin-dl-lysine can control
seizures rapidly and provides the best effects.
FP-H-009
New progress of drug treating epilepsy
J. Peng
Beijing Military region Department of Pediatrics General
hospital, Beijing, China
Epilepsy is a common and high morbidity disease of
pediatric neural system. After many years of research, functional
mechanism of drugs treating epilepsy has made
progress. This article expounds two problems: (1) when
valproic acid is combined with carbamazepine, the blood
concentration of valproic acid decreases and the dosage of
the latter drug should be increased. (2) If valproic acid,
phenytoin sodium, ethosuximide and clonazepam are
combined with carbamazepine, their blood levels will
decrease. This report also introduces how to choose new
drugs treating epilepsy and how to treat refractory epilepsy.
FP-H-010
Change of serum prolactin levels following children
seizures
Q.-Y. Guo, S.-G. Ming
JiangMen Central Hospital, JiangMen, GuangDong, China
We studied serum prolactin levels following seizures in
49 children with epilepsy; 24 with pseudoseizures; ten
normal children. The epilepsy group showed significantly
increased serum prolactin concentration than the normal

496
Abstracts
group, when the plasma sample was obtained within 60 min
of an attack; but the pseudoseizures group was not significantly
different from the normal group. Different type of
seizures has different effect on serum prolactin; serum
prolactin concentration increased significantly after generalized
tonic – clonic and complex partial seizures, while
simple partial seizures, myoclonic seizures and absence
seizures were not associated with increased serum prolactin
concentration. Our findings showed that prolactin is useful
in differencing epileptic seizures from pseudoseizures.
FP-H-011
Ictal video-EEG and clinical characteristics of infantile
spasms
X. Zhao, F. Wang
Department of Pediatrics, Tangdu Hospital, Fourth Military
Medical University, Xi’an, China
Objective: To analyze the clinical and video-EEG characteristics
of infantile spasms. Methods: 18 children with
infantile spasms were monitored by video-EEG. The EEG
data and behavior recordings were analyzed simultaneously.
Results: (1) The seizure types of infantile spasms included
flexor, extensor and mixed. The spasm seizures occurred in
scatter or cluster that coincided with sleep and wakefulness
onset. On average, there were 16 seizures in one cluster of
seizures. The seizure lasted 1–3 s and the interval of seizures
was 5–20 s. (2) Infantile spasms can occur with partial and
other types seizures. (3) The interictal EEG of infantile
spasms had hypsarrythmia and the ictal EEG had slow
spike-and-wave complexes or suppression-burst. Conclusion:
Infantile spasms have unique type of seizures and
can appear as partial or other type seizures. The frequency
of seizures is high. It is easily found by video-EEG. The
ictal and interictal EEG of infantile have unique manifestations.
FP-H-012
Coincidence of Rolandic and absence features: rare, but
not impossible
P.S. Dimova, D.S. Daskalov
Clinic of Child Neurology, University Hospital of Neurology
and Psychiatry ‘St. Naum’, Sofia, Bulgaria
This study’s purpose is to evaluate the incidence of
concomitant clinical and EEG absence and rolandic
discharges. The 1997–2000 medical files and EEG records
of all children with Rolandic epilepsy, childhood, and juvenile
absence epilepsy were retrospectively analyzed. Out of
the 66 patients with Rolandic epilepsy, six children showed
during the antiepileptic treatment clinical and/or EEG
features of absences. Most of them were treated initially
with carbamazepine and had generalized spike-wave
discharges of a secondarily generalized type. Five cases
from the group of 34 children with childhood absence
epilepsy and three cases from the group of 11 patients
with juvenile absence epilepsy were identified with an
EEG focus of rolandic type in the first or in subsequent
EEGs. None of the patients with absences experienced
rolandic seizures during the disease course. All atypical
EEG phenomena were registered while awake, thus precluding
the possibility of sleep activation. The likely relation of
absence features in Rolandic epilepsy to the treatment is
considered as well as the probability of their manifestation
as a sign of atypical, complicated course of Rolandic
epilepsy. On the other hand, the presence of a Rolandic
focus in the course of typical childhood or juvenile absence
epilepsy makes the coincidence of these entirely distinct
phenomena, even if very rare, not exceptional, and suggests
that a genetic link between partial and generalized EEG
discharges could exist. Further studies are required to elucidate
the mechanisms and carriers of inheritance and liability
to the most childhood epilepsies.
FP-H-013
Optimal treatment of Rolandic epilepsy: priority of
valproate over carbamazepine
P.S. Dimova, D.S. Daskalov
Clinic of Child Neurology, University Hospital of Neurology
and Psychiatry ‘St. Naum’, Sofia 1113, Bulgaria
It was the aim of this study to evaluate the treatment effects
of the anticonvulsants most often used in BECTS. The medical
charts and EEG records of 66 children with the diagnosis
of BECTS were retrospectively analyzed. Out of these
patients, 48 children were treated initially with CBZ, and
17 – with VPA. In general, CBZ and VPA had similar efficacy
in seizure control and reduction, but differed in terms of
lacking efficacy and seizure aggravation. Main differences
between these drugs were found in their effects on the EEG
abnormality. The epileptic potentials disappeared or
improved significantly more often under VPA, and were
unchanged or activated significantly more often with CBZ.
This EEG-response reflected on the better effects of VPA on
the neuropsychological disturbances in some patients. These
results lead us to suggest that VPA could be a drug of first
choice in BECTS.
FP-H-014
Self-control study of depakine for the treatment of
childhood epilepsy
X.-H. Shao
Department of Pediatrics Neurology, Xinhua Hospital
Affiliated to Shanghai Second Medical University, Shanghai,
China
Objective: To verify the curative effect and safety of depakine
for the treatment of childhood epilepsy. Methods: An

Abstracts 497
open-label, add-on, self-control study was undertaken in 26
children with epilepsy. The seizure frequencies in 6 months
before and after therapy were compared in these epileptic
patients. The patients were treated with depakine at the
dose of 15–30 mg/kg per day, and the seizure-rate and
side-effects were noted. Results: The seizure frequency
decreased significantly ($50%) in 24/26 (92.3%) patients
after therapy, depakine was effective for all type of seizures,
in particular for generalized tonic-clonic convulsions.
Patients could tolerate depakine well. Most adverse events
including vomiting and dizziness were mild or moderate in
severity, and occurred occasionally (4/26, 15.4%). Conclusion:
Depakine is an effective, broad-spectrum and had a
favorable safety for the treatment of childhood epilepsy.
FP-H-015
Diagnostic analysis of recurrent seizures in 86 children
beginning with febrile convulsions
J.-X. Liao, L. Chen, Y.-H. Xiao, B. Li, T.-S. Huang, Z. Wei
Epilepsy Center, Shenzhen Children’ s Hospital, Shenzhen,
China
Background and objectives: Childhood onset of multiple
febrile convulsions continued with fever beyond 6 years, or a
febrile seizures occurred. It was not mentioned in the international
classification of epilepsy syndromes about how
these seizures are diagnosed. Based on research of a large
multigenerational family, Scheffer IE and Berkovic SF advocated
a new epilepsy syndrome, the GEFS 1 . The commonest
phenotype is denoted as FS 1 . We analyzed the diagnosis of
recurrent seizures beginning with febrile convulsions in children.
Methods: We investigated children attending the pediatric
neurology service of the out-patient department from
1994 to 2001 in whom detailed clinical records were available.
Results: There were 86 individuals that comprised
about 5.0% of seizure patients of our out-patient department.
Two individuals were diagnosed as having febrile seizures,
64 (74.4%) FS 1 , eight FS 1 and absences, five FS 1 and
myoclonic seizures, three FS 1 and myoclonic-astatic
epilepsy, two FS 1 and temporal lobe epilepsy and two FS 1
as suspected and temporal lobe epilepsy. Conclusions: There
are a few GEFS 1 individuals among small family or sporadic
patients. The recognition of GEFS 1 is helpful for the treatment
of these patients.
FP-H-016
The clinical use of digital ambulatory
electroencephalography
Y.-H. Xiao, J.-X. Liao, Y. Chen, W.-Y. Chen, P. Xia
Epilepsy Center and Department of Pediatric Neurology,
Shenzhen Institute of Pediatrics and Shenzhen Children’s
Hospital, Guangdong, Shenzhen, China
Objectives: To investigate the characteristics of digital
ambulatory electroencephalography (AEEG) and the efficacy
of AEEG in the diagnosis and differential diagnosis
of paroxysmal diseases in children. Methods: With 16-channel
digital AEEG, 88 patients whose diagnosis had not yet
been determined, but clinical features made them suspected
of having epilepsy, although routine electroencephalograms
had been tested, were examined for 24 h. The AEEG monitoring
also included activating tests such as opening- closing
eyes and hyperventilation, etc. The positive results meant
that epileptiform discharges and/or abnormal background
activity appeared. Results: Digital AEEG was very convenient
with its multiple channels up to 16, enabling to change
any montage as needed, and adopt wave amplitude and
scanning velocity, and the quality were as high as that of
routine EEG. Of 88 patients the positive rate was 67% (59/
88), and 44 were diagnosed with epilepsy definitely, their
epleptiform discharges was recognized and well localized,
their epilepsy type was classified. Two patients had no
epileptic seizures but had typical epileptiform discharges.
Referred to clinical manifestations, the diagnosis of epilepsy
was excluded in 19 patients. Conclusion: Digital AEEG
might obtain much more information than traditional
magnetic tape analog AEEG, and the focus might be determined
more precisely, and it is a great progress of electroencephalography.
FP-H-017
Continuous intravenous infusion of midazolam for
treatment of intractable seizures in children
J.-M. Zhong, J.-H. Li, M.-Z. Zhou
Jiangxi Children’s Hospitol, Nanchang, Jiangxi, China
Objective: To determine the efficacy and safety of midazolam
given as a continuous infusion in the treatment of
prolonged seizures and a cluster of seizures in children.
Methods: Thirty-two patients admitted to the neurological
ward in our hospital met the criteria of status epilepticus or a
cluster of seizures with duration of more than 30 min and the
seizures were unresponsive to diazepam, clonazepam,
phenobarbital, and valproic acid. They were 24 boys and
eight girls. Mean age was 2.5 ^ 2.9 years (ranging 37 days–
12 years), and 46.9% (15/32) of cases were under 1 years of
age. The type of seizure was generalized in 25 cases, partial
in seven, myoclonus in two, respectively. Etiologically, 13
children had primary epilepsy, 12 central nervous system
infection, one intracranial hemorrhage, one drowning, and
the remaining five had toxic encephalopathy. All patients
received intravenous midazolam at 0.10–0.26 mg/kg as
bolus, followed by a continuous infusion at 0.10 mg/kg
per h initially; the dose was increased gradually up to 0.35
mg/kg per h or until the complete control of seizures was
achieved. Time to control seizures, infusion rate and sideeffects
were monitored. Results: Among 32 children, 21
cases were brought under complete control, and there was
seizure improvement (more than 50% reduction of seizures)

498
Abstracts
in seven. The remaining four showed no responses. Mean
time to control seizures was 10.1 ^ 14.9 h (ranging 1 min–
43 h) and seizures stopped with 5 min in 47.6% of cases.
Mean infusion rate was 0.18 ^ 0.09 mg/kg per h (ranging
0.10–0.35 mg/kg per h). Mean duration of infusion was
62.0 ^ 38.3 h (ranging 14–152 h). None of the children
had clinically important changes in blood pressure, heart
rate, respiratory status and retention of urine attributable
to the use of midazolam. Conclusions: Midazolam infusion
is an effective and safe therapeutic approach to control
intractable seizures.
FP-H-018
The use of goggles flash visual evoked potential in
disorders of children’s central nervous system (CNS)
S.-H. Li, Y. Chen, Z.-X. Qiao
Harbin Children’s Hospital, Harbin, China
Purpose: To obtain the normal value in group of children
and the abnormal value in group of children having
impaired consciousness in different degree by goggles flash
visual evoked potential (GO-VEP). Methods: To stimulate
the eyes by goggles flash using the drawing instrument
(SEEG) of 16 channels colors EEG and evoked potential
made by DanDe company in Denmark. And the potentials
were recorded with silver plate electrode in Oz point,
stimulating both eyes. Each eye must be examined twice,
which will give better reappearance. Conclusion: GO-VEP
can not be affected by the power and sensitivity of sight,
and can be used to detect baby, infant and child with
impaired consciousness who have disorders of CNS. It is
a new way to find the visual nervous damage and can be
repeated. At the same time, it is foundation to detect the
early damage of visual nerves. It can be an assistant
method for pattern reversal visual evoked potential (PR-
VEP).
FP-H-019
Analysis of brain electrical activity mapping of 100
children with generalised epilepsy
S.-H. Liu, X.-Q. Wu
Shenyang Medical and Health Care Center of Children and
Women, Shenyang, China
Epilepsy is a common emergency in clinical pediatrics;
the diagnosis depends upon the seizures and electroencephalogram.
Brain electrical activity mapping (BEAM) is a
new electrophysiological technique that is recently widely
used clinically, but its value in the diagnosis of childhood
epilepsy is rarely reported. The analysis was done in the
BEAM results of 100 cases of childhood epilepsy with a
confirmed diagnosis of generalised epilepsy. It was found
that 91 cases had abnormal BEAM (91%), and the abnormal
ratio increased with the age, there was 30% local
BEAM abnormality in generalised epilepsy. It was also
found that the local BEAM abnormality is coincident
with the part of brain CT abnormality. BEAM can not
display the epileptic waves such as sharp wave, spike
wave, spike and slow waves and so on, but it can show
different brain electrical activity signals which depend on
different frequency of power pedigrees in corresponding
simulated EEG, and make the brain electrical activities
more quantitative, objective, imaginable, and directive. It
is helpful in making diagnosis and observing the remedy
effect of epileptic children.
FP-H-020
Convulsive disorders in PICU: an etiological analysis of
182 cases
S.-H. Liu, Y. Li
Shen Yang Childrens’ Hospital, Liaoning, China
Objective: To summarize and analyze the commonly
encountered reasons of convulsions in pediatric intensive
care unit (PICU). Methods: According to the age, the 182
children from 1 month to 14 years old were divided into four
groups, then according to the sex subdivided into eight
groups. Results: The percentages of different reasons in
these groups were reported. Febrile convulsions, vitamin
Ddeficient hypocalcemic seizures, epilepsies, and central
nervous system infections are most commonly encountered
etiological factors in this study, whereas hereditary metabolic
disorders may constitute one of the relatively rare
causes.
FP-H-021
Applying topiramate to treatment of infantile epilepsy
Y.-Q. Zhang, J. Zhu, D. Li, P.-Y. Zhang, X.-L. Yu
Department of Neurology, Tianjin Children Hospital, Tianjin,
China
Objective: To evaluate the efficacy and tolerance of topiramate
as monotherapy and adjunctive therapy in infantile
epilepsy. Method: Forty-five patients were included, aged
15 days to 2 years, median 9 months (male 27, female 18),
weighed 2.8–15 Kg (median 9.5 Kg). Age distribution: #3
months 11, 26 months 13, 21 year 12, 22 years nine. The
course ranged from 4 h to 21 months. Secondary epilepsy
38, and cryptogenic epilepsy seven. Twenty-five of 45
received monotherapy and 20 adjunctive therapies. Dosages
of topiramate were adjusted to optimal clinical response.
Results: The frequency of all seizures was reduced $50%
from baseline in 91% of 45 patients, and the rate of seizurefreedom
was 37.8%, when the mean duration of topiramate
therapy was 3 months. The frequency of all seizures was
reduced $50% from baseline in 91% of 34 patients, and the
rate of seizure-freedom was 52.9%, when the mean duration
of therapy was 3 months. The frequency of all seizures was

Abstracts 499
reduced $50% from baseline 100% of 25 patients with
monotherapy and 80% of 20 patients with adjunctive therapy.
The incidence of adverse effects was 20%. Adverse
effects of topiramate were mild. Hypohidrosis was notable.
In 28 patients, blood routine/urine routine and function of
liver and kidney was normal during taking drugs. Conclusion:
The study shows that topiramate is an effective and
well tolerated agent in monotherapy and adjunctive treatment
for infants with epilepsy. Adverse effects were mild.
FP-H-022
Febrile convulsions in children: a clinical analysis of 108
cases
Y.-Z. Zhang, M. Feng
Shanghai Baoshan central Hospital, Shanghai, China
To investigate the characteristics and related factors of
febrile convulsions, 108 cases of young children were
divided into relapse group and non-relapse group to
analyse the high risk recurring factor. They were also estimated
in terms of their convulsion characteristics (including
seizure types, duration time and temperature, etc.) and
white blood cells count (including lymphocyte count and
neutrocyte count), by using of logistic multiple regression.
The result shows that there is a significant difference
between the relapse group and the non-relapse group in
the first attack age, the first occurring temperature, seizure
types and family history. There is no significant correlation
between white blood cells count and the characteristics of
convulsions. The results suggest that white blood cells
count is of no value in diagnosis of febrile convulsions in
children. Febrile convulsions are susceptible to recurrence,
and are closely related with the first occurring age, the first
seizure types and family history. It is necessary to estimate
children with high fever by means of those risk recurrence
factors mentioned above in clinical experience. Take
proper intervention when necessary.
FP-H-023
Magnetoencephalographic study of patients with
continuous spikes and waves during slow sleep (CSWS)
H. Hattori a , A. Yasuhara d , K. Tanaka a , T. Tsutada b ,N.
Tsuyuguchi c , M. Shimogawara e , H. Ishida a , O. Matsuoka a ,
T. Yamano a
Departments of a Pediatrics, b Geriatrics and Neurology, and
c Neurosurgery, Osaka City Univeristy Medical School,
Osaka, Japan; d Department of Pediatrics, Kansai Medical
College Kori Hospital, Neyagawa, Japan; e Applied Electonics
Laboratory, Kanazawa Institute of Technology,
Tokyo Japan
Objective: We analyzed the diffuse spikes and waves in
patients with CSWS using magnetoencephalography
(MEG) in order to know the pathogenesis of CSWS.
Patients: MEGs were measured in seven patients who
were electroclinically diagnosed as CSWS. The patients
were 7–11 years old (one boy and six girls). Methods:
MEGs were measured in a magnetically shielded room,
using the newly developed, whole-cortex MEG that has a
160-channel, first-order gradiometer. Data were filtered
with a band pass of 3–250 Hz and digitized to 1000 Hz.
We estimated the dipoles of the spikes in the simultaneously
recorded EEG. These estimated dipoles were
superimposed on the magnetic resonance images. Results:
We could estimate the dipoles in five patients as mapping
onto the focal cortical area bilaterally, localizing almost
symmetrically. Short time differences were recognized
between sides. These time differences were from 14.2 to
35.3 ms. The impulse will transfer through the corpus
callosum and make miller focus. The dipole was localized
in the Sylvius area in two patients, the parieto-oocipital
area in two patients, and the frontal area in one patient.
We could not estimate the dipoles in the other two patients.
Conclusion: Secondary bilateral synchronization will form
diffuse spikes and waves in majority of CSWS patients,
and MEG can define their focus. However, some patients
with CSWS have primary bilateral synchronization. Pathogenesis
of CSWS was considered heterogeneous.
FP-H-024
The magetoencephalographic findings in infantile
spasms
S. Hanaoka, M. Kawatani, M. Izumi, M. Fukumizu, K.
Sugai
Department of Child Neurology, National Center Hospital
for Mental, Nervous and Muscular Disorder, National
Center of Neurology and Psychiatry, Kodaira, Japan
Infantile spasms is an early onset epilepsy with characteristic
clinical and EEG findings. There are already many
approaches to its electrophysiological mechanism, but it is
yet unclear. The MEG is one of useful instruments to detect
the source of paroxysmal discharges, and there is no report
to analyze them in infantile spasms. We examined the
patients affected to infantile spasms by a whole brain type
MEG in order to approach to its electrophysiological
mechanism from the viewpoint of magnetic field. Subjects.
Method: The subjects are five cases of infantile spasms form
the age of 5 months to 1 year and 4 months, three males and
two females. By using Neuromag 204, we analyzed MEG
findings and the source of paroxysmal discharge in comparing
with simultaneous EEG records. Results: All of five
cases showed multifocal pattern and their dipole sources
were scattered on bilateral hemispheres. By the analysis
about more than 30 points of paroxysmal discharges, the
dipole sources showed a tendency to accumulate in the
parietal and its adjacent lesions. The dipole sources accumulated
around the posterior horns in four of five cases,
which seemed to correspond to watershed areas. Conclu-

500
Abstracts
sions: The results suggest the importance of pathophysiological
changes of the watershed areas as a development of
infantile spasms.
FP-H-025
A comparative clinicoelectrical study on the influences of
influenza infection versus vaccination on the epilepsies in
the severely handicapped patients
Y. Yamatogi, T. Misaki, K. Kawakubo, K. Araki, A. Tsutsui
Okayama Prefectural University, Soja, Okayama, Japan
To clarify the influence of influenza vaccination on
epilepsy is important for the clinical management of the
severely handicapped patients who are vulnerable to infection.
Methods: Three clinicoelectrical investigations were
made on those without change in antiepileptic medication
around vaccination or infection; (1) influences of the initial
vaccination on clinical seizures and epileptic discharges in
28 patients; (2) influences of the repeated vaccination in 24
patients; and (3) influences of influenza infection in 12
patients. Results: (1) The initial vaccination transiently
increased epileptic discharges more than 50% in seven
patients (25.0%), with recovery within about 6 months.
Allergic predisposition and age at vaccination may relate
the worsening. (2) By the repeated vaccination, worsening
in epileptic discharges increased from eight patients
(33.3%) by the first vaccination to 14 patients (58.3%)
and that in clinical seizures from none to seven patients
(29.2%). Fortunately, both adverse effects were reversible.
(3) Influenza infection also transiently worsened epileptic
discharges in six (50.0%) and clinical seizures in two
(16.7%). Conclusion: Influences on epilepsy may be the
same in both influenza infection and the initial vaccination,
but repeated vaccination is suggested to have a risk to accelerate
the worsening of EEG and clinical seizures. Influenza
vaccination should be carefully evaluated to perform in
those with intractable epilepsy, particularly due to severe
brain damage.
FP-H-026
Changes of cerebral hemodynamics in children suffering
from epilepsy
S.A. Goulyaev, A.A. Ovchinnikova, S.E. Goulyaeva, I.V.
Archipenko
Vladivostok Medical State University, Vladivostok, Russia
Changes of cerebral hemodynamics in 410 children
suffering from epilepsy were studied. It is determined, that
a vascular factor is one of the main causes of development
mechanism of epilepsy. During the period of pre-natal
development the lack of blood provision makes worse
genetically determined imbalance of mediator exchange,
then it turns out to become a regulator of degeneration
process development in nerve tissue. Dynamics of clinical
manifestations of epilepsy and its EEG characteristics
reflect fading of epileptic manifestations of cerebral activity
with age. Computer tomography of cerebrum allows to
visualize morphological defects in cerebrum structures in
83.7% cases of epilepsy. Abnormality of anatomic structure
of cerebrum and its atrophy can be observed in equal
percentage. Transcranial dopplerography capacities in diagnosing
changes of cerebral hemodynamics in epilepsy can
reach 86.3%. During the period between the attacks one can
observe the following: changes of vascular tonus of microcirculatory
mouth; different combinations of local changes
of manifestation of cerebral hemodynamics. Among the
local changes we can differentiate three changes reflecting
abnormality of anatomic structure of cerebral vascular
system: alteration turbulent and normal blood flow; combination
of signs of different degree of manifestation of stenosis;
unexpected alterations of findings of peripheral
resistance. All of those mentioned above could be observed
in the adjacent sections of one and the same vessel. In
epilepsy such changes were observed in 22% of cases.
During the period of epilepsy attack one can observe
synchronous registration of generalization of epileptic activity
on EEG and sonic sound of angiospasm reactions on
Monitor-Dopplerograph that arises later 0.3–1.2 s than
generalization of epileptic patterns on EEG, but it remains
even after their disappearance.
FP-H-027
Case report: progressive neuronal degeneration (Alpers-
Huttenlocher disease)-seizure free on Topamax add-on
therapy
V. Mejaški-Bošnjak, K. Fumić, J. Bošnjak, L. Lujic, Z.
Ardašanić, B.M.-D. Marina
Children’s Hospital Zagreb, University Medical School,
Zagreb, Croatia
Progressive cerebral poliodystrophy – Alpers-Huttenlocher
disease (A-H d) is a clinico-pathological syndrome
with no specific biochemical marker. A-H d is usually
presented as an early-onset progressive encephalopathy
with intractable seizures and liver dysfunction. Etiology is
still obscure, though respiratory chain mitochondrial disorders
have been postulated. Valproate treatment of the
seizures may trigger hepatic failure and fatal outcome. We
present the case of a 6-year-old girl who, following a period
of developmental delay and failure to thrive during infancy,
suffered at age of 17 months from myoclonic seizures. After
valproate administration the condition of the child worsened
leading to serial seizures of various type (myoclonic, generalised
clonic, etc.) and mild hepatic dysfunction progressing
into coma. Valproates were immediately withdrawn, and
barbiturate was given intravenously along with support of
vital functions. The girl survived this acute life-threatening
episode, deteriorating in terms of severe mental impairment,
along with autistic traits, and intractable epilepsy of predo-

Abstracts 501
minately partial complex and generalised tonic fits. Paraclinical
investigation: biochemical tests, neurophysiological
and neuroimaging findings, liver and muscle biopsy with
analysis of respiratory chain enzymes supported A-H d.
Further anticonvulsive polytherapy consisted of various
drugs, being modified repeatedly due to inefficiency since
the girl had 10–30 fits/day. At age of 5 years she was put on
Topiramate add-on therapy slowly titrated. Fits were gradually
reduced in number and severity, and at the dosage 8 mg/
kg she became seizure-free for 1 year. There was no adverse
effect of the drug. The mechanism of beneficial effect of
Topiramate in the case of A-H d is discussed.
FP-H-028
Lamotrigine in the treatment of epilepsy in patients with
Rett syndrome
V. Mejaški-Bošnjak, L. Lujić, B.M.-D. Marina, R.
Duplančić, V.D⁄ uranović, T. Gojmerac
Children’s Hospital Zagreb, University Medical School,
Zagreb, Croatia
RS is a progressive neurodevelopmental disorder, occurring
almost exclusively in female patients, clinically characterized
by acquired microcephalia, mental retardation,
autistic behavior, ataxia, loss of purposeful use of the
hands. Epilepsy and/or EEG abnormalities are common
problems in patients with RS. We present ten girls (aged
4–12 years), who met diagnostic criteria of typical RS,
followed-up for 1.5–9 years. At last assessment 6 of them
were classified as III stage and 4 as II stage of RS. Seven out
of these ten RS patients have epilepsy (generalized clonic/
tonic and/or myoclonic fits), while three were seizure free,
showing multifocal EEG abnormalmalities. Epilepsy was in
all RS patients intractable, treated with various antiepiletic
drugs, most commonly using valproates. Lamotrigine was
given as add-on therapy in all seven RS patients having
epilepsy, and as monotherapy in three patients with abnormal
EEG. Effect of Lamotrigine therapy was evaluated after
1–1.5 years. One RS girl became seizure free, while six
other have marked stabilization of epilepsy in terms of
severity and frequency of the seizures. Remaining three
seizure free patients, during period of follow up were still
seizure free, with improved EEG abnormalities in two. We
conclude on beneficial effect of Lamotrigine in the treatment
of epilepsy in patients with RS.
FP-H-029
Ketogenic diet in four Chinese children
W.-W. Cheng, P.W.T. Tse
Caritas Medicalcentre Caritas Medical Centre, Hongkong,
China
The ketogenic diet is a special diet that was first used as a
treatment for epilepsy in 1921 with increased popularity
recently. In our Developmental Disability Unit, 118 children
had epilepsy, of which 30 were refractory. In the past 2.5
years, ketogenic diet was started in four of them. All of
them had been tried on at least four anticonvulsants. There
were two failures in terms of no improvement in seizure
frequency despite attaining deep ketosis during a 4-month
and a 6-month trial period respectively. In the third child,
medium-chain triglyceride ketogenic diet was implemented
at the age of 5 years. There was ,50% improvement in
seizure frequency initially. After changing to classical ketogenic
diet, .90% improvement was attained. The general
well being of the patient improved. He has been on the diet
for 30 months and experienced no complications such as
growth failure, hypercholesterolaemia and renal stone
formation. In the fourth child, ketogenic diet was started at
the age of 18 months. She attained 50% improvement in
seizure frequency. Prior to the diet, she suffered catastrophic
seizures and status epilepticus which disappeared after the
diet. At the age of twenty-three months, a renal stone of 6 mm
was revealed. Balancing the risk of withdrawal from the diet,
ketogenic diet was continued with close monitoring of the
complications. Conclusion: Ketogenic diet is worthwhile
trying especially in those children for whom epilepsy surgery
will unlikely be an option to them.
FP-H-030
The application of Topamax w to children with epilepsy
J.-F. Guo, H. Zhao, X. Wang, J.-C. Guo, Y.-L. Guo, T. Chen
Panjin No. 2 People’s Hospital, Panjin City, Liaoning,
China
We reported the results of using Topamax w , a new antiepileptic
drug, to 30 cases of children’s epilepsy in our hospital
since June, 1999. Out of the 30 cases, 17 cases were male,
13 cases were female, and they were at the age of 2–12,
average 5.6. The courses of disease were 3 months–10
years. During the period of treatment, of five cases with
simple partial seizures, three cases were seizure free, one
case was markedly controlled and one case partially
controlled. The total effectiveness is 100%. Of eight cases
with complex partial seizures, three cases were seizure free,
one case was markedly controlled, two cases partially
controlled, and two cases not controlled. The total effectiveness
is 75%. Of six cases with tonic clonic seizures, two cases
were seizure free, one case markedly controlled, two cases
partially controlled and one case not controlled. The total
effectiveness is 83.3%. Of six cases with myoclonic seizures,
two cases markedly controlled, two cases partially controlled
and two cases not controlled. The total effectiveness is
66.7%. Of five cases with atypical absence seizures, two
cases partially controlled, three cases not controlled. The
total effectiveness is 40%. Totally eight cases with seizure
freedom occupied 26.7%. five cases with markedly
controlled seizures occupied 16.7%. Nine cases with
partially controlled seizures occupied 30%. The seizures

502
Abstracts
were not controlled in eight cases. The total effectiveness was
73.3%. The best results were obtained in the patients with
simple partial seizures, tonic clonic seizures and complex
partial seizures, in this order. It was relatively difficult to
control the patients with myoclonic seizures and atypical
absence seizures. Our results indicated that 50% infant
patients had adverse events in 1–3 weeks after initiating
medicine. This showed that these adverse events occurred
during the period of dose-increasing. As the time went on,
the adverse events will get improved.
FP-H-031
Infantile autonomic epilepsy: clinical analysis of 32 of
patients
J.-F. Guo, H. Zhao, X. Wang, J.-C. Guo, Y.-L. Guo, T. Chen
Panjin No. 2 People’s Hospital, Panjin City, Liaoning,
China
The study makes an analysis of 32 children with autonomic
epilepsy diagnosed in our section from 1995 to 2001. Out of
the 32 cases, 19 are male, and 13 female. They are at the age of
3–12.Therearefivecasesattheageof3–5,19attheageof6–8,
eight at the age of 9–12. The criteria for diagnosing autonomic
epilepsy are as follows: (1) no positive symptoms were
checked out for nervous system in all of these cases to eliminateorganicdiseaseswhichbelongtothedepartmentofinternal
medicine and those which belong to the surgical
department. (2) Repeated seizures. (3) Autonomic symptoms
are dominant. (4) Abnormal EEG. (5) Antiepileptic drugs are
effective in patients whose EEGs are normal. All cases had
EEG examinations. Abnormal results appeared in the EEG of
23 cases (71.9%). Spikes or spiky slow waves appeared in the
EEG of 15 cases, scattering spikes and sharp waves appeared
in the EEG of eight cases, and the other nine cases had normal
EEGs. As for the distribution of paroxysmal discharges, they
appeared in different regions; 13 cases had notable paroxysmal
discharges in the central area, six in the frontal areas, and
four infrontalorone sideof thefrontalareas. Theantiepileptic
drugs (carbamazepine, phenobarbital, valproic acid, etc.)
were used for the therapy in all of these cases. We gave the
drugs to the patients according to the routine dosage or individual
difference. Notable clinical outcome has been
achieved.
FP-H-032
Analysis of electroencephalogram in 415 children with
febrile seizures
Y. Chen, Y.-H. Xiao, J.-X. Liao
Epilepsy Center, ShenZhen Children’s Hospital, Shenzhen,
China
Objective: To analysis the relationship between recording
time after FS and abnormal rate of EEGs; And to imply
abnormal EEG of FS can predict further seizures and the
occurrence of later epilepsy. Method: EEGs were recorded
2 , 3 days after FS in 415 FS children at the age 5
months , 6 years old. Some of them were recorded again
15 , 20 days after FS. Result: There was no relationship
between the abnormal rate of EEG and the FS history of
their relatives, but it was related to clinical features of FS
(local seizure and FC lasting .15 min) and ages. The rate of
abnormal EEGs was 80% 2 , 3 days after FS. It contained
61% of slow activity and indefinite spikes and 19% of spikes
or spike-slow wave. In records taken again after 15 days, the
rate of spikes and spike-slow wave discharges was 17%.
There was no significant relation between EEG at different
times. The rate of further seizures was raised in children
with abnormal EEGs, and some of them (four of them)
developed epilepsy. Conclusion: There may be no relationship
between the abnormal rate of EEG and FS history in
relatives. It was related to clinical features of FS. EEGs
recorded 2 , 3 days after FS was as important as those
after 15 days. Further seizures might be related to abnormal
EEGs and abnormal EEGs may lead to epilepsy.
FP-H-033
The clinical analysis of 158 cases of iatrogenic
intractable epilepsy in children
G.-B. Bian, Y.-P. Qiu
Hospital Attached to Ningxia Medical College, Yinchuan,
Ningxia, China
In order to explore the causes of iatrogenic intractable
epilepsy and its solutions, 158 cases have been classified
and analysed intensively. Among these causes are: improper
diagnosis and classification of epilepsy, lack of aim of drug
choices, abuse of multi-drugs, improper drug dosage, lack of
individuality, the drug usage are not in accordance with pharmacokinetics,
improper drug variety and dosage regulation,
too short courses, too early drug termination, no consideration
of sick children’s general status. Here is to ask pediatrician
clinical doctors to master epilepsy diagnosis and its
classification skilfully and fully understand pharmacological
characteristics of pharmacokinetics and pharmacodynamics
on different anti-epileptic drugs. With the combination of
examination measures such as EEG and serum drug concentration,
doctors are able to give a regular and individual treatment
to each case after the detailed analysis of the interaction
among diseases, sick children’s status and drugs.
FP-H-034
Determination of neuropeptide Y (NPY) in the plasma
and its clinical significance in children with epilepsy
D. Liu a , Q.-Y. Zhang b
a Department of Pediatrics, Central Hospital Affiliated to
Shenyang Medical College, Shenyang, China; b Department
of Pediatrics, The First Clinical College, China Medical
University, Shenyang, China

Abstracts 503
Objective: To observe the change of NPY in the plasma
in children with epilepsy and thereby to evaluate its possible
application in clinical practice. Methods: In the early
morning, 2 ml venous blood was collected from all
enrolled subjects, including 53 children with epilepsy
(43 cases with primary epilepsy, ten cases with symptomatic
epilepsy). Among the children with primary
epilepsy, 34 cases had not been treated, nine cases had
seizures after being treated. Twenty-one healthy children
were enrolled to serve as normal controls. The levels of
plasma NPY were measured by means of radioimmunoassay.
Results: (1) The levels of NPY in children with
epilepsy (210.57 ^ 71.28 pg/ml) were markedly higher
than those of normal control subjects (159.99 ^ 65.10
pg/ml), the difference was significant, P , 0:01. (2) The
levels of NPY in children with primary epilepsy
(210.57 ^ 71.28 pg/ml) compared with that with symptomatic
epilepsy (219.25 ^ 97.14 pg/ml), the difference was
not significant, P . 0:05. (3) The levels of NPY in children
with primary epilepsy who had not been treated
(205.57 ^ 63.89 pg/ml) compared with that with primary
epilepsy who had seizures after being treated
(229.44 ^ 96.61 pg/ml), the difference was not significant,
P . 0:05. Conclusions: The results indicated that the
levels of NPY in children with epilepsy were markedly
increased and the change of NPY did not correlate to
pathogenesis and treatment.
FP-H-035
A study on epileptic children’s intelligence quotients and
affecting factors
Y.-L. Hu
Department of Pediatrics, First Peoples’ Hospital of
Yunnan Province, Kunming, Yunnan, China
Objective: To study the IQ and affecting factors of epileptic
children. Methods: IQ was assessed in 28 children with
epilepsy and 30 normal children. Thirteen possible affecting
factors of the intellectual development were investigated.
Results: IQ of epileptic children was significantly lower
than normal children. With stepwise regression analysis,
the main risk factors were in proper order: age, type of
disease, frequency of disease, course of disease and time
for controlling symptom. Conclusions: Childhood epilepsy
should be diagnosed early and treated correctly, which helps
to reduce the incidence of mental retardation.
FP-H-036
The clinical and EEG analysis of 120 cases of benign
focal epilepsy of childhood with centrotemporal spikes
Y.-W. Zheng a , Q. Zhang b , C.-G. Fong b
a Children’s Hospital Affiliated to Chongqing Medical
University, Chongqing, China; b Kunming Children’s Hospital,
Kunming, China
Objective: To recognize the relationship between the
prognosis of benign focal epilepsy of childhood with centrotemporal
spikes (BFECT) and its clinical seizures as well as
its EEG features. Method: The clinical materials and EEG
features of 120 BFECT cases summarized and analyzed.
Result: Age 6–10 is the high incidence period (70.8%) of
BFECT which has the close relations to sleep, the basic type
of epileptic seizure is general tonic-clonic and partial, its
EEG features are partial spikes or sharp waves, 86.6% of
which are in the central part of centrotemporal region.
Conclusion: The literature indicates that the BFECT occurs
during a particular growing period of childhood, the clinical
prognosis of BFECT and its response to treatment with
antiepileptic medicines are good.
FP-H-037
Clinical and electrophysiologic features of patients with
Cockayne syndrome
H.-S. Wu
Beijing Children Hospital, Beijing, China
Objective: Cockayne syndrome (CS) is a rare autosomal
recessive inherited disorder. We studied the clinical and electrophysiological
features in sixteen patients with CS. Methods:
Data of all patients with classical CS admitted in the
Beijing Children’s Hospital between September 1987 and
April 2001 had been reviewed. Results: There were 14
boys and two girls, ranging from 18 months–13 years.
Sixteen patients showed premature ageing, cachectic dwarfism,
microcephaly and mental retardation; 15 patients had
photosensitivity and ataxia, 13 patients had pigmentary
retinal degeneration, and 13 patients had sensorineural hearing
loss. Age-matched control study showed, results of electromyography
and nerve conduction velocity analysis
(EMG/NCV) were abnormal in ten of 14 patients. Both
motor and sensory nerves conduction velocities of median
nerve reduced in eight cases (26.0 , 34.3 and 26.9 , 40.0 m/
s, respectively), whereas in two cases, only motor nerves
conduction velocities reduced in both median and peroneal
nerves (40 , 42 and 30.7 , 38.8 m/s, respectively). All
patients were abnormal on cranial CT or MRI. CT scan
showed calcifications of the bilateral basal ganglia in 15
patients. In one case, it was normal on CT scan, but MRI
showed long T1 and long T2 signal lesions in left basal ganglia.
Conclusions: Our study showed that the most useful diagnostic
tool in CS is cranial neuroimaging. The findings of
EMG suggested the presence of primary demyelinating
neuropathy in peripheral nerves.
FP-H-038
Topamax as a first-choice drug in the treatment of West
syndrome
L.-P. Zou
Beijing Children Hospital, Beijing, China

504
Abstracts
Objective: To establish the efficacy, tolerability and
related events of using topamax as a first-choice drug in
children with West syndrome. Methods: Fifty-four patients
with West syndrome were given Topamax as monotherapy
or as add-on therapy to nitrazepam. We followed up them
ranged from 6 to 21 months (mean, 13 months). Results:
31cases (57.4%) were seizure-free more than 6 months. A
significant reduction from baseline in seizures frequency
was found in 44 cases (81.4%), poor or null response ten
cases (18.6%). The average, maximal and minimal doses
were 5.2, 26 and 1.56 mg/kg per day, respectively. The
adverse reactions included poor appetite (three cases),
absent sweating (three cases), to difficult falling asleep
(three cases). Conclusion: Topamax was proved to be an
effective and safe drug as a first-choice drug in the treatment
of West syndrome.
FP-H-039
Effects of topiramate on immunological function in
patients with West syndrome
L.-P. Zou
Beijing Children Hospital, Beijing, China
Objective: To explore the effect of topiramate on immunological
function in patients with West syndrome. Methods:
We used flow cytometry to measure the proportion of
lymphocytes subsets in peripheral blood of the patients
with West syndrome, and analysis/rate nephelometry to
measure immunoglobulin level in the serum of the patients
before and after topiramate treatment. Twenty-five normal
health infants were taken as controls. Results: After topiramate
treatment, CD4 1 cells in the patients were markedly
decreased (P , 0:05). Compared to control group, CD19 1
cells and serum IgA levels were significant decreased either
before or after topiramate treatment (P , 0:001 and
P , 0:05, respectively). NK cells in the patients were markedly
increased after topiramate treatment (P , 0:05). Serum
IgG levels were significant increased after topiramate treatment,
and nearly reached normal level. Conclusions: Treatment
with topiramate could affect immunological function of
the patients with West syndrome, and it may play a role on its
control to seizures.
FP-H-040
The relationship between the electroencephalogram
(EEG), head computerized tomography (CT) and the
prognosis of the West Syndrome
S.-X. Yu a , H.-Y. Dong a , Z.-X. Li b
a Jiading distract central hospital, Shanghai, China; b Jilin
city children Hospital, Jilin, China
Objective: To observe the relationship between the
EEG, head CT and the prognosis of the West Syndrome.
Methods: All of the 47 cases had EEG and head CT, and
we had a follow-up study to compare with their prognosis.
Results: Nineteen (60.6%) of 31 cases with abnormal
head-CT, and 14 (87.5%) of 16 cases with normal head-
CT showed progress. Twenty-nine of 34 cases with high
rhythm disorder of EEG demonstrated progress, whereas
only four cases with other kinds of EEG disorder did.
Conclusions: There was no obvious relation between the
prognosis of West Syndrome and the morphology of head
CT (P . 0:05). But there was an obvious correlation
between the prognosis and the change of EEG. The prognosis
of patients with high rhythm disorder of EEG was
better than those with the other kind disorders of EEG
(P , 0:01).
FP-H-041
TPM as adjunctive therapy in the long-term
management of children epilepsy
W. Zhou, H. Li, X.-Q. Liu
Qingdao Municipal Hospital, Qingdao, China
Purpose: A total of 35 children (mean age, 6.6 years; range
6 months , 14 years) with partial and/or generalized
seizures previously resistant to AED therapy (median baseline
seizure rate, six seizures/month) were treated with openlabel
topiramate (TPM) at dosages of 3.2 , 12.5 mg/kg per
day. Methods: The mean duration of TPM treatment was
447 ^ 142 days (range, 14 , 582 days) and the mean TPM
dosage was 6.75 mg/kg per day (range, 3.2 , 14.0 mg/kg per
day). Seizure reduction was calculated from seizure counts
during the last 3 months and last 6 months of TPM therapy
compared with baseline. Results: .70% of patients achieved
$50% seizure reduction. A total of 38% of patients were
seizure free for $33 months at the last visit; 43% of patients
were seizure free for $6 months at the last visit. This robust
therapeutic response was consistent for children patients
receiving TPM dosages ,4, 4 , 8 and .8 mg/kg per day.
The most commonly reported adverse events were related to
the central nervous system. Over the 1.5-year treatment
period, 26% of patients discontinued TPM therapy because
of adverse events and inadequate seizure control. Conclusions:
TPM as adjunctive therapy was well tolerated and
safe. It is a useful AED for long-term seizure control and
resulted in seizure freedom for extended periods despite failing
previous AED therapy.
FP-H-042
Treating children with refractory epilepsy with
additional topiramate
X.-W. Wang, Y. Sun, W.-D. Zi
Department of Pediatrics, People’s Hospital of Xingjiang,
Xingjiang, China
Purpose: This paper is to observe the curative effect and
safety in treating childhood refractory epilepsy with the

Abstracts 505
addition of topiramate. Method: Of the twenty cases we
treated, there were four cases of Lennox-Gastaut; three
cases of West syndrome; seven cases of complex partial
seizures; and six cases of generalized tonic-clonic seizures.
In addition to the primary drug treatment, topiramate was
added on with dosage from 0.5 mg/(kg per day), with an
increase of 0.5–1.0 mg/kg every 7–10 days, to 8.0 mg/(kg
per day) at its maximum, the purpose of which is to
observe the effect and side effect. Result: Of the twenty
treated cases, the drug was fully effective in eleven (55%),
seizures reduced by 75% in three (15%), 50% in three
(15%), and it was ineffective in two (10%). One failed to
return for a checkup. In terms of side effects, two cases had
anorexia so that further treatment became impossible; one
became obviously weak; two had symptoms of adiapneustia
and low fever; and the rest showed no obvious undesirable
symptoms. Conclusion: Topiramate is remarkable in
its effect and safe when it is added on in treating children
with refractory epilepsy. It also has low side effects.
However, the side effects, adiapneustia and low fever,
have not been reported up till now. Whether or not topiramate
has effect on the secretion of sweat gland need
further observation and research.
FP-H-043
K ATP channel subunit Kir6.2 and NMDA receptor
subunit 1 mRNA expression in status epilepticus rat
model
Z.-Z. Xia, K.-W. Jiang, Q.-X. Shui
Department of Neurology, Affiliated Children’s Hospital of
Medical College, Zhejiang University, Hangzhou, China
K ATP channels play a key role in the neurons excitability
for coupling the intracellular metabolic state to electrical
activity at the plasma membrane. To understand the role
of the K ATP channels in status epilepticus (SE), in the present
study, we determined the alterations of K ATP channels subunit
Kir6.2, and NMDA receptor subunit 1 mRNA expression.
SE was induced in male adult rats by single i.p.
pilocarpine. The survivals (n ¼ 43) were sacrificed for
mRNA assay at 1, 4, 6, 12, and 24 h after SE by in situ
hybridization. One hour after the seizures Kir6.2 mRNA in
CA1 and CA2 hippocampus increased, reached the highest
points at 4 h, and was still higher than that of normal control
group at 24 h after seizure. The up-regulation of the
NMDAR1 mRNA expression was slower than that of
Kir6.2 mRNA. SE evoked the expression of the Kir6.2
mRNA may activate the K ATP channels of the hippocampus
and the excitability modulation of the neuron itself. There
was no correlation between the expression of Kir6.2 mRNA
and NMDAR1 mRNA. It suggested that SE altered the
excitability by activating the NMDAR1.
FP-H-044
Teachers and children with epilepsy
I. Prpić a , Z. Korotaj a , I. Vlašić-Cicvarić a , E. Paučić-
Kirinčić a , A. Valerjev b , V. Tomac c
a University Children Hospital ‘Kantrida’ and Medical
Faculty of University of Rijeka, Rijeka, Croatia; b Primary
school ‘Kantrida’, Department of University Children
Hospital School, Rijeka, Croatia;
c Institute for Public
Health of Primorsko-Goranska County, Rijeka, Croatia
The purpose of the study was to investigate primary
school teachers’ attitude towards children with epilepsy.
Two hundred and sixteen teachers were investigated regarding
their opinion or knowledge about children with epilepsy.
The results reveal that teachers do not have an accurate
conception of the capabilities of children with epilepsy
and that those attitudes differ from teacher to teacher.
Nearly half of the teachers believe that children with
epilepsy differ from healthy children by their behaviour.
The majority of teachers (60%) do not get information
about child’s disease from the parents, but from other
sources. One third of teachers do not feel confident in
their work with children with epilepsy. Great majority
(91.80%) of teachers feel necessity to get additional information
and education about epilepsy. Primary school
teachers who work with children with epilepsy have inconsistent
approach to the capabilities, behaviour and an inadequate
way of working with children. Therefore they are
eager to get more information on epilepsy. Our results are
concordant with similar studies performed in various countries,
which prove that epilepsy as a disease has a similar
social effect on the affected person with no regard of cultural
or social background. It is the duty of the medical team to
offer teachers the necessary information, so that the
approach, work and life quality of children with epilepsy
can be improved.
FP-H-045
Effects of febrile seizures on peripheral blood leukocyte
count
J. Roganović, I. Prpić, E. Paučić-Kirinčić, I. Klarić
University Children Hospital “Kantrida” and Medical
Faculty of University of Rijeka, Rijeka, Croatia
In children presenting with febrile seizures, peripheral
leukocyte count is often determined to evaluate the source
of the fever. However, some studies have shown that an
increased leukocyte count, without any other obvious clinical
signs indicating a bacterial infection, might be
explained by the seizure duration itself rather than by the
nature of the infection. We assessed data of 82 patients (38
boys and 44 girls) with the history of 129 febrile seizures,
aged 6 months–5 years, referred to the Department of
Paediatrics, University School of Medicine, Rijeka, Croatia,

506
Abstracts
between January 1995 and July 2001. The characteristics
included duration of the fever prior to seizure, duration of
seizure, temperature at seizure and peripheral blood leukocyte
count. The parameters for bacterial infection were
defined as follows: obvious clinical finding, sedimentation
rate .40, and positive C-reactive protein. Our results indicate
that peripheral blood leukocyte count was significantly
higher in children with febrile seizures accompanied by
bacterial infection (16.58 ^ 0.86) compared to viral infection
(10.07 ^ 0.4), due to increased neutrophile count. In
children with bacterial infection no association between
seizure duration and blood leukocyte count was found. In
contrast, in children with febrile seizures and viral infection
the leukocyte count in the peripheral blood was significantly
related to the duration of seizures. We conclude that leukocyte
count should be used only as an additional tool to a
carefully performed patient history and physical examination.
FP-H-046
Mutation analysis of Na v 1.1 and GABRG2 in patients
with severe myoclonic epilepsy in infancy and its
borderland
I. Ohmori a , Y. Ohtsuka a , M. Ouchida b , K. Shimizu b ,J.
Hattori a , H. Ota a , M. Oka a , K. Abiru a , M. Inutsuka a ,F.
Endo a , E. Oka a
a Department of Child Neurology, b Department of Molecular
Genetics, Graduate School of Medicine and Dentistry,
Okayama University, Okayama, Japan
Purpose: To investigate genotype-phenotype correlation,
we performed mutation analysis of the voltagegated
sodium channel a1-subunit (Na v 1.1) and GABA A
receptor g2-subunit (GABRG2) in patients with severe
myoclonic epilepsy in infancy (SME) and its borderland
(SMEB) who lack myoclonic seizures. Subjects and methods:
Ten patients with SME and 11 patients with SMEB
were recruited. Genomic DNA was extracted from peripheral
blood leukocytes. All coding exons of the Na v 1.1 and
GABRG2 genes were amplified by PCR and analyzed by
direct sequencing. We also investigated clinical and EEG
findings on these patients. Results: Mutations of the
Na v 1.1 gene were identified in 18 of the 21 patients
(85.7%) with SME and SMEB. There was no difference
of detection rate and mutation type between the two
groups. No mutation was found in the GABRG2 gene.
Eight patients with SME (80%) had photoparoxysmal
response on EEG transiently during the clinical course,
in contrast to none with SMEB. Conclusions: It is thought
that the Na v 1.1 mutations were related to the common
clinical characteristics in the both groups. Other factors
may influence occurrence of myoclonic seizures and
photoparoxysmal response in SME.
FP-H-047
The occurrence of migraine in families of children with
benign rolandic epilepsy
H. Bazigou, M. Savani, A. Papavasiliou
Pendeli Children’s Hospital, Section of Pediatric Neurology,
Athens, Greece
The relationship of benign rolandic epilepsy (BRE) and
migraine has not been universally accepted and this study
intended to examine the occurrence of migraine in their
relatives as compared to healthy controls. Methods: Fiftynine
patients with BRE, 31 males, £ 4.7 years and 82
healthy children, 45 males, £ 8.2 years were included.
Detailed histories regarding migraine in first and second
degree relatives were obtained International diagnostic
criteria for migraine and ILAE classification for BRE
were utilized. Results: One child with BRE had migraine
VS none of the controls. This patient developed migraine at
the time of resolution of the BRE (13 years).Thirty BRE
patients (51%) VS 18 controls (22%) had either first or
second degree relatives or both who met the diagnostic
criteria for migraine. A chi-square test resulted in a
P , 0:05. Eleven were first degree relatives (36.6%) of
BRE patients versus 12 (66.6%) in the controls. Three
members from the first group and six from the second had
both first and second degree relatives with migraine. There
was a maternal prevalence (20 out of 30) in the relatives of
the BRE patients. Conclusions: Our results support observations
of some investigators regarding the increased
frequency migraine among relatives of BRE. The association
of these two entities might be due to a common defect
with variable expression. Further clinical and genetic
studies are needed to further clarify the possible association
between the two disorders.
FP-H-048
The effect of subconvulsant discharges on hippocampal
LTP and learning memory of space changes in rats
L. Gu, X.-Y. Lui
Department of Pediatrics, the affiliated railway hospital of
Shanghai, Tongji University, Shanghai, China
In order to study the effect of SED on learning and
memory, we set up a model of subconvulsant cerebral
discharges (SCD) by giving subconvulsant electric stimulation
(SES) on anterior frontal lobe cortex of rats. The results
showed that the SES in anterior frontal lobe could cause
SCD in cortex and hippocampal. The SCD interfered with
obtaining and store information and obstructed the transmission
from short-term memory to long-term memory. In the
rats which have formed the long-term memory, SCD can
inhibit extracting of information. These cognitive impairments
are reversible. Study about effect of SCD on hippocampal
LTP indicated that continuous discharges for 3 min

Abstracts 507
could temporally suppressed LTP built in the dentate gyrus
in hippocampus. The suppression phenomena disappeared
after stopping stimulation for 5 min. This phenomenon
revealed that LTP suppression is one the mechanisms of
SCD resulting in TCI. Presumably under the effect of
SES, EAA was over released, resulting in NMDA receptor
channel continuously opened. This leads to intracellular
excessive Ca 21 accumulation and TIC of neuronal function.
This may be one of the mechanisms of SED infecting learning
memory.
FP-H-049
Reasons for drug treatment failure in patients with
juvenile myoclonic epilepsy
R. Naumovski, M. Demerdziev
Clinic of Neurology, Clinical Centre, Skopje, Macedonia
Fourteen patients were referred to our dispensary for
epilepsy due to seizure pharmacoresistance (generally due
to generalized tonic-clonic seizures, GTCS) in the period of
1995–2000. The group consisted of six men (42.85%) and
eight women (57.14%), aged between 12 and 28 years
(18.78 1 4.33) who had previously been treated in pediatric
dispensary unsuccessfully. The disease onset ranged
between 5 and 14 years (10.7 1 3.17); all had myoclonic
seizures (previously not being diagnosed), 13 (92.85%) of
these patients had GTKS, and six (42.8%) had typical
absences as well. None of them previously was diagnosed
to have JME being treated with phenytoin, primidone,
carbamazepine, and part of them administered valproate;
none of them registered the number and the type of the
seizures and the therapy administered. After revision of
the diagnosis, the previous therapy was gradually interrupted
and valproate was administered as monotherapy in
therapeutic doses. Within a year, all patients achieved
complete control of myoclonic and GTS, and one patient
only reported occasional occurrence of absences. Our
results clearly suggested the superiority of valproate in
control of the mixed seizures in JME and the need of its
early diagnosis.
FP-H-050
Clorazepate for refractory epilepsies: excellent efficacy,
tolerance and adverse effects in 200 cases of personal
experience
K. Sugai, S. Hanaoka, M. Fukumizu, M. Sasaki
Department of Child Neurology, National Center Hospital
for Mental, Nervous and Muscular Disorders, National
Center of Neurology and Psychiatry, Kodaira, Japan
Prolonged efficacy, effective epilepsy classifications and
seizure types of clorazepate (CLZ) are controversial. Its
efficacy for refractory epilepsies, tolerance and adverse
effects were studied. CLZ was added on or replaced with
conventional AEDs in 200 patients with refractory epilepsies
unresponsive to three to ten conventional AEDs (average
5.9 AEDs), and its short-term efficacy was studied after
.4 weeks of CLZ administration. Long-term efficacy was
examined in 110 cases administered CLZ for .6 months.
CLZ was started at 0.3–1.0 mg/kg and increased by 0.2–0.5
mg/kg every 1 or 2 weeks up to 2.5 mg/kg. Seizures reduced
by .50% in 136 of 200 short-term subjects, including 45
seizure-free cases, and in 87 of 110 long-term subjects, with
25 seizure-free cases. Both short-term and long-term efficacies
were more favorable in the patients with localizationrelated
epilepsies or focal epileptiform discharges on EEG.
Short-term efficacy was better for partial seizures. Sixtyseven
episodes of adverse effects occurred in 60 patients
including 42 episodes of drowsiness, and CLZ was withdrawn
in 32 episodes. The frequency of adverse effects
decreased by modifications of initial dosage and increase
rate. Tolerance developed in 60 of 130 effective cases, by
2 months in 29 cases, by 4 months in 17 cases, by 6 months
in seven cases, and later than 6 months in seven cases. CLZ
again became effective in 51% of such cases by maintaining
or increasing the dosage. CLZ has prolonged efficacy for
refractory epilepsies. Frequent tolerance and adverse effects
were major but manageable problems.
FP-H-051
Localization-related idiopathic occipital epilepsy –
difficulties in classification
I.S. Ivanov
Department of Pediatrics, Plovdiv Higher Medical School
Hospital, Plovdiv, Bulgaria
The recognition of the types of childhood epilepsy with
occipital spikes (CEOS) is a widely discussed development,
which should promote prognosis and treatment of the
affected children. Aim: To test the clinical application of
the criteria for early-onset CEOS (Panayiotopoulos type)
and late-onset CEOS (Gastaut type). Methods: Retrospective
analysis of the clinical, electrophysiologic, imaging and
other data of the 126 epileptic children registered at the
epilepsy clinic of the pediatric department in the last 2
years. Results: Clinical and EEG data of occipital lobe
epilepsy were revealed in 11 children: four were diagnosed
as symptomatic or criptogenic, three – as late-onset type of
CEOS, and one – as early-onset type of CEOS. Three other
children disclosed signs of benign partial occipital epilepsy
but did not strictly fit the criteria for the CEOS types: The
first child had symptoms of early-onset CEOS (eye deviation,
vomiting, impaired consciousness, occurrence at night,
bilateral occipital discharges) but starting at the age of 9
years. The second patient was an 8 years old girl with occipital
sharp waves on the EEG which had only three brief
episodes of amaurosis with chaotic eye movements at the
earlier ages of 3, 4 and 5 years that ceased without treatment.
The third patient complained of frequent photopsies

508
Abstracts
induced by TV, rarely followed by secondary generalized
seizures, and focal occipital paroxysmal activity with photosensibility.
All but four patients were easily controlled by
carbamazepine or oxcarbazepine. Conclusion: The clinical
practice exposes a variety of symptoms that do not fit
exactly to the criteria for CEOS. Nevertheless strict adherence
to them is essential for unification purposes.
FP-H-052
Epileptic seizures in the first 5 years after hypoxicischaemic
encephalopathy – results from a prospective
study
I.S. Ivanov
Department of Pediatrics, Plovdiv Higher Medical School
Hospital, Plovdiv, Bulgaria
Epilepsy is a major sequela after HIE but prospective
studies on this topic are not numerous. Aim: To estimate
the frequency and characteristics of epileptic seizures after
HIE. Methods: A prospective study of the first 43 children
from a cohort of HIE patients that were followed-up from
birth though their 1st year and at 5-years age undergoing
neurological, kinesiological, developmental, psychological
and neurophysiological examinations. Results: Ten children
(23.2%, Sp ¼ 4.6%) developed epileptic seizures before
their sixth birthday. They were mostly partial (n ¼ 6),
followed by infantile spasms (n ¼ 2) and complicated
febrile convulsions (n ¼ 2). The age of onset was variable.
Valproates were the most frequently applied treatment.
Only two children continued to have seizures at 5-years of
age and three others were still on continuous medication. CP
was more frequent in the epileptic group (3/10) than among
children without epilepsy (0/33) while the occurrence of
mental retardation (MR) did not differ significantly (2/10
versus 4/33). Even when CP and MR patients were excluded
from analysis the results on the neurodevelopmetal Michelsson-Ylinen
test were worse in the epileptic group (33.6
versus 11.8, P , 0:01) and children with more than 24
points (suspected minor neurological dysfunction, MND)
were more common in that group (3/6 versus 1/27). The
occurrence of epilepsy correlated with worse neurodevelopmental
results at 1-year of age. Conclusions: 23.2%
(Sp ¼ 4.6%) of children that suffered HIE developed epileptic
seizures in their first 5 years. They were most often
partial and frequently associated with CP and MND and
abnormal neurodevelopmetal status at age 1 year.
FP-H-053
The alterations of K ATP subunit Kir6.2, SUR1, and
NMDA receptor subunit 1 mRNA in cerebral cortex and
hippocampus of rats with picrotoxin-induced seizure
Q.-X. Shui, K.-W. Jiang
Department of Neurology, Affiliated Children’s Hospital of
Medical College, Zhejiang University, Hangzhou, China
Previous reports showed that the K ATP channels play a
pivotal role in neuroprotection induced by epileptic preconditioning.
To study the role of K ATP channels in chronic
epilepsy, we investigate the alterations of K ATP subunit
Kir6.2, SUR1, and NMDA receptor subunit 1 mRNA in
cerebral cortex and hippocampus of rats with picrotoxin
(PTX) induced seizure. Two weeks after the last continuous
PTX injection (20 times, i.p.), those achieved a completely
kindling state (n ¼ 36) were divided into four groups
randomly. And three groups were injected with PTX once
more to kindle the seizures, which sacrificed at 1 h, 1 and 3
days after the seizures, respectively. Another two groups
were the interictal group and control group. K ATP subunits
Kir6.2, SUR1, and NMDA receptor subunit 1 mRNA
expression were detected by in situ hybridization. The
results showed the levels of Kir6.2 mRNA and SUR1
mRNA of the 1 h, 1 and 3 days after seizures groups were
higher than those of interictal and control groups
(P , 0:05). especially the 1 h group. These mRNA
expressed highest in hippocampus CA1. There was no
significant difference of NMDAR1 mRNA among all
seizure model groups, but with a significant difference
compared with the normal control group. Our results
suggested that model process was also a preconditioning
to activate the excitability modulation of the neuron itself,
and the activation of NMDAR1 may result in kindling of the
chronic epilepsy.
FP-H-054
Malignant migrating partial seizures in infancy
V. Gross-Tsur, B. Ben-Zeev
Neuropediatric Units of Shaare Zedek Medical Center and
Sheba Medical Center, Jerusalem, Israel
We report two cases with malignant migrating partial
seizures in infancy, a disorder described in only 20 other
patients. Patient 1, a 3 year old girl, was born with microcephaly
of unknown origin and at age 4 months intractable,
multifocal seizures appeared with bilateral deviation of the
eyes and head, tonic elevation of the arm and flushing of the
face. Despite vigorous AED therapy, the seizures became
virtually continuous and her development arrested so that at
age 3 years she just smiles and responds to voices. A second
pregnancy was terminated at 32 weeks because of progressive
microcephaly, head circumference three standard
deviations below normal. Patient 2 had normal development
until 3 months of age when multiple episodes of partial
status epilepticus appeared, characterized by decreased
motor activity, head deviation, eye blinking and cyanosis.
Aggressive AED therapy was ineffective and at her death at
18 months she had severe psychomotor delay. Interictal
EEG in both cases showed diffuse slowing of background
activity and multifocal spikes. During seizures, the EEG
was characteristic of the disorder, showing rhythmic theta
activity restricted to one region spreading to adjacent areas

Abstracts 509
or the entire hemisphere. In consecutive attacks the epileptogenic
activity shifted between hemispheres (ping-pong
seizures). MRI showed mild atrophy. MRS (patient 2) was
normal. An extensive biochemical and metabolic workup
was normal; TORCH and karyotype in patient 1 and CSF
neurotransmitters in patient 2 were also normal. In
summary, we present two patients with malignant migrating
partial seizures in infancy. The etiology of this syndrome is
unknown but the familial microcephaly in patient 1 is
suggestive of a genetic basis for at least a subgroup of
patients. Alternatively, neurotransmitter dysfunction with
abnormal excitatory activity may explain the continuous
erratic epileptic activity.
FP-H-055
Topiramate as add-on therapy in children with
refractory partial seizure
J. Wu, Z.-P. Wang, K.-R. Bao, X.-P. Xu
Shanghai Xin Hua Hospital, Shanghai Second Medical
University, Shanghai, China
Objective: To evaluate the efficacy and safety of TPM in
children (age 4–14 years) as adjunctive therapy for intractable
partial-onset seizures with or without secondarily
generalized seizures. Methods: Patients with at least six
partial-onset seizures during the 12-week baseline phase
while maintained on therapeutic doses of one or two appropriate
AEDs were treated with TPM. The initial TPM doses
were 0.5–0.7 mg/kg per day and increased by 0.5–1.0 mg/kg
per day increments at 1-week intervals. Top dose was ,8
mg/kg per day. Results: Eight patients (25%) were seizure
free for at least 7 months and more than 50% reduction in
seizure was found in 14 patients (43.75%). Inefficacy was
21.88 and 6.25% of the patients had their seizure frequency
increased during treatment. Two patients (6%) had transient
anorexia, dizziness and somnolent, two (6%) had dizziness,
bluntness and impairment of memory, one (3%) had speech
decreased, and one (3%) had abnormal behavior. The
adverse event developed during increment of TPM and
disappeared gradually within 2–4 weeks. Conclusion:
TPM therapy was effective and well tolerated in most of
the patients.
FP-H-056
Predictors of intractable epilepsy – a case-control study
in a cohort of Chinese epileptic children
L.K. Kwong, W.K. Chak, K.T. So
Department of Paediatrics, Tuen Mun Hospital, Hong
Kong, China
Informationaboutearlypredictorsofintractableepilepsyin
childhood is scarce. We performed a case-control study to
identify early predictors of medically intractable epilepsy in
children. Cases were patients who had an average of at least
one unprovoked seizure per month during an observational
period of at least 2 years. Controls were children having
achieved at least 2 seizure-free years. Strong univariate association
was observed between intractability and the following
factors: onset of seizure in infancy, high initial seizure
frequency, remote symptomatic etiology, infantile spasms
and mixed seizure types, abnormal neurological status,
history of status epilepticus, neonatal seizure and early breakthroughattacks
after treatmentinitiation.Independentpredictors
of intractability with multiple regressions were: age of
seizure onset (OR 0.53, 95% CI 0.35, 0.97), abnormal neurological
status (OR 18.6, 95% CI 5.19, 63.6), .3 attacks in the
second half year of treatment (OR 21.86, 95% CI 7.35, 65.01)
and febrile seizures (OR 25.9, 95% CI 1.41 455.7). Our study
suggested that seizure intractability could be predicted early
in the course of illness in children with abnormal neurological
status, high initial seizure frequency, early presentation of
epilepsy and lack of early response to treatment.
FP-H-057
Seizure recurrence in epileptic children after
withdrawal of anti-epileptic drugs
B. Törer, N. Şenbil, Ö.F. Aydin, Ü. Ertan, Y. Gürer
Dr. Sami Ulus Children’s Hospital, Ankara, Turkey
Aim and method: We investigated the rate and the predictive
factors of seizure recurrence after AED discontinuation
in a retrospective analysis of 76 children with epilepsy who
had not had a seizure for at least 2 years, infantile spasms
were excluded. Prognostic criteria analyzed in the patients
were: sex, age at seizure onset, age at AED discontinuation,
type of seizure, etiology, type of epilepsy, family history of
epilepsy in first-degree relatives, mental retardation, delay
in neuromotor development, motor deficit, EEG abnormalities,
multiple medication, history of neonatal and febrile
seizures, history of status epilepticus, time from first to
second seizures, duration of epilepsy, duration of AED
treatment, number of seizures before medication, number
of seizures during first 6 months of treatment, time required
for seizure control, number of seizures experienced until
seizure control, seizure free interval. Results: The overall
recurrence rate was 32.9%. More than half of the recurrences
occurred within 1 year after discontinuation of therapy
and all within 2 years. The factors indicating a higher
recurrence risk were older age at AED discontinuation,
longer duration of epilepsy, longer duration of treatment,
longer time required for seizure control (P , 0:05, Mann–
Whitney U-test). Conclusion: Although some factors
provide an estimate of an individual’s likelihood of recurrence,
the final decision regarding medication withdrawal
must be individualized and should be made jointly by the
physician and the family. The decision process must take
into account the statistical risk of seizure recurrence, the
potential consequences of such a recurrence, and the potential
adverse effects of continued AED therapy.

510
Abstracts
FP-H-058
Case-control study and transmission/disequilibrium test
of childhood absence epilepsy in Chinese population
J.-J. Lu, H. Pan, Y.-H. Zhang, X.-R. Wu
Department of Pediatrics, Peking University First Hospital,
Beijing, China
CAE is one of the most frequently recognized syndromes
of the idiopathic generalized epilepsies. The inheritance of
CAE is complex. Genes responsible for susceptibility to
CAE have not been identified. Pharmacological studies of
animal models have implicated the GABAergic system
involved in the mechanism of this disease. Our previous
study suggested that the GABA A receptor subunits genes-
GABRA5 and GABRB3 may be either directly involved in
the etiology of CAE or in linkage disequilibrium with
disease- predisposing sites. To further confirm the results
we used the SNPs found in the coding regions of the
GABA A receptor subunits genes GABRA5 and GABRG2
as genetic markers to detect TDT in 90 Chinese nuclear
families. The TDT results suggested that GABRA5 and
GABRG2 may not be the susceptible genes to CAE. On
the other hand, animal models suggested that the GABA B
receptor plays a critical role in the epileptogenesis of
absence seizures. As a result, we used the SNPs found in
the GABA B receptor subunits gene GABBR2 as genetic
markers to conduct a case-control study in 96 CAE patients
and 96 controls from North China. The frequency of the
alleles and genotypes of SNPs showed significant discrepancy
between CAE patients and normal controls. The
results suggested that GABBR2 may either directly
involved in the etiology of CAE or in linkage disequilibrium
with disease-predisposing sites.
FP-H-059
Therapeutic drug monitoring and the pharmacokinetics
of carbamazepine in children
B.-M. Li, R.-P. Sun, J.-W. Wang, G.-F. Lei, R.-M. Hu, S.-H.
Guo
Pediatric department, Qilu hospital, Shandong University,
Jinan Shandong, China
Objective: To investigate the factors affecting CBZ serum
concentration in children with epilepsy. Methods: The minimum
steady-state concentrations of CBZ of 68 specimens of
49 children with epilepsy were measured using HPLC.
Results: The minimum steady-state concentrations of CBZ
increased with dose, but no statistical difference was found
among the groups of the children administrated different
doses (P . 0:05). It was seen that CBZ concentrations
increased with weight and age of children. However, sex
and the time after administrating CBZ did not affect the
CBZ serum concentrations significantly. Conclusions: The
factors affecting concentration of CBZ were mainly body
weight, age and dosage. There was no significant correlation
between the concentration of CBZ and sex or time after
CBZ administration. Therefore, CBZ doses that would be
suitable for pediatric patients of different ages or weights are
proposed. And a small CBZ dose at the beginning is the best
choice for administration to children with epilepsy.
FP-H-060
Clinical analysis of childhood convulsion in 508 cases
S.-Y. Shao
Department of Pediatrics, First Affiliated Hospital, Inner
Mongolia Medical College, Huhhot China
Aim: To study the pathogenesis, the season in which the
disease occurred and the preventive measures of childhood
convulsions. The results are that febrile convulsions are
mainly induced by upper respiratory tract infections with
high fever and non-febrile convulsions of infants are mainly
induced by hypocalcemia and CNS infections. Childhood
convulsions can be seen in four seasons of 1 year, especially
in Winter and Spring. Prevention of childhood upper
respiratory tract infections and CNS infections and good
care of high-risk infants are significant factors in decreasing
the percentages of childhood convulsion.
FP-H-061
Febrile seizures: factors affecting risk of recurrence in
hospital in Pakistani children
S. Akram, Z. Habib, S. Ibrahim, B. Hasan
Department of Pediatrics and Physical Therapy, Aga Khan
University Hospital, Karachi, Pakistan
Objectives: This is the first descriptive study that
addresses risk factors for seizure recurrence in the emergency
room (ER) in Pakistani children. Aims of the study
were to: (a) describe the effect of temperature rise on seizure
recurrence in the ER; (b) investigate the effect of age,
gender, family and developmental history, type, duration
and multiple seizures, past history and number of seizures
and treatment given (either late or early) on seizure recurrence
in the ER; and (c) explore prognostic indicators for
seizure recurrence in the ER. Methods: Data from 352 children
[ages 3–84 months; 220 males (62.5%) 131 females
(37.2%)] was gleaned from chart reviews for the years January
1998–August 2000 inclusive, from the Pediatric department
of the Aga Khan University Hospital. Descriptive
statistics, chi-square, odds ratios, 95% CI and discriminant
analysis were used. Results and conclusions: Of the 52
(16%) cases that had seizure recurrence in the ER, majority
(36.5%) occurred in .38.5 #39.58C temperature range. The
percentage declined to 15% at higher temperatures. Bivariate
tests showed that age, family and developmental history,
type of seizure and treatment given did not affect seizure
recurrence in the ER. Past history number of seizures

Abstracts 511
(P ¼ 0:008, OR ¼ 2.4) and duration of seizure (.5 min)
(P ¼ 0:000, OR ¼ 2.2) were the two most important factors
affecting seizure recurrence in the ER at the bivariate level
of analysis. Duration of seizure was also the most important
prognostic indicator for FS recurrence in the ER at the
multivariate level with b ¼ 0.79. Early treatment did not
affect recurrence, suggesting timely anti-pyretic versus
anti-epileptic medication use.
FP-H-062
Tuberous sclerosis and epilepsy
D. Plesca, F. Buruiana, D. Dragomir
Department of Pediatric Neurology, Dr. Victor Gomoiu”
Children’s Hospital, Bucharest, Romania
Introduction: Tuberous sclerosis is one of the most
commonly occurring and recognized neurocutaneous
syndromes, with a prevalence of about 1 in 6000 newborns.
It is a multi-system disorder affecting predominantly the
CNS and skin. Neurological manifestations associated
with tuberous sclerosis include: recurrent seizures, retardation,
psychological and behavioral disturbances. Epilepsy,
occurring with an incidence ranging between 80 and 90% in
tuberous sclerosis patients, represents the most important
clinical problem. Method: Fourteen children with tuberous
sclerosis associated seizures were diagnosed and evaluated
between 1995 and 1999 at the Department of Pediatric
Neurology. Their clinical manifestations and evolution
under therapy are evaluated in this study. Results: Different
types of seizures were encountered (infantile spasms, focal
and generalized tonic-clonic seizures). The clinical and
electrical patterns were found to be related to the age of
the patient. The patients benefited from anticonvulsant therapy.
The patients were reassessed at 3, 6, 12 and 24 months
in the order to evaluate their response to the therapy and
neurological and mental stage of their development.
Conclusions: This study confirms the existence of a relationship
between the early onset of seizures and the severity of
mental retardation. Failure to obtain seizure control under
therapy represents an unfavorable predictive factor for
neurological and mental outcome of children with tuberous
sclerosis.
FP-H-063
Difficulties of diagnosis in a case of seizures in infancy –
case report
D. Plesca, M. Moiceanu, F. Buruiana, D. Dragomir
Department of Pediatric Neurology, ‘Dr. Victor Gomoiu’
Children’s Hospital, Bucharest, Romania
Background: Menkes disease is a neurodegenerative
illness with an X-linked inheritance. The degenerative
process affects the grey matter in the central nervous
system. The diseased infant presents with particular features
of the hair and facies. The genetic defect is located on the
long arm of the chromosome X (Xq13.3). The authors report
the case of a 2 months and 3 weeks old, male infant (S.O.)
admitted on 17 September 2000 for intractable myoclonic
seizures. On physical examination the patient exhibited an
expressionless facies with rounded cheeks, sparse, short,
rough hair, dry, pale skin, obvious generalised muscular
hypotonia and absent deep tendon reflexes. There were no
eye movements elicited by a moving object and no sounds
produced. EEG revealed irrigative elements and slow, highvoltage
waves located alternatively on the right and on the
left leads. Eye funduscopy showed bilateral optic atrophy.
Serum copper and ceruloplasmin were extremely low (0 and
5.2 mg/dl, respectively). The presumed diagnoses of
Menkes disease was confirmed by a microscopic examination
of the infant’s hair that featured a twisted, fractured
aspect and multiple knots which are quite typical for
Menkes disease. Enzymatic assays were not feasible.
Conclusions: Based on the aforementioned clinical and
paraclincal data, a diagnosis of Menkes disease was established.
Despite its low incidence this disease, still represents
a cause of seizures in infants.
FP-H-064
Benign rolandic epilepsy in two pairs of identical twins
with mirror image localization of spikes
R. Amit
Division of Neurology, Tod Children’ Hospital, Youngstown,
Ohio, USA
Background: BRE is an age and localization related
epileptic syndrome believed to be genetically determined
by autosomal dominant trait. Objective: In the absence of
reported studies of BRE in identical twins, to report this
epileptic syndrome in two pairs of identical twins with
mirror image of the localization of the spikes. In one pair
of twins a discordant handedness was present. Methods:
Medical history, seizures history, physical and neurological
examinations as well as imaging studies of the brain, routine
and long term video-EEG were used. In one case of doubted
identity a monozygocity test was used. Conclusion: BRE
does exist in identical twins. The transcription of the epileptogenic
area may have a mirror image pattern.
FP-H-065
CSF glucose concentrations in convulsive infants with
and without fever and the role of acetaminophen
M. Mohammadi a , M.R. Mohebbi b , F. Naderi c
a Children’s Medical Centre, Tehran University of Medical
Sciences (TUMS) b TUMS,
c Bahrami Children Hospital,
TUMS, Tehran, Iran
Hyperglycaemia is common following FS. This leads to
hyperglycorrhachia within 1–2 h after a convulsion. The

512
Abstracts
aim of the present study was to explore the relationship
between the CSF glucose concentrations, and body temperature,
seizure, and prior use of acetaminophen. The cases
consist of 117 convulsive infants aging 3–18 months.
Patients were divided in to several groups based on body
temperature, duration of fever and convulsion, and prior use
of acetaminophen. There was a linear correlation between
CSF glucose level and body temperature in those who had
not taken acetaminophen before admission (r ¼ 0:515,
n ¼ 83, P , 0:001 Spearman’s correlation analysis). The
CSF glucose levels were significantly higher in the febrile
infants (75.33 mg/dl, n ¼ 70), compared with the nonfebriles
(66.15 mg/dl, n ¼ 13, P ¼ 0:014). There was a
negative correlation between the CSF glucose level and
body temperature in those who had taken acetaminophen
before admission (r ¼ 20:389, n ¼ 34, P ¼ 0:023).
Although the mean CSF glucose concentration was higher
in those who had taken acetaminophen compared to the
controls, the difference was not statistically significant
(P ¼ 0:076). Other factors such as age, type of FS, duration
of fever and convulsion and multiplicity of convulsions
were not significantly correlated with CSF glucose concentration.
Our study shows that both fever and convulsion are
related with increased CSF glucose concentrations. The role
of acetaminophen deserves more investigation.
FP-H-066
Benign infantile convulsions associated with mild
gastroenteritis
J.-H. Wu, L. Yin, L.-H. Cao
Department of Pediatrics, Maternity and Children Hospital,
Tangshan, Hebei, China
Objective: To delineate and study the so-called benign
infantile convulsions associated with mild gastroenteritis
(BICE) in our hospital which is located in northern China.
Methods: A clinical study was conducted between January 1,
1999 and April 30, 2001 in all the patients who were treated
for gastroenteritis (GE) accompanied by seizures, to delineate
the clinical characteristics, then follow up these patients
to clarify the prognosis of this syndrome. Results: There were
898 patients who were admitted to hospital during the study
period. Of them, there were 33 patients with GE accompanied
by seizures during the course of the disease: of these 16
patients had mild GE accompanied by a febrile seizures.
There were nine boys and seven girls, the peak age was
between 1 and 2 years old (12 cases, 75%): the youngest
was 4.5 months, the eldest, 2.2 years. The clinical courses
were mild in all cases, accompanied by a febrile seizures of
generalized tonic-clonic pattern (GTCS). The inter-ictal
EEG study (12 cases) and video-EEG study (four cases)
disclosed 4–5 Hzu activity with a background activity of
normal sleep EEG patterns. Neither spike and wave nor
sharp wave activities were seen. Other laboratory findings
including serum electrolytes, blood sugar, liver enzymes
were within normal limits. In three cases the CSF was also
examined and all specimens were normal. In ten cases a CT
scan of brain was performed, with normal results. The
seizures were easy to control. During follow-up study of 14
cases after discharge from hospital (mean period: 1.2 years),
these children have had no further seizures and have normal
development in terms of physical and mental expectations in
the follow-up period. Conclusion: A febrile convulsions
associated with mild gastroenteritis occur in northern
China, with an occurrence rate about 1.78% in the in-patients
with GE in infants. The seizure patterns were GTCS, with
good out-comes. Therefore this syndrome could be called
BICE.
FP-H-067
Does long-term use of valproate cause weight gain in
prepubertal epileptic children
H. Çaksen, G. Deda, M. Berberoǧlu
Ankara University Medical School Departments of Pediatric
Neurology and Endocrinology, Ankara, Turkey
VPA is an effective anticonvulsant in many types of childhood
epilepsies. Use of VPA, however, is associated with an
increase in body weight. The pathophysiology of weight gain
is not fully understood. One theory is that elevated levels of
serum leptin cause the weight gain in patients receiving VPA.
In our study we evaluated 15 prepubertal children before and
after VPA treatment for weight gain by calculating their BMI
and serum leptin levels. Our study included 15 patients with
new diagnosed epilepsy and 16 healthy age matched controls.
The patients’ ages were between 9 months and 12 years. The
BMI before VPA treatment in the study group and the control
group were as follows: 16.25 ^ 1.63, 16.34 ^ 2.31. The
serum leptin levels before VPA treatment in the study
group and the control group were respectively; 3.79 ^ 2.39
and 4.28 ^ 2.43. After VPA treatment the BMI in the study
and control groups were as follows; 16.20 ^ 1.75,
16.62 ^ 2.37. The serum leptin levels after VPA treatment
in the study and control group were, respectively;
4.70 ^ 2.80 and 5.44 ^ 2.79. There was no difference
between the groups for BMI and serum leptin levels. In
conclusion, our findings showed that long-term use of VPA
did not cause weight gain in prepubertal children receiving
VPA therapy, and there was no significant difference between
the serum leptin levels of the study and control group.
FP-H-068
Remarks on clinical efficacy of the treatment of
childhood epilepsy in 2000–2001
N.T. Ung, L.T. Huong
Neurology Department, National Institute Pediatrics,
Hanoi, Vietnam
Objective: To study 464 children with epilepsy aged

Abstracts 513
from 1 month to 15 years and examine the efficacy of the
treatment. Methods: By retrospective methods we studied
464 with childhood epilepsy in 2000–2001. Result: There
are 49.8% under 1 year, 25% from 1 to 3 years, 12.3%
from 3 to 6 years, 8.8% from 6 to 11 years, 4.1% from 11
to 15 years. A total of 59.5% was boys, and 40.5% girls.
The following seizure patterns and frequencies were
observed: 21.3% simple partial, 3.7% partial with secondarily
generalized, 75% generalized seizures (61.6% tonicclonic,
3.7% tonic, 2.2% atonic, 2.8% absence, 4.1% West
syndrome, 0.6% Lennox Gastaut syndrome. A total of
72.4% were on monotherapies, 27.6% were on polytherapies
(9.7% a combination of valproate and phenobarbital,
6.4% valproate with carbamazepine, 2.4% valproate with
vigabatrin, 3.2% valproate, with carbamazepine with
phenolbarbital, 0.8% valproate with phenobarbital with
vigabatrin). The treatment was ineffective in 0.7% cases.
A total of 81.9% became seizure free, 12.7% had at least
80% reduction of seizures, 4.7% had on 50–80% reduction,
0.7% had less than 50% reduction. Side effects were
observed 1% cases.
FP-H-069
Comparative study of epilepsy in cerebral palsy and in
developmentally normal children
M.M. Rahman, S. Akhter, M.N. Islam, M.A. Mannan, K.
Akhter, F.A. Khan, S. Parveen
Department of Paediatrics, Bangabandhu Sheikh Mujib
Medical University, Dhaka, Bangladesh
This is a prospective analytical hospital-based study done
from September 2000 through February 2001 to observe the
clinical profiles of epilepsy in CP and in developmentally
normal children. Fifty children with CP and 50 developmentally
normal children, aged 1–15 years, comprised the study
population. In both groups male outnumbered female children.
In both groups most of the cases were under 5 years
old. Before the 5th year of age, onset of seizures was 46
(92%) in the CP group (group A) and 39 (78%) in developmentally
normal children (group B). Family histories of
seizures were present in 10 (20%) cases in group A
compared to 8% cases in group B. Perinatal asphyxia 53
(45%), and LBW, 25 (22%), were the commonest risk
factors in group A. On the other hand, in group B perinatal
asphyxia was observed in 14 (26%) cases, and LBW in six
(11%) cases. Spastic tetraplegia, 34 (65%), was the
commonest motor defect in CP patients. Generalized
tonic-clonic was the commonest type of seizure found in
both group - A 23 (43%) and in group B 30 (57%). In
group A 38 (76%) cases had cryptogenic and symptomatic
generalized epilepsy, 12 (24%) had localization-related
epilepsy. In contrast, in group B 42 (84%) cases had idiopathic
generalized epilepsy and eight (16%) cases had localization
related epilepsy. Response to anticonvulsants was
less good in group A than in group B. The clinical profile of
epilepsy in cerebral palsy patients differs from that of developmentally
normal children.
FP-H-070
Gelastic seizures in children
H.-F. Lee, C.-S. Chi, S.-C. Mak, C.-H. Chen
Department of Pediatrics, Taichung Veterans General
Hospital, Taichung, China
Objectives: To describe the etiology, clinical evolution of
epilepsy, and administration of AEDs in patients with GSs.
Methods: Fourteen patients whose seizures were characterized
by typical laughing attacks were observed between
1990 and 2002. All patients received EEG and MRI study.
Results: One patient’s disorder was related to a HH and four
to abnormal MRI findings without evident hypothalamic
lesions. In nine patients, MRI was negative. EEG in the
HH patient showed 2–2.5 Hz SWC. In the group with abnormal
MRI findings, all four patients had focal epileptic
discharges. In the group with normal MRI findings, EEG
in six patients revealed focal epileptic discharges and three
had normal results. The epileptic syndrome in the HH case
was drug-resistant, with the response rate to AEDs less than
25%. The response rates of AEDs in the group with abnormal
brain MRI findings were less than 25% in one, 25–50%
in one, 75–90% in two cases. In the other nine cases without
abnormal MRI findings, three cases became seizure free and
six cases had drug resistance or partial response to treatment.
Conclusions: GSs are the frequent findings in HHs,
but GSs may also be observed in patients without MRI
lesions and with normal neurological status. In these
patients, clinical evolution and response rate to AEDs
seem to be more benign.
FP-H-071
Study on the quality of life in families of children with
epilepsy
Y.-L. Huang a , X.-Y. Wu a , P. Wang a , K.-Z. Ma b , P.-J. Shen b
a Department of Pediatrics, Union Hospital, b Department of
Medicine, Tongji Medical College, Huazhong University of
Science and Technology, Wuhan, China
Objectives: During the 2–4 years needed for standard
antiepileptic therapy in epileptic children, their guardians’
cooperation plays an important role in the administration of
the treatment. The guardians’ quality of life (QoL), attitudes
to therapy, and, their compliance are of special importance,
because these factors affect the success of therapy. The
purpose of the study is to investigate the QoL of the parents
of the epileptic children and to find out their attitudes to
epilepsy and to therapy and lastly, the factors affecting
QoL in the family, so that correct advice may be given to
them, allowing them to retain normal psychological profiles,
and achieve compliance for the standard therapy; and,

514
Abstracts
finally, create good environments for the lives and education
of the sick children and to improve their QoL and get the
best results from their physical and psycho-therapy. The
guardians’ attitudes to the anti-epileptic therapy are closely
related to the success of the treatment, education, psychological
development and QoL of the sick children. This
study is aimed at investigating the QoL of parents of epileptic
children, in order to offer appropriate advice to them and
to ensure the success of the standard anti-epileptic treatment.
Methods: The experimental group includes 100
families with epileptic histories of 4 months ,14 years.
The control group consists of 100 families with children
who suffered acute diseases without seizures. The degrees
of anxiety and depression were assessed in both groups. The
questionnaires, including the epileptic situation, family
profiles, parents’ attitudes to epilepsy, the effects of epilepsy
on their lives, etc were offered simultaneously. Results: (1)
In the experimental group, the total score of anxiety and
depression (AD) was: 20.32 ^ 4.44, the anxiety scoring
(A): 10.71 ^ 2.15 and the depression scoring (D):
9.72 ^ 2.68. In the control group, the total score of anxiety
and depression (AD) was: 5.93 ^ 1.55, the anxiety scoring
(A): 3.34 ^ 1.25, and the depression scoring (D):
2.71 ^ 0.65. There was a significant difference between
two groups. (2) Questionnaire: most parents (80%) were
unwilling to let others know of their children’s disease,
and 55% were unwilling to let their children know of the
disease. A total of 81% worried about the effects of the
disease on growth, education, and QoL. Conclusion: Parents
were commonly in a state of anxiety and depression. The
quality of life in these families was decreased. It is
suggested that the mood changes of the parents will exert
negative influences on epileptic therapy. It is therefore
reasonable to give psychological advice to epileptic
families.
FP-H-072
Subcortical band heterotopia – clinico-radiological
analysis of eight cases
A. Kelemen, P. Barsi, G. Rásonyi, J. Janszky, G. Filicki
National Institute of Neurology and Psychiatry, Budapest,
Hungary
Subcortical band heterotopia is considered to be the mildest
end of the lissencephaly – pachygyria – band heterotopia
spectrum, genetically a heterogeneous group. In this work,
we correlated the clinical characteristics (sex, age of the
onset of epilepsy, seizure type, mental and neurological
deficit) with MRI characteristics (antero-posterior gradient,
distribution, asymmetry, additional findings). The first
seizure appeared around puberty in most patients (6/8).
All the patients had multiple seizure types. In patients
with the frontal dominant form (all females) the seizure
disorder begun with GTCS and these patients have lower
IQ (5/5) regardless of the band width, distance and abnormality
of the overlying cortex and extension of the malformation.
Both the males had the posterior dominant form.
Cerebellar hypoplasia (in the frontal form) and aberrant
corpus callosum (mainly in the posterior dominant form)
were the most frequent additional MRI findings. Subcortical
band heterotopia is clinically heterogeneous, with some
distinct clinicoradiological features. Should the currently
ongoing genetic tests reinforce our observations, the clinical
features would enable targeted genetic analysis and counselling.
FP-H-073
Clinical analysis of 375 cases childhood seizures
W. Hou
Department of Pediatrics, DaGang Oil Field Worker’s
General Hospital, Tianjin, China
A review the data of patients with convulsion admitted in
our hospital from 1989 to 1993. There were 246 males
(66%) and 129 females (34%). Thirty-seven of them
(10%) were newborns, 254 (68%) below 3-year old, 62
(17%) below 6-year old. For the newborns, the major
cause was HIE, which occurred in 20/37 (54%), followed
by hypocalcemia in 14/37 (38%). For the infants, the most
frequent causes were tetany, which occurred in 119/254
(47%), febrile convulsions in 99/254 (39%) and epilepsy
in 13/254 (5%). For the preschoolers, the major cause was
febrile convulsions, occurring in 46/62 (745), followed by
epilepsy in 7/62 (11.2%), viral encephalitis in 3/62 (5%).
For the school-age children, the most common reasons were
viral encephalitis in 10/22 (45%) and epilepsy in 9/22
(41%). Among 375 cases, the commonest causes in order
of frequency were febrile convulsions in 150/375 (40%),
infantile tetany in 133/375 (35.5%), epilepsy in 30/375
(8%), viral encephalitis in 17/375 (4.5%) and toxic bacillary
dysentery in 6/375 (1.6%).
FP-H-074
Evaluation of sleep-deprivation EEG in the diagnosis of
childhood epilepsy
L.-H. Chen, Q.-Z. Pang, L.-Q. Chen, Y.-F. Lu
Shanghai Children’s Hospital, Shanghai, China
Objective: To evaluate the sensitivity of sleep-deprivation
EEG examination in children. Method: Sixteen children
suspected of epilepsy clinically but with normal standard
EEG examinations were selected at random (male 12,
female four, age 3–11 years, mean 6 years). Sleep-deprivation
EEG was performed individually. Periods of sleep
deprivation were 18–20 h for children below 7 years and
24 h for the older ones. Results: All the EEG showed slowing
of background electric activity. In addition, eight cases
(50%) showed spike/sharp-slow wave complexes, three
cases had bursts of high voltage u activity, and five cases

Abstracts 515
had normal recordings. The rate of EEG abnormality was
68.75%. Conclusion: Sleep-deprivation EEG is more sensitive
than standard EEG in showing abnormal electric activity,
and has practical value clinically.
FP-H-075
The follow-up survey on the prognosis of 82 cases with
febrile convulsions
P.-F. Xu, Z.-S. Zhou
Department of Pediatrics, China-Japan Friendship Hospital,
Beijing, China
Objective: To explore the relapse of patients with febrile
convulsion (FC) and the relationship between FC and
epilepsy (EP). Subjects and methods: One hundred eight
cases with FC (66 boys and 42 girls) were admitted to our
hospital from 1998 to 2001. Their ages averaged 4.2 ^ 2.4
years. Questionnaires were taken to survey subsequent
conditions. Results: Amongst 108 in-patient hospital cases
with FC during the survey period, 82 cases (75.9%) actually
took part in this survey. There were 70 cases with simple FC
(85.3%) and 12 cases with complex FC. Of 70 cases with
simple FC, relapses occurred in 28 cases (38.8%), of whom
24 cases (85.7%) relapsed within 1 year of onset. Six cases
converted into EP accounting for 7.3% of all 82 cases. None
died. Conclusions: (1) FC had good prognoses, but nearly
half the cases had recurrences. Most of these relapsed within
1 year of onset. (2) Both the number of recurrences of FC
and the interval between onset and recurrence were risk
factors for secondary EP.
FP-H-076
Effect of immunoglobulin G and phenytoin (PHT) on
NMDA receptor-1 density in the brain of
pentylenetrazol (PTZ) induced convulsion rats
C.-N. Zheng a , Z.-S. Zhou a , M. Ariizumi a,b , M. Jinnai b , K.-D.
Wang a
a Pediatric department, China-Japan Friendship Hospital,
Beijing, Republic of China; b Department of Pathology,
Koshigaya Hospital, Dokkyo University School of Medicine,
Japan
Objective: To clarify the anti-epileptic mechanism of
IgG, we studied the effect of IVIG on density of N-
methyl-d-aspartate receptor (NR-1) positive neural cells
in the brain of rats with PTZ induced convulsion,
compared with that of phenytoin (PHT). Subjects and
methods: Wistar rats were divided into four groups:
group A, normal control; group B, PTZ 1 saline; group
C, PTZ 1 IVIG; group D, PTZ 1 PHT. PTZ 50 mg/kg
was intraperitoneally injected every day. IVIG 200 mg/
kg and saline were each intravenously injected twice a
week, while PHT 6–10 mg/kg was given orally every
day 2 h before seizure onset. The brains were removed
on the 15th day and NR-1 detected by immunohistochemical
staining. NR-1 positive and negative neural cells were
counted in the CA1 and CA3 of hippocampus, dentate
gyrus, frontal cortex and cerebellum. Results: In group
B, NR-1 positive neural cells were significantly reduced
in CA1 and cerebellum, and the total number of neural
cells tended to decrease in CA3. Group C showed moderate
reduction of NR-1 positive neural cells in CA1, but
not in the other regions. Group D had only minimal
NR1 neural cell reduction in the cerebellum. Conclusion:
These results suggested that the anti-epileptic action of
IgG is possibly related to NR-1 mRNA expression
or NMDA activation through immunomodulation. IgG
might also possess a protective action against neuron
loss due to severe convulsions, comparable to that of
PHT.
FP-H-077
Study on frequency – amplitude gradient in pediatric
sleep EEGs
Z.-C. Guo, F.-C. Cai
Children’s Hospital, Chongqing University of Medical
Sciences, China
Objective: To explore the clinical significance of the
frequency-amplitude gradient (FAG) in pediatric sleep
EEGs. Method: The patients and normal children were
divided into corresponding five groups in accordance
with age (,1, ,3, ,5, ,10 and ,14 years as groups I–
V). Simultaneous EEGs and brain electric activity mapping
were performed during waking and sleep states. The
energy ratio of slow waves between parietal-occipital and
frontal prefrontal areas was calculated as FAG. The timeamplitude
of EEG was converted into frequency-energy
relation. Results: Thirty-one normal children and 43
patients with encephalopathy joined the study. The mean
values of FAG in normal children of groups I–III were
higher than 1.0 and were interpreted as normal FAG. The
mean values of FAG in patients of groups I–III were lower
than 1.0 and were interpreted as absence of FAG. significant
differences were found between those of the normal
children groups and the patient groups (Ps , 0:05 , 0:01).
The mean values of FAG in group IV normal children
group were not different from that in the patients’ groups
(Ps . 0:05). The mean values of FAG in group V normal
children were lower than 1.0. No significant difference was
found between the corresponding normal children group
and the patients’ group (P . 0:05). Conclusions: The
EEG pattern of FAG was well-developed in normal children
at 4 months of age, with a progressive decrement till
10 years old and absence of FAG when approaching 14
years. Neurological illness may be associated with loss of
FAG. The FAG examination was a valuable parameter in
distinguishing normal sleep EEG from encephalopathic
illness.

516
Abstracts
FP-H-078
Clinical and experimental study of prophylactic
management for febrile seizures with topiramate
Z. Huang, F.-C. Chai, X.-P. Zhang, W.-Z. Zhou
Department of Neurology, Children’s Hospital, Chongqing
University of Medical Sciences, China
Objective: To explore the effect, values and side effects of
TPM in the prevention of complex febrile convulsions
(CFC) in animals and children. Methods: (1) Thirty Wister
rats aged from 10 to 30 days, sensitive to FC were selected
randomly, and observed for changes of liability to FC after
taking TPM. (2) Ninety children diagnosed with CFC were
selected randomly. There were 49 males and 41 females.
The patients were 8.9–110.1 months old (average
52.7 ^ 28.7 months). The durations of disease were 2.7–
69.7 months (average time of 25.3 ^ 22.2 months). The
dosages of TPM were 3–6 mg/kg per day (average
50.7 ^ 19.6 mg per day). Observation time was 6.5–18.9
months (average 50.17 ^ 19.6 months). (3) To observe the
side effects on Wister rats and children after taking TPM.
Results: (1) The duration of CFC seizures was significantly
different (t ¼ 48:15, P , 0:01) after taking TPM (respectively
251.27 ^ 246.98 and 96.43 ^ 92.41 s). The incidence
of the CFC seizures declined by about 80% (24/30,
P , 0:01) and the severity of seizures was noticeably
reduced (x 2 ¼ 24:093, P , 0:01). (2) The seizure frequency
of 90 children’s CFC was reduced from 6.5 ^ 4.8 times/
year to 0.38 ^ 0.82 times/year: the difference was significant
(t ¼ 7:23, P , 0:01). Eighty-two of 90 cases had no
CFC seizure during the observation time (91.11%). (3) A
total of 36.67% (11/30) of Wister rats and 11.1% (10/90) of
CFC children had side effects such as narcolepsy, loss of
appetite, and loss of weight. The difference was very significant
(x 2 ¼ 6:635, P , 0:01). However, their routine blood
tests and liver and kidney function had not changed. Conclusion:
The results suggested that TPM had values such as
high clinical effect and infrequent side effects, as confirmed
by animal experiments. So, TPM had good clinical value. In
one word, we thought TPM, as a single drug, had a promising
future in the prevention of CFC in children.
FP-H-079
Effect of high temperature on electrical seizure-induced
neuronal damage in explant culture of rat hippocampus
S.-A. Chae a , D.-W. Kim b
a Chung-Ang University, Seoul, Korea; b Inje University,
Koyang, Korea
The immature brain has a greater susceptibility to the
development of prolonged provoked seizures in response
to environmental stimuli such as fever. Therefore, the influences
of high temperature (HT) on normal and epileptic
brain were studied in organotypic explant cultures of rat
hippocampus. Fourteen days-in-vitro cultures from 8 dayold
rat pups were perfused with CSF bubbled with 95/5%
O 2 /CO 2 in a microchamber. At 368C initially, after
discharge (AD) was evoked. In the HT group, the cultures
were subjected to 398C for a period of 8 min before the
second stimulus train was applied. They were then restored
to 368C for 10 min. In the control group, temperature was
maintained at 368C for the second stimulus train. The
cultures were returned to the incubator and observed serially
for neuronal damage. The cultures on the same insert that
were not stimulated served as the unstimulated groups.
There was no significant difference in neuronal damage
between the unstimulated groups. Neuronal damage on
AD induced cultures during the febrile setting (n ¼ 16)
was significantly higher than in the non-febrile setting
(n ¼ 16). In CA1, percentage damage (mean ^ SEM) was
6.63 ^ 2.56; 0.92 ^ 0.45 at 24 h (HT group; control group;
P ¼ 0:036, Student’s t-test); 26.37 ^ 7.44; 4.99 ^ 2.23 at
48 h (P ¼ 0:010); and 38.59 ^ 9.63; 6.48 ^ 2.30 at 72 h
(P ¼ 0:003). In CA3, percentage damage was
27.86 ^ 8.68; 7.54 ^ 3.74 at 72 h (P ¼ 0:04). The results
suggest that at high temperatures, seizures in epileptic brain
may be more injurious than seizures at normal temperatures.
FP-H-080
A genome-wide linkage analysis of febrile seizures in a
Japanese affected sib-pair population
J. Nakayama a,b , N. Yamamoto a , K. Hamano c , N. Iwasaki b ,
S. Nakahara d , M. Ohta e , Y. Horigome b , A. Matsui b ,T.
Arinami a
a Department of Medical Genetics; b Department of Pediatrics,
University of Tsukuba,
c Kitaibaraki Municipal
General Hospital,
d Kensei General Hospital,
e Toride
Kyodo General Hospital, Ibaraki, Japan
FS are the most common form of childhood seizures, and
they occur in about 7% of Japanese children. Four putative
FS loci, FEB1 (chromosome 8q13–q21), FEB2(chromosome
19p), FEB3 (chromosome 2q23–q24) and FEB4
(chromosome 5q14–q15), have been mapped. Furthermore,
some mutations in the voltage-gated sodium channel b1
subunit gene (SCN1B), the neuronal voltage-gated sodium
channel gene (SCN1A) and the GABA A receptor b2-subunit
gene (GABRG2) were identified in families with a clinical
subset of seizures termed GEFS 1 . Except for FEB4, these
loci are linked to some large, Caucasian families. In this
study, we conducted a genome-wide linkage screening of
48 Japanese nuclear families (192 members) with FS children
(59 affected sib-pairs) using 400 microsatellite markers
separated by an average distance of 10 cM. We used the
GENEHUNTER program to perform nonparametric multipoint
linkage analysis. In addition to FEB4, which we have
previously reported (Hum Mol Genet 2000;9:87–91),
another peak with a non-parametric linkage score (NPL)
.2.0 was observed on chromosome 1 (NPL score ¼ 2.54).

Abstracts 517
Taken together with the report by Baulac et al. who found
evidence for linkage to the near region on chromosome 1q
in a large family with both FS and temporal lobe epilepsy
(Ann Neurol 2001;49:786–792), our data further support the
existence of FS susceptible gene(s) on chromosome 1q.
FP-H-081
Convulsions in children with different types of
helminthiases
Sh.Sh. Shamansurov, Sh.Kh. Saidasisova
Tashkent Institute of Postgraduate Medical Education,
Uzbekistan
Background: The problem of helminthiases and their
effect on the state of nervous system remain actual and
important for today. Helminths creating a stable focus of
stimulation in the central nervous system may lead to the
appearance of convulsions in children. We have investigated
45 children, aged 1–15, with convulsive paroxysms
and invasions by helminths. Lambliasis, dwarf tapeworm,
broad tapeworm and ascarides were found. Mixed invasions
were noted in 92% of cases. Results: In additional to anticonvulsive
therapy dehelminthization was performed.
Convulsions stopped immediately after dehelminthization
in 39% of cases; stopped gradually during 1–3 months in
43% of cases, and became more frequent and stopped with
time in 11% of cases (6 months after finishing of treatment
and repeated dehelminthization). They did not stop and
required prolonged anticonvulsive therapy in 7%. Conclusion:
Thus, during treatment with anticonvulsants, of
convulsive states in children, it is necessary to take into
account the possibility of invasion of helminths into the
children’s bodies.
FP-H-082
Application of a new method of auto-antibodies
detection in children with convulsive state
Sh.Sh. Shamansurov, D.N Gulyaniova
Tashkent Institute of Postgraduate Medical Education,
Uzbekistan
Aim: Application of a new ‘Epitest’ method of detection
of the level of auto-antibodies to fragments of glutamate
receptors, quisqualate membrane protein in blood serum
of children with paroxysmal states. Methods: Forty-two
children were studied, at ages from 3 months to 3 years.
Fifteen of them had non-febrile fits and 17 of them had
febrile fits: and, for comparison, ten of them were healthy
children of the same age. They were examined with ‘Epitest’,
in order to find out whether there were glutamate receptors
in blood. Results: The level of auto-antibodies to
glutamate receptors in healthy children was low:
1.01 1 0.08 ng/ml. Children with non-febrile epilepsy had
elevated levels of receptors in blood: 2.66 ^ 0.13 ng/ml.
But in children with febrile fits it was 1.855 ^ 0.205 ng/
ml. So, by this method the following was investigated: the
increases in the levels of auto-antibodies to glutamate receptor
of AMPA in children with convulsive states compared
with healthy children. In studies of children with epileptic
syndromes and paroxysmal states of non-epileptic origin
and their outcomes, there is, undoubtedly, confirmation of
a hypothesis of damage to glutamate receptors secondary to
high glutamate concentrations in pathological processes and
also intensive production of auto-antibody receptors which
are initiators of a rise in convulsive reactions. Conclusions:
A new method of detection of auto-antibodies to AMPA
receptors relating to cerebral pathology was found very
effective in the clinical diagnosis of convulsive syndromes
in children. It could be used widely to reveal and prevent
paroxysmal states in their early stages.
FP-H-083
The relation of epilepsy related factors to anxiety and
depression scores in epileptic children
A. Oguz, S. Kurul, E. Dirik
Dokuz Eylül University Faculty of Medicine, Izmir, Turkey
Cognitive and behavioral impairments are found more
often among epileptic children than among their peers. In
this study, we aimed to evaluate the anxiety and depression
in epileptic children, to compare their results with that of a
healthy control group and to determine the relation of anxiety
and depression scores to epilepsy-related factors. The
State Trait Anxiety Inventory and the Children Depression
Inventory were applied to 35 epilepsy patients aged 9–18
years (mean age 12.9 ^ 2.52) and to 35 healthy children
served as the control group. Both study and control groups
were divided into two age groups (9–11 and 12–18 years) to
exclude the effect of puberty on anxiety and depression
scores. Significant depression and suicidal ideas were determined
in the study group. The mean trait anxiety score was
significantly higher in the 9–11 age group of epileptic
patients than the corresponding control group
(35.90 ^ 6.90 and 29.33 ^ 2.84, P , 0:05). The mean
state anxiety score (33.90 ^ 3.90 and 30.40 ^ 6.02,
P , 0:05), trait anxiety score (38.20 ^ 6.84 and
32.20 ^ 3.90, P , 0:05) and depression score
(16.65 ^ 8.32 and 8.15 ^ 3.15, P , 0:05) were significantly
higher in the 12–18 age group of epileptic children
than in the control group. Among the epilepsy-related
factors, epilepsy duration, seizure frequency and polytherapy
were found to increase the anxiety and depression; and
age of seizure onset, seizure type and EEG findings were not
related to anxiety and depression. In conclusion, symptoms
of anxiety and depression are common among epileptic
children, especially during puberty. State Trait Anxiety
Inventory (STAI) and Children Depression Inventory
(CDI) may be used as tools to give information to the clinicians.

518
Abstracts
FP-H-084
Magnetoencephalographic statistical parametric
mapping of interictal spikes in epileptic patients
K. Imai a , K. Yanagihara a , N. Kamio a , R. Sakakibara a ,K.
Shimono a , T. Okinaga a , N. Hirabuki b , T. Yoshimine c ,K.
Ozono a
a Department of Pediatrics,
b Radiology,
c Neurosurgery,
Osaka University Graduate School of Medicine, Osaka,
Japan
Purpose: To evaluate a new MEG imaging with spatial
filtering and statistical analysis to investigate spatial distribution
of interictal spikes. Method: Ten cases of localization-related
epilepsy were investigated. Thirty interictal
spikes with similar morphology and distribution were
recorded from each subject using 64 channel whole-head
MEG system (CTF; Canada). The following two methods
were used for analysis: (a) single dipole model (SDM); and
(b) statistical-SAM (synthetic aperture magnetometry, CTF;
Canada) (stat-SAM). In stat-SAM, the region of interest was
set to include the entire cerebrum with a 5 mm voxel resolution.
The signal power of each voxel was estimated using
spatial filter (SAM). Changes in the beta band signal power
between the spike phase (200 ms) and the prespike phase
(200 ms) of each voxel were statistically analyzed using
Student’s t-test. The distribution of t-values obtained from
each voxel was superimposed on individual MRI images.
Results: Using the stat-SAM, the position and spatial distribution
of interictal spikes could be superimposed on MRI
images in each case. These results correlated well with
SDM. In subjects with localized cerebral lesions, stat-
SAM revealed the precise information of the spatial relationship
between epileptic foci and brain lesions. Conclusion:
While SDM is useful and reliable for the analysis of
interictal spikes generally, complicated results may lead to
difficulties with interpretation. Stat-SAM can easily demonstrate
the 3-dimensional distribution of interictal spikes,
although poor in temporal resolution. Furthermore, this
new statistical method may enable us to make the automatic
MEG distribution images of interictal spikes.
FP-H-085
The therapeutic effect of topiramate in childhood
epilepsy: a hospital-based study
W.-T. Lee, J.-S. Liang, T.-W. Yu, Y.-Z. Shen
National Taiwan University Hospital, Taipei, Taiwan,
Chinese Taipei
Topiramate has been shown to be safe and effective in
refractory epilepsy in adults. Pharmacokinetic studies show
that the clearance of topiramate is greater in children than
in adults; therefore, higher doses may be needed in children
than adults. In the present study, we investigate the
effect of topiramate used adjunctively in Taiwanese children
with refractory partial and generalized epilepsy. A
total of 51 children (F:M ¼ 29:22) with epilepsy were
enrolled in the present study. The mean age was 9.5
years, and the mean seizure history was 5.6 years. Of
these 51 children, 68% of children took two or more anticonvulsants
before topiramate was added. Our result showed
that the average seizure reduction rate was 59% after
adding topiramate, and the mean follow-up duration was
8.2 months. Overall 22% of the children became seizurefree,
and another 42% showed more than 50% of seizure
reduction after adding topiramate. Of ten children with
adverse effects, four of them dropped out finally secondary
to adverse effects. Of five children with infantile spasms,
two became seizure free and two showed seizure reductions
of more than 50%. We conclude that topiramate is an
effective agent for the adjunctive therapy of partial and
generalised epilepsy in children. Treatment-limiting
adverse events may be managed by slow titration.
Although comparative studies with the other newer anticonvulsants
are required, topiramate may be an important
addition to the range of drugs that can be used to treat
refractory epilepsy in children.
FP-H-086
Electroencephalographic-video monitoring in
pediatrics: Philippine Children’s Medical Center
experience
R.G. Valeriano, M.H. Ortiz, A.S. Salonga, M.F.A. Soto
Philippine Children’s Medical Center, Quezon City, Philippines
EEG-video monitoring is a valuable tool in evaluation
and management of children suspected of having seizures
or paroxysmal disorders. The prolonged monitoring of the
brain activity simultaneous with prolonged video monitoring
of patients permits correlation of clinical behavior and
EEG activity. Its primary purpose, especially in pediatrics,
is to address fundamental problems of evaluation and
management of children suspected of having seizures. At
Philippine Children’s Medical Center we present our
experience in video-EEG. Since 1998, we have a total of
70 patients who underwent prolonged EEG monitoring.
The youngest patient was 2 months and the oldest, 19
years old: the average age was 4.61 years. The majority
were males 44/70 (63%), with females 26/70 (37%). The
commonest reason, in 59/70 (82%), for prolonged video-
EEG monitoring was to determine if an abnormal movement
noted in a patient had an EEG correlate. Other
reasons were, to determine if there were multiple seizure
types, 3/70 (4%); to localize the area from which epileptiform
discharges were coming 2/70 (2.8%); to determine
the seizure type 2/70 (2.8%). One out of 70 patients underwent
monitoring to confirm whether the seizures were
infantile spasms, to confirm whether there was frontal
lobe epilepsy, to confirm the findings in routine EEGs in

Abstracts 519
patients who did not have clinical seizures and to confirm
whether other clinical findings such as headaches, flashes
of lights, and sudden generalized weakness are actually
seizures. The abnormal movements requiring confirmation
were mostly myoclonic jerks, stiffening of extremities,
blank stares, head turning, tremors of the hands and
shoulders, cyanosis and sudden transient weakness. In
our experience video-EEG monitoring confirms our diagnosis
of epilepsy and helps clinicians in decisions on
giving antiepileptic drugs and eliminating these drugs if
they are not necessary. We have determined different
seizure types, and we have localized the areas from
which the discharges were coming. Prolonged EEG-video
monitoring, in our experience, has helped us in the diagnosis
and treatment of our patients.
FP-H-087
Improvement in the knowledge, attitude and practice
regarding epilepsy among participants of the Epilepsy
Camp conducted in Philippine Children’s Medical
Center
R.G. Valeriano, M.L. Bolanos, M. Sosa, M.H. Ortiz
Philippine Children’s Medical Center, Quezon City, Philippines
Epilepsy Camp is a yearly activity of the Philippine
Children’s Medical Center which aims to improve the
awareness of patients with epilepsy, their parents and caregivers.
This pilot study aims to evaluate the effectiveness
of the camp in attaining the objective of the annual activity.
We would like also to assess the knowledge, attitude
and practice of participants before the Camp and determine
the areas of weakness and use these to address their needs
in the future epilepsy camp. Methods: We administered
pre-tests and post-tests to the participants of the year
2001 Epilepsy Camp. A 60-item test questionnaire, written
in vernacular language was prepared. Questions about
knowledge on etiology, diagnosis, and treatment, attitude
towards the disease, dealing with people with the disease
and common practice regarding emergency management
during active seizures, importance of clinic follow-up and
compliance in giving of medications. The same set of questions
was prepared for the pre-test and post-test. Results:
Sixty-two respondents were able to answer both pre- and
post-tests. In the pre-test, 98% of the respondents are aware
that epilepsy is caused by sudden changes in brain activity.
More than half still believe in the myths and misconceptions
about epilepsy. Sixty percent are aware that etiology
can be undetermined. Ninety percent know about electroencephalography
as a diagnostic procedure. Eighty two
percent believed that persons with epilepsy can live normal
lives. Less than 50% are aware of the emergency measures
during active seizures. Although 70% believe in the importance
of follow up, the majority has doubts about giving
maintenance medication. There is a statistically significant
difference between the results of the pre- and post-tests
with improvement in the results. Conclusion: The Epilepsy
Camp is effective in improving the knowledge, attitude and
practice among participants.
FP-H-088
Seizure recurrence after antiepileptic drug (AED)
discontinuation in children – the Philippine Children’s
Medical Center experience
J.M.B. Ahorro, M.H. Ortiz, A.M. Salonga
Philippine Children’s Medical Center, Quezon City, Philippines
The last decade has seen striking changes in epilepsy
management in children. Identifying seizure etiology and
semiology has significantly helped in the management of
these cases. In all patients with epilepsy, the estimated probability
of achieving seizure remission is 65%. Reported
relapse rates from foreign studies following AED discontinuation
varied from 6 to 76% in general and 6–40% in
children; risk factors that were shown to have significant
correlation to recurrence were age at onset of seizure,
seizure type, and presence of slowing on EEG. This retrospective
study was conducted to determine the rate of
seizure recurrence following AED discontinuation and identify
risk factors predictive of outcome. A total of 30 records
of patients whose anticonvulsants were discontinued were
available for review. The overall relapse rate of seizures in
those patients whose AEDs were discontinued after a minimum
seizure-free period of 2 years, as well as the risk
factors for seizure relapse in these patients, will be
discussed.
FP-H-089
Anticardiolipin antibody and childhood epilepsy
K. Haginoya, H. Yokoyama, M. Munakata, M. Ishitobi, T.
Kitamura, M. Koda, N. Togashi, C. Nara, K. Iinuma
Tohoku University School of Medicine, Sendai, Japan
Background: Anticardiolipin antibody (aCL) is reported
to be positive in patients with antiphospholipid syndrome.
However, aCL has been reported in patients with various
CNS disorders. We examined the frequency of aCL in
patients with various kind of epilepsy in order to clarify
whether aCL is involved in the pathogenesis of childhood
epilepsy. Methods: Serum samples from 175 epileptic
patients and 71 non-epileptic patients were obtained after
informed consent. The aCL IgG was measured using an
ELISA kit purchased from MBL (Nagoya, Japan). The
175 epileptic patients included nine with idiopathic generalized
epilepsy, 46 with symptomatic generalized epilepsy
(SGE) including 21 with West syndrome, 73 with cryptogenic
or symptomatic localization-related epilepsy (C/
SLE), 29 with idiopathic localization-related epilepsy,

520
Abstracts
and 18 with unclassified epilepsy. Results: Twenty-four
percent of epileptic patients and 15% of non-epileptic
patients were positive for aCL. When epileptic patients
were divided into aCL-positive (42 patients) and aCLnegative
(133 patients) groups, there were no significant
differences between the two groups in terms of sex, polypharmacy,
antinuclear antibody, antiDNA antibody, and
drugs used for treatment. However, SGE and age less
than 1 year were significantly more frequent in the aCLpositive
group (P ¼ 0:014 and ,0.0001, respectively).
Patients whose seizure type was epileptic spasm including
West syndrome were significantly more frequent in the
aCL-positive group (P ¼ 0:0001). Conclusions: There
was a significant relationship between aCL and those
who had SGE and whose seizure type was epileptic
spasm, suggesting that aCL was involved in the pathogenesis
of epilepsy in those patients.
FP-H-090
Epilepsy after febrile seizures
M. Kuturec, F. Duma, V. Sabolic-Avramovska, O.
Lekovska
University Children’s Hospital, Skopje, Macedonia
Data for subsequent epilepsy after febrile seizures varies
form 2 to 12%, depending on the different possible risk
factors: atypical febrile seizures, positive family history of
epilepsy, presence of neurodevelopmental abnormalities
prior to febrile seizures, etc. During the period of 2
years 706 patients were registered with febrile seizures
(401 with clinical features of simple febrile seizure –
group P, and 305 with clinical features of complex febrile
seizures – group C). The longitudinal follow up was done
during a period of 3–6 years for: 327/401 and 216/305.
The follow up (every 3 months the 1st year, and then every
6 months) contains data for: febrile illness, recurrent
febrile convulsions, development of a febrile seizures,
neurodevelopmental status, EEG, AED, etc. An epilepsy
developed after simple febrile seizures in 17, 5.20%. An
epilepsy developed after complex febrile seizures in 27,
12.5%. In both groups the main transformation occurred
within 36 months of an initial or last febrile convulsion,
accounting for 10 (58.82%) of children in group P, and 14
(51.85%) in group C. The causal relationship between
febrile seizures and the subsequent epilepsy is still controversial,
and depends on many different risk factors. In our
study the connection between the febrile seizures and
subsequent epilepsy was more definite and more robust
for group C, with even one positive factor, such as the
clinical feature of an atypical or complex febrile seizure,
the mental, motor or neurological deficit prior to first
febrile seizure, and the initial febrile seizure after 3rd
year, being of importance.
FP-H-091
EEG biofeedback in the treatment of attention deficit
hyperactivity disorder in children
N. Pop-Jordanova a , S. Markovska b
a Department of Psychophysiology, Pediatric Clinic, Faculty
of Medicine, b Research Center for Energy, Informatics and
Materials, Macedonian Academy of Sciences and Arts,
Skopje, Macedonia
In the school age population, ADHD has been diagnosed
worldwide in from two to even ten percent of children.
Precise evidence for Macedonia is lacking and the only
treatment applied so far has been pharmacotherapy, mainly
with Ritalin, which has to be purchased from abroad. The
subjects consisted of 12 children aged 6–13, categorized
into three types of ADHD: predominantly ADD, hyperactive-impulsive,
and, combined type. The diagnosis is made
clinically (ICD-10) and confirmed by psychometric instruments
such as Conners Questionnaire for parents and
teachers, as well as WISC-R. The children participated in
a 4-month program of EEG biofeedback (neurofeedback)
training, twice a week, with sessions of 50 min, using
Biograph/Procomp version 2.0. The aim of neurofeedback
training was beta (16–20 Hz) enhancement and theta (4–8
Hz) suppression. All patients showed considerable changes
in EEG activity. The mean values of theta amplitudes at the
end of treatment were 20–30% lower compared to the initial
states, whilst the simultaneous increase in beta amplitudes
was over 30%. In the cases of two patients with extremely
low concentrations, where neurofeedback was combined
with Ritalin, the increase of beta amplitudes was the highest,
reaching 100%. However, such a synergetic effect was not
observed in theta amplitudes. The comparison of IQ performances
(by WISC-R) before and after the neurofeedback
training program has indicated higher intelligence test
scores. Also, enhanced behavior and learning, improvement
in school grades and increased self-esteem have been
achieved.
FP-H-092
Staring episodes in generalized and focal epilepsies
P.S. Baxter a , A.K. Chattopadhyay b , R.H Kandler b
a Children’s Hospital, Neurophysiology Department, b Royal
Hallamshire Hospital, Sheffield, UK
Staring or ‘blank’ episodes can be due to epileptic
seizures, attentional difficulties, daydreaming, tiredness or
other causes. This can cause diagnostic confusion. We
examined the clinical features of epileptic staring episodes,
by reviewing videos of children having seizures during EEG
recordings. A total of 185 staring episodes were studied in
74 children aged 7 months–15 years (median 7 years). Eighteen
(10%) were isolated stares. A total of 154 episodes had
additional features such as gaze deviation, eyelid flutter,

Abstracts 521
and/or automatisms. During seven the eyes remained closed
without additional features. Six could not be assessed.
Isolated stares occurred significantly more often in children
whose eyes were open rather than closed at seizure onset
(P , 0:001). The 18 isolated stares were seen in 12 children,
ten of whom had other ictal episodes with additional
features during the same recording and two of whom only
had a single seizure recorded. Seizure types were primary
generalised with 3 Hz spike and wave (37), other primary
generalised (89), 3 Hz spike and wave with a focal onset
(13), other generalised with a focal onset (27), focal (7), and
unclassified (12). Isolated stares were as common in those
with focal or generalized onsets. In conclusion, staring
episodes without additional features are not typical of
epileptic seizures. The likelihood of recording an isolated
stare of the habitual type is improved by performing the
video-EEG with eyes open.
FP-H-093
Correlation of hemiplegic or hemiparetic CP and later
development of partial epilepsy
M. Carignani
Neurology Institute, Universidad Nacional de Rosario,
Rosario, Argentina
The CP, a static, residual, pathology secondary, generally,
to hypoxic-ischemic events, seen most commonly as an
expression of variable patency, previously or more recently,
of the basal branches of the cerebral arteries (anterolaterals
or fluted branches) or of the terminal veins, of the internal
cerebrum and the choroid plexus, with subsequent infarction
of the subependymal germinal matrix, is one of the pathologies
formerly studied in pediatric neurology and defined
according to location, affected vessels, lesional sequence
during its development, etiology, and, more recently by
the presence of dysplasias of the cortical mantle due to
genetic factors and/or disturbances of neuronal migration.
It is the intention of the author to consider the hemiparetic
and hemiplegic forms, and their risks for development of
partial epilepsy, the latter’s evolution and factors that can
indicate refractoriness and consequent indications for
surgery. The study is prospective and includes 11 patients
between 8 months and 14 years of age with static unilateral
neurological deficits, all without previous treatment, who
were taken care of between 1 and 14 days after the first
seizure. The group was divided in an arbitrary way,
randomly. The majority were not treated with AED and
the subsequent risk for recurrence was greater than in the
group treated immediately after the first seizure. The cumulative
risk according to delay in the start of treatment was:
60% had repetition of seizures within 36 months. The treated
population was statistically significantly different for the
recurrence risk, after setting down parameters such as 1 and
2 months free of seizures. Of the patients who received
immediate treatment, 85% had a year free of attacks and
67% had repeated seizures 2 years later. Of the group with
delayed treatment, (percentage in which seizures are
increased based on the duration from 18 to the beginning
of therapy) those 46% free of attacks are divided equally
between the 1st year, and 33% both 1os. years. Only 25% of
the first group of patients had almost complete or complete
remission of the attacks. That number rose to 56% in the
patients treated initially. The rest had little or no response to
drugs, with increases, additions or changes of these, and
continued to have partial seizures, thus presenting themselves
as candidates for the surgery. In hemiparetic or hemiplegic
C.P., therapy with anticonvulsant drugs is
recommended prior to the appearance of seizures.
FP-H-094
Lamotrigine vs levetiracetam in treatment of JME,
newly diagnoses epilepsy
M. Carignani
Neurology Institute, Universidad Nacional de Rosario,
Rosario, Argentina
We tried 19 patients with lamotrigine (200–400 mg/day),
who had a diagnosis of JME, versus another group of 16
patients with same pathology on levetiracetam monotherapy
(1000–2000 mg/day). It is well known that levetiracetam’s
mechanism of action is still unclear, apart from the anticonvulsant
effect on rats treated with PTZ or electroshock
seizures. Adverse effects were most frequently dizziness,
often headaches, asthenia and somnolence; also renal and
hepatic functions must be carefully checked. Some cases
had increases in, or developed myoclonus, with eventual
aggravation of seizures. Lamotrigine is a relatively new
AED, with a structure and a pharmacological profile similar
to that phenytoin and carbamazepine. As a triazine derivative,
it is effective in partial and generalized seizures. The
best-studied mechanism of action of lamotrigine is its inhibitory
effects on voltage-sensitive sodium and calcium
channels, which occur in use and time dependent manners.
The most dangerous effect of the drug is a skin reaction
which can be of variable severity, sometimes with associated
multiorgan failure and death; it can be avoided with
very slow titration, reduction of drug dosage when the rash
appears, followed by further slower increases, or withdrawal.
The groups were between 12 and 19 years old. Both
had normal MRI studies, typical clinical profiles of JME,
and normal evaluated results with interictal HMPAO-
SPECT, interictal EEG and factor analysis of creatine or
creatinine ratios. We also checked the statistical analysis
with the Epilepsy Outcome Scale (EOS). We analyzed
thalamic volume, finding reduced concentration of NAA
in the prefrontal cortex, and low values in the hypothalamus.
We found better results in clinical trials with LTG in terms
of reduced myoclonus in the mornings, abolished GCT
seizure rates, less sleep and psychiatric disorders and better
cognitive, and behavioral performances. We also found

522
Abstracts
better mental condition with this drug. Also migraine,
frequently co-morbid with JME, improved in patients treated
with LTG. Pregnant patients had safe outcomes with
both AEDs. Assays with spectroscopy images and neuropsychological
tests showed improvement: the tests after treatment
started were slightly better with lamotrigine.
Monitoring of drug therapeutic and toxicokinetic effects
on larger number of patients is needed to establish more
reliable conclusions. Meanwhile, there is a lack of wider
statistical data.
FP-H-095
Pyridoxal phosphate should replace pyridoxine for
intractable childhood epilepsy
H.-S. Wang a , S.-C. Huang a , M.-F. Kuo b
a Division of Pediatric Neurology, Chang Gung Children’s
Hospital, and Medical College of Chang Gung University,
Taoyuan; b Division of (Pediatric) Neurosurgey, Department
of Surgery, National Taiwan University Hospital,
Taipei, Chinese Taipei
We have reported a child whose intractable seizures were
controlled with pyridoxal phosphate (PLP), the active form
of pyridoxine, instead of pyridoxine per se. This study is to
compare the efficacy of PLP with pyridoxine in the treatment
of children with intractable epilepsy. Ninety-four children,
aged from 2 months to 15 years, with idiopathic
intractable epilepsy for more than 6 months were enrolled
in the study. Under EEG monitoring, in addition to the
previous antiepileptic medicine, intravenous PLP of 10
mg/Kg was infused first, then 40 mg/Kg of PLP was
added if there was no significant improvement. For those
patients whose seizures could be controlled by PLP infusion,
PLP was then replaced by oral pyridoxine at the same
dose. If a seizure recurred, parenteral PLP was infused again
to stop it and subsequently oral PLP 50 mg/kg per day was
used. If no seizure recurred in a week, the previous antiepileptic
regimen was gradually tapered. Eleven of the 94
(12%) pediatric patients with idiopathic intractable epilepsy
responded to PLP dramatically. Five of the 11 patients were
free of their previous antiepileptic medicine within 6
months of the treatment and were followed for an average
period of 11 months. Three of them were controlled with
PLP and the remaining two were treated with pyridoxine.
The other six patients responsive to PLP therapy still need
one to three kinds of antiepileptics to control their seizures.
Three of them were treated with PLP and the other three
with pyridoxine. In these 11 patients responsive to the initial
PLP therapy, six of them failed to be treated with pyridoxine
and needed PLP for seizure control. The results show that
PLP is effective in 12% of patients with idiopathic intractable
epilepsy. PLP can replace pyridoxine satisfactorily, but
not vice versa. The value of vitamin B 6 in the treatment of
epilepsy is well known. In this study, it is demonstrated that
the responsive rate to PLP is higher than pyridoxine (12
versus 5%). It seemed that infantile spasms were more
affected by vitamin B 6 than other epilepsy types. The
authors suggest that intravenous infusion of PLP, instead
of pyridoxine, should be tried first when patients with
intractable seizures, especially children with seizure onset
under the age of 15 months, come for treatment. For the
patients who fail to respond to pyridoxine treatment, PLP
should not be abandoned.
FP-H-096
Febrile seizures in a referral hospital
A. Charuvanij, N. Hoonsawad
Department of Pediatrics, Bhumibol Adulyadej Hospital,
Bangkok, Thailand
A prospective study of 149 first episode febrile seizure
patients admitted to the pediatric wards of Bhumibol
Adulyadej hospital between March 1997 and February
1998 was performed to determine the epidemiology and
seizure recurrences. The male to female ratio was 1.22:1.
The mean age was 17.15 months. The average body
temperature when first seen was 39.158C. The mean duration
of fever before arrival at the hospital was 20.45 h. The
cause of fever was mostly upper respiratory tract infection
(71.8%). Fourteen percent and 0.7% of the patients had
histories of febrile seizures and epilepsy, respectively, in
first-degree relatives. Ninety one percent had simple febrile
seizures. The average income of the parents was 8004.61
bath per month. Eighty five percent of the patients were
followed up periodically up to at least 6 months. Seizures
recurred in 15 patients (11.81%) and most (14/15) had the
episodes within 6 months after the first attack. A positive
family history of febrile seizures was the only risk factor
which gave significant differences (P ¼ 0:006).
FP-H-097
Electrical status epilepticus during sleep (ESES): clinical
follow-up and EEG changes during the course of
treatment in 14 cases
M. Topcu, D. Yalnizoglu, G. Turanli, A. Degerliyurt, D.
Genc-Acikgoz, E. Erdogan
Hacettepe University Children’s Hospital, Ankara, Turkey
ESES consists of sleep-induced paroxysmal EEG activity,
lasting for months or years, which may appear continuously
or discontinuously during sleep and is usually diffused
bilaterally. The epilepsy is relatively easily controlled.
However the prognosis is complicated by cognitive and
behavioral issues. Fourteen patients, aged between 7 and
18 years of age, were studied. The male/female ratio was
10/4. Age of onset of epilepsy ranged between 11 months
and 14 years. Age of onset of ESES ranged between 3 and
15 years; duration of ESES ranged between 2 months and 7
years. MRI was abnormal in six of 14 patients. All patients

Abstracts 523
showed attention and behaviour problems, one had autistic
features. Three patients had mental retardation, two reported
low school performance. Conventional AEDs including
CBZ, valproate, ethosuximide, clonazepam, phenytoin,
phenobarbital, primidone were tried in different combinations.
Pridoxine was tried in two patients and ACTH in one.
New generation AEDs vigabatrin, lamotrigine, oxcarbazepine,
clobazam and topiramate, were used as add-on medications
in nine patients. EEG showed focal discharges
which were concentrated over the frontal, occipital or
centrotemporal regions in 8/14 patients; 6/8 were on topiramate
or clobazam or both. Behavioral and cognitive
problems improved along with the improvement of the
EEG. New generation AEDs could be promising options
in the treatment of ESES, sparing the patients from the
side effects of steroids. Focal features in EEG might be
transitory findings during the normalization of the EEG.
Topiramate and clobazam may help decrease the duration
of ESES. Therefore further studies are required to determine
the effects of new AEDs in ESES.
FP-H-098
Routine neuroimaging studies in children with complex
febrile seizures
S. Aysun, G. Haliloglu, D. Yalnizoglu
Hacettepe Children’s Hospital, Department of Pediatric
Neurology, Ankara, Turkey
The relations between intractable temporal lobe epilepsy,
a prolonged febrile seizure in childhood and mesial
temporal sclerosis are well known, but do not necessitate
a causal relationship. High-resolution magnetic resonance
imaging provides opportunities to define underlying pathology.
The aim of this study is to demonstrate neuroimaging
findings in children with complex febrile seizures who are
subgrouped according to age of seizure onset and frequency.
Of the 65 children with routine cerebral imaging, we identified
abnormal findings either related or unrelated to
seizures in 40% (26/65), mesial temporal sclerosis being
the most common underlying pathology 42.3% (11/26).
The likelihood of demonstrating an abnormal finding even
with routine neuroimaging studies is 33.3% in patients with
an onset of febrile seizures at more than 6 years of age. We
conclude that children with a febrile seizure onset and recurrence
of seizures after 6 years of age, either as febrile or a
febrile seizures, should be evaluated with magnetic resonance
imaging, even by the care-giving pediatrician.
FP-H-099
Favorable seizure outcome in Kabuki make-up
syndrome associated with epilepsy
A. Ogawa a , S. Yasumoto a , Y. Tomoda a , M. Ohfu a ,A.
Mitsudome a , Y. Kuroki b
a Department of Pediatrics, School of Medicine, Fukuoka
University, Fukuoka; b Division of Medical Genetics, Kanagawa
Children’s Medical Center, Yokohama, Kanagawa,
Japan
Purpose: Kabuki make-up syndrome (KS), was first
described in the same year independently by Niikawa et
al. and Kuroki et al. In large previously reported series of
patients with KS, the incidence of seizures was about 10–
40%. However, the appearance of seizures has not been
adequately reported or thoroughly evaluated for this
syndrome. In this study, we analyzed seizure characteristics
and differences in nine patients with KS. Methods: Nine
patients with KS associated with epilepsy were identified
among the patients followed at Fukuoka University Hospital
and Kanagawa Children’s Medical Center. The diagnosis of
KS was based on the presence of characteristic findings.
Results: Of the nine patients, five were male and four
were female. Patient ages at the time seizures started ranged
from 7 months to 12 years with a mean of 6 years. Four
patients have generalized seizures and two patients have
complex partial, with secondary generalization. West
syndrome, complex partial seizures and atonic seizures
were seen in one case each, respectively. Electroencephalogram
showed focal spikes in seven cases, frontal dominant
diffuse spike and wave bursts in one and hypsarrhythmia in
one. Seizures were well controlled in eight cases, and
incompletely controlled in only one case. Conclusions: KS
is a mental retardation-malformation syndrome affecting
multiple organ systems, with a broad spectrum of neuromuscular
dysfunction and mental ability. Epilepsy, together
with mental retardation, may be a primary feature of KS.
KS associated with epilepsy is a mainly localization-related
epilepsy with a favorable seizure outcome.
FP-H-100
Severe myoclonic epilepsy of infancy: A drug regimen
for ‘optimal’ treatment?
B. Ceulemans a,b , M. Boel a,b,c , L. Dom d , H. Willekens e ,P.
Thiry f , L. Lagae a,b,c
a Epilepsy Center for Children and Youth, Pulderbos;
b Department of Neurology, University Hospital Antwerp,
Edegem;
c Department of Child Neurology, University
Hospital Gasthuisberg, Leuven; d K. Paola Childrens Hospital,
Antwerp; e A.Z. St.-Lucas, Gent; f St.-Oda Institute, Overpelt,
Belgium
It is well accepted that SMEI or Dravet-syndrome (International
League Against Epilepsy 2001) is a very therapyresistant
epileptic encephalopathy. It is only recently that
the etiological genetic defect, a severe damage of the alfasubunit
of the sodium channel SCN1 of the brain, has been
defined. This gives a new view in the treatment of these
patients. In eleven Belgian patients with SMEI, where the
genetic mutations are known, a strategy for treatment is
proposed. It is clear that anti-epileptic drugs working mainly

524
Abstracts
by blocking sodium channels are not effective in SMEI.
They may even worsen attacks which are mainly myoclonic.
This is certainly the case for carbamazepine, phenylhydantoin
and probably also lamotrigine. Broad-spectrum antiepileptic
drugs such as sodium valproate and topiramate
are more effective. Benzodiazepines are the drugs of choice
for acute seizure treatment. Stiripentol, an inhibitor of cytochrome
P450, is not available in Belgium. An anti-epileptic
drug regimen, mainly based on a combination of broad
spectrum AED’s, and a fairly aggressive seizure-treatment
will be presented. We believe that this regimen is preferable
to a complicated multiple AED regime.
FP-H-101
Interleukin-1 beta and interleukine-1 receptor
antagonist gene polymorphisms in patients with febrile
seizure
Ş. Haspolat, Y. Baysal, M. Coşkun, O. Yeǧin
Akdeniz University Medical School, Department of Pediatric
Neurology, Antalya, Turkey
Febrile seizures are the most common type of seizure in
children. There is a strong genetic heterogeneity for febrile
seizures. IL-1b is a major pro-inflammatory cytokine that
plays role in inflammatory responses and fever. The interleukine-1
receptor antagonist (IL-1Ra) is a naturally occurring
anti-inflammatory cytokine which competitively binds
to interleukine-1 receptors, thus modulating the potentially
injurious effects of IL-1. We studied the biallelic polymorphisms
at position 2511 and 13953 of the IL-1b
gene and pentaallelic polymorphism of IL-1Ra genes in
73 patients with febrile seizure (36 simple, 37 complex
febrile seizures) and in 154 controls. The distribution of
the biallelic polymorphism at position 2511 of the IL-1b
gene was significantly different in patients with febrile
seizures (P , 0:05). Allele frequencies for IL-1b 13953
polymorphism were not different in patients and the control
group. IL-1Ra polymorphisms were found to be significantly
different when the patient group was evaluated
together (73 patients). But when the groups were classified
as simple and complex febrile seizure groups no significant
difference was present for IL-1Ra polymorphisms. We also
looked for a relation between family history of febrile
seizures, family history of epilepsy or gender and the polymorphisms.
No relationship could be found. Our data
suggest that, the biallelic polymorphism at position 2511
of the IL-1b gene may play a role in the pathogenesis of
febrile seizures.
FP-H-102
Favourable outcome of epileptic blindness in children
E. Shahar, S. Barak, D. Savitzki, J. Andraus
Child Neurology Unit and Epilepsy Service, Rambam Medical
Center, Rappaport School of Medicine, Haifa, Israel
Blindness is a rare phenomenon of epileptic seizures.
We report on thirteen children with epileptic amaurosis,
investigating the ictal manifestations, EEG findings,
analyzing the response to therapy and elucidating the
outcome in relation to termination of blindness and
seizures. Most experienced single or recurrent episodes
of acute visual obscuration, lasting for 1–30 min, mainly
manifesting in the ictal phase and only a few in the preictal
phase. Seven patients had accompanying generalized
seizures, which could be induced by photic stimulation in
three children. CT of brain performed in five children was
unremarkable. All children in this group, treated with
valproic acid, regained full constant vision and became
seizure free within a period of up to 5 years. Three children
who had the constellation of ictal amaurosis, occipital
EEG paroxysms and migrainous headaches, compatible
with the syndrome of occipital lobe epilepsy, also became
asymptomatic on therapy: two on valproate and one on
carbamazepine. Three children with epileptic blindness
also had either focal motor seizures and/or unilateral
EEG epileptic discharges, with a normal CT. On carbamazepine
therapy they regained full vision and the seizures
abated. Our data suggest a relatively benign course and a
favorable outcome for children with epileptic blindness
and associated seizures. They regain full constant vision
in association with control of seizures and preservation of
cognition.
FP-H-103
Cortical developmental malformations and epilepsy:
review of 62 pediatric patients
R.M. Valério, F.N. Arita, S. Rosemberg
Santa Casa of São Paulo Medical School, São Paulo, Brazil
We studied a series of 62 children with cortical developmental
malformations (CDM) in order to determine the
incidence of the different types, the clinical presentation
and the occurrence of epilepsy in each group. The 62
patients (32 males, 30 females) were divided into different
groups according to the MRI results: 34 (18 males, one
females) had polymicrogyria and/or schizencephaly
(PMG/SCH) (ten bilateral opercular PMG; nine focal
PMG; nine bilateral and six unilateral SCH); ten (seven
males, three females) lissencephaly/pachygyria complex
(LIS); one (female) type II lissencephaly; one (female)
double-cortex; four focal cortical dysplasia of Taylor
(FCDT) (one males, three females); two HMG (2m);
seven nodular heterotopia (NH) (two males, five females)
and three, complex unclassified CDM. Twenty-four
patients (38.7%) did not report epilepsy on the first consultation
or during the follow-up (14 PMG/SCH), six LIS, two
NH and two, complex unclassified CDM). Among the
remaining 38 epileptic patients, the seizures were
controlled with AEDs in 15 (39.4%). All patients with
abnormal cortical proliferation and differentiation disorders

Abstracts 525
(FCDT and HMG), 9 with PMG/SCH (26.5%), five with
LIS/type II LIS (45.4%) and two with NH (28.6%) had
intractable epilepsy. Almost all patients had been referred
for psycho-motor developmental delay or abnormal motor
signs. We conclude from our unselected CDM series that
the occurrence of epilepsy is far from being mandatory and
when present it may be clinically controlled. These data
differ from most reported series of CDM in which the
incidence and severity of epilepsy are more prominent
probably because in these series the patients are referred
to pre-surgical evaluation for intractable epilepsy.
FP-H-104
The study of toleration to achieving the target dose
rapidly in infants with epilepsy treated by Topamax
X.-L. Xie, Y.-Q. Zhong, J. Wu, M. Wu, W.-G. Hu
Chengdu Children Hospital, Chengdu, China
Objective: To explore an appropriate dosage and a speed
of increasing dosage of Topamax through observation of
tolerability in infants, and to increase therapeutic effects
and decrease side effects. Methods: We studied 23 infants
with epilepsy from our hospital in 2001. We increased the
Topamax to the target dosage rapidly at 2 or 3 weeks
intervals, by increasing 0.5 mg/kg per day once every 3
days. The average target dosage was 6.2 mg/kg per day.
Therapeutic evaluation was: excellent, if seizure-free;
better, if the frequencies decreased $75%; good, if
$50%; and, bad or ineffective if ,50%. Results: Therapeutic
effects were excellent in 11 of 23 cases (47.8%);
better in seven (30.4%); and, good in five (21.7%). No case
was defined as ineffective or deteriorated. Conclusion: The
therapeutic effects and side effects of Topamax had individual
differences, so methods of giving the drug are important.
Side effects occurred less frequently in infants than
that in other children over 1 year old. It was reasonable to
reach the target dosage of Topamax rapidly in infants with
epilepsy.
FP-H-105
Comparison of partial and generalised childhood
epilepsies using principal and independent component
analysis
C.P. Unsworth a , J.J. Spowart b , G. Lawson b , J.K. Brown b ,B.
Mulgrew a , R.A. Minns b , M. Clark b
a Department of Electronics and Electrical Engineering;
b Department of Child Life and Health, University of Edinburgh,
Edinburgh, Scotland, UK
A typical EEG consists of roughly 20 electrical recordings
which are received from electrodes located at different
regions of the scalp. Each recording represents a
mixture of signals from a variety of brain activities.
Depending upon a patient’s activity such signals can
become very complicated to interpret. This is true in the
case of epilepsy. Independent component analysis (ICA) is
a signal-processing tool that can take data from a multichannel
system, as in the EEG, and statistically de-mix the
real signals from their mixtures. As long as the number of
electrodes is larger than the number of real signals that
exist, ICA is valid. Recently ICA has claimed to be able to
de-mix the real epileptic signal components from the
mixtures of an EEG. This seems quite promising as the
EEG has a large number of channels and the signals
detected during an epileptic attack appear relatively simple
and to have few generating sources. In this paper we
examine a variety of partial and generalised epilepsies,
some of which are evoked and others un-evoked, which
occur in children. By examining the correlation between
the principal components of the epilepsies presented here,
it is possible to compare and contrast the different forms of
epilepsy, thus, gaining an insight into nature of the epilepsies
and their connection to each other. In addition, from
this analysis is possible to draw conclusions on the applicability
of ICA to the EEG for the different forms of
epilepsy.
FP-H-106
A study of supporting school life of children with
epilepsy
H. Nagao a , Y. Suzuki b , T. Morimoto b
a Department of Pathophysiology, Faculty of Education,
b Department of Pediatrics, Faculty of Medicine, Ehime
University, Ehime, Japan
In order that children with epilepsy can lead safe and
active lives, and also be encouraged to pursue full school
activities, an activity-based school safety map was made.
Subjects were 27 students who had experienced seizures
within the past 2 years, selected from 51 children with the
diagnosis of epilepsy, in a school for mental disabilities
(with a total population of 236). This activity based school
safety map was planned by the school teachers and the
author. The results showed that the students were divided,
according to the guidance of daily life in children with
epilepsy proposed by author, group A: four, group B:
seven, group C: 11, and group D: five. The degree of aid
required in daily activities was categorized into: (1) hand in
hand; (2) within reach of hand; (3) within reach of
eyesight; and (4) no supervision necessary. The school
environment was categorized into five groups according
to the risk and supervision necessary, as follows: (a) swimming
pool; (b) stairs and edge of stage; (c) corridors; (d)
training classroom; and (e) ordinary classroom. This study
proved that making an activity based school safety map for
individuals with epilepsy according to individual needs,
enabled the children to pursue full school activities as
much as possible.

526
Abstracts
FP-H-107
Language disorders and epilepsy
A. Al-Qudah
Division of Pediatric Neurology, Jordan University Hospital,
Amman Jordan
Background: A number of language disorders have been
associated with epilepsy or EEG epileptiform abnormalities.
These include Landau-Kleffner syndrome (LKS), electrical
status epilepticus of sleep, developmental dysphasia and
possibly autism. LKS patients have been treated with antiepileptic
drugs and steroids without apparent success. LKS
aphasic patients have been recently treated with immunoglobulins
with good results. This study reports relatively
successful results after treatment of LKS patients with immunoglobulins.
Patients and methods: Four patients with LKS
have been included in the study since November 1999. They
were all males with age range of 5.5–14 years (Mean ¼ 9.5).
Patients were scheduled to receive five consecutive doses of
immunoglobulins over 5 days and one dose every month for 6
months. MRI was done on all patients. Results: Age of onset
of seizures was 3.5, 5.3, 7.5, and 1.5 years and of aphasia was
3.5, 5.9, 8, and 2 years, respectively. Duration of aphasia was
7, 1, 0.8 and 4 years and follow-up was 2 years, 2 months, 5
months and 1 month, respectively. Onset of response to
immunoglobulins was at day 7, 5, 9 and 7, respectively, for
the four patients. Patients 2 and 3 had complete recovery of
their language. Conclusion: This study has added further
support to the little literature on the beneficial value of immunoglobulins
in aphasic LKS patients.
FP-H-108
Diagnosis, types, management of convulsion disease of
the children
R. Sutar
Bhubaneswar Municpal Corporation Hospital, Bhubaneswar,
India
Convulsive disease of children is the most difficult,
complicated disease because of its causes, different types,
diagnosis and treatment which are not yet clear and definite
like all types of diseases seen in the world. Convulsive
disease is the result of deranged brain cells due to direct
disease of the brain cells or effects of other diseases of the
body affecting the brain cells. As in other diseases of people,
advanced modern miracle treatment, such as treatment by
new exchange of organs etc., an alternative process, can
give better life in chronic disease, as seen in other diseases.
But such a clear cut, easy and definite modern facility for cure
of convulsive disease of children is not yet given, by any
modern medical scientist of the world, as a scientific topic
in publication. The parents are still in a dilemma as to
whether they will get hope of a positive permanent cure of
convulsions, as seen with other diseases of people. These
problems are seen more in developing and poor countries
which have no advanced facility for this disease, and, as a
result, people are forced to believe in the old unscientific
processes of treatment. So a universal, reasonably acceptable,
amicable process of diagnosis, facilities, treatment and
education for the people in relation to the diseases should be
given to all concerned in the world, irrespective of city or
remote, inaccessible area, considering the standard living of
the people and facilities available therein. The modern good
effect of the treatment as seen in other diseases of the world,
will be shown to the people in cases of convulsive disease for
their belief and acceptance; and, guidelines of education will
be given to the people for the prevention of the so called
incurable convulsive disease of children. This topic has
been presented in television for discussion and newspaper
for popular science.
FP-H-109
Clinical and EEG study of seven patients with chronic
progressive epilepsia partialis continua of childhood
A. Muto, H. Oguni, K. Imai, Y. Maeda, M. Funatsuka, M.
Osawa
Department of Pediatrics, Tokyo Women’s Medical University,
Tokyo, Japan
Chronic progressive epilepsia partialis continua (EPC) of
childhood is characterized by intractable partial epilepsy
and slowly progressive neurological deficits affecting one
side of the body. Most of the patients with this syndrome
have been regarded as Rasmussen syndrome (RS), because
chronic localized encephalitis has been shown to be
responsible for this epilepsy. We studied the clinical,
neuroimaging and EEG findings of patients with chronic
progressive EPC to reveal it’s long-term evolution.
Subjects: The subjects fulfilling the clinico-electrical
features of chronic progressive EPC were seven patients
including two with pathologically confirmed RS. Methods:
We retrospectively studied their medical records, EEG files
and neuroimaging findings. Results: Two patients had uveitis
and one had impetigo before epilepsy onset. The median
age at onset of epilepsy was 6 years and 3 months. The
period between the onset of epilepsy and that of daily
seizures ranged from 1 month to 9 years. Four children
ran a rapidly progressive clinical course and eventually
developed hemiplegia, while the remaining three had a
slower clinical course and two eventually developed mild
hemiparesis. MRI showed progressive atrophy of one
hemisphere in all cases. As for treatment, five patients
received steroid pulse therapy without significant benefits.
Three cases underwent epilepsy surgery. One child died of
pneumonia. Conclusion: This study suggested the presence
of two subtypes in patients with chronic progressive EPC
or RS, one of which has an early epilepsy onset and rapidly
progressive clinical course, while the other has a later
epilepsy onset and more slowly progressive course.

Abstracts 527
FP-H-110
Lamotrigine pharmacokinetics in children
M.I. Arranz a , M. Manero a , M. Aparicio b , Lorenzo b ,E.
Saenz c , B. Tirapu a , R.J. Del a , J. Mercader a , E. Ripoll a
a Bioquímica, b Pediatría, c Hospital Ramón y Cajal, Neurología,
Hospital Gregorio Marañó, Madrid, Spain
Lamotrigine (LTG) is a new AED approved as adjunctive
therapy or monotherapy. LTG is effective against seizures:
partial, primarily generalized tonic-clonic, absences, and
drop attacks associated with the Lennox-Gastaut syndrome.
Its metabolism is affected by co-medicated AEDs. TDM in
children is useful because of age-related differences in
metabolism. We present LTG pharmacokinetic behavior
in children. Methods: We reviewed LTG data from May
2000 to February 2002. Concentrations were determined
by HPLC. Results: From 137 LTG levels, 58 in patients
on monotherapy; and, in association with: VPA (n ¼ 15);
clobazam (CLB) (n ¼ 15), carbamazepine (CBZ) (n ¼ 10),
ethosuximide (ETX) (n ¼ 4), phenobarbital (PB) (n ¼ 1)
and topiramate (TOP) (n ¼ 1); with: VPA 1 CLB (n ¼ 8),
VPA 1 ETX (n ¼ 6), VPA 1 CBZ (n ¼ 3), VPA 1 TOP
(n ¼ 3), ETX 1 CLB (n ¼ 4), PB 1 primidone (PRIM)
(n ¼ 2), CBZ 1 CLB and CBZ 1 TOP (n ¼ 1); to: PB 1
ETX 1 PRIM (n ¼ 2) and VPA 1 CBZ 1 TOP (n ¼ 1),
and with: VPA 1 PB 1 CLB 1 PRIM (n ¼ 1). Mean LTG
levels (mg/l) were lower on monotherapy (2.5 1 1) than
with VPA alone (3.2 1 1.8), or, plus other AEDs
(3.7 1 1.3) except CBZ, and lower when AEDs other than
VPA were added (1.9 1 0.8). Highest LTG values were
found with: VPA 1 TOP (4.9 1 0.8), VPA 1 CLB
(3.9 1 1.4), VPA (3.2 1 1.8), and VPA 1 ETX
(2.8 1 1.3). Lamotrigine mean doses ranged from 130 to
275 mg/24 h. Conclusions: Lamotrigine efficiency increases
with VPA alone or with others, except CBZ; and is underused
with AEDs other than VPA.
FP-H-111
Topiramate as monotherapy in childhood and
adolescent epilepsy
M. Verdecchia, P. Parisi, M. Montanaro, C. Arpino, P.
Curatolo
Department of Pediatric Neurology, Tor Vergata University
of Rome, Rome, Italy
TPM has proved to be effective as add-on therapy in children
with partial onset seizures. This study was carried out to
evaluate the effectiveness of TPM as monotherapy in epileptic
children and adolescents with no or limited exposure to
other antiepileptic drugs. Twelve patients aged 3–17 years
with partial or generalised epilepsies were treated with topiramate
for a median period of 10 months (range 6–20 months).
Of the 12 patients enrolled, six were children with newly
diagnosed epilepsy. Five patients had idiopathic tonic-clonic
generalised epilepsies, three had benign rolandic epilepsies
and four had symptomatic partial epilepsies. The starting
dose was 0.5–1.0 mg/Kg per day. TPM was increased slowly
at a mean rate of 25 mg/week up to clinical response or to a
maximum of 2–4 mg/Kg per day. The concomitant AED, if
present, was discontinued as TPM was titrated to target
dosages. Eight patients were seizure-free and two had 50%
or greater reduction in seizure frequency; two patients with
rolandic benign epilepsies discontinued because of inadequate
seizure control. Efficacy was seen against simple and
complex partial seizures and generalised tonic-clonic
seizures. The most common adverse effects were anorexia
and weight loss recorded in three cases; only one patient
experienced paresthesiae. Side effects were mild and transitory,
usually occurring during the first 3 weeks of treatment.
Our experience suggests that TPM is safe and efficacious as
monotherapy in a wide spectrum of childhood epilepsies.
The incidence of adverse events was lower than reported
with TPM polytherapy, suggesting that some side effects
observed with TPM as add-on therapy may result from pharmacodynamic
interactions with other AEDs.
FP-H-112
Liposteroid therapy for refractory seizures in West
syndrome
S. Tanaka, A. Araki, Y. Kobayashi
Department of Pediatrics, Kansai Medical University,
Osaka Japan
It was reported recently that liposteroid (LS) was effective
for the treatment of West syndrome (WS), with fewer
side effects than those of ACTH. We report a patient with
WS who was successfully treated with LS after ACTH therapy
was ineffective. A female infant was born at term after
an uneventful pregnancy and delivery. Tonic spasms developed
at 2 months of age. An EEG showed hypsarrythmia,
and she was diagnosed as having WS. Magnetic resonance
imaging of the brain disclosed no abnormality. Vitamin B6
and zonisamide were started without any effect. ACTH therapy
was commenced at 3 months of age (0.01 mg/kg for 2
weeks). The number of seizures decreased, but tonic spasms
were not controlled completely. Intravenous administrations
of gamma-globulin were also not effective. Liposteroid therapy
was begun at 4 months of age. In line with the original
method reported by Yamamoto et al., LS (0.25 mg/kg) was
given intravenously once a week for the first 4 weeks, once
every 2 weeks for the next month, and once a month for the
3rd month. Three weeks after the initiation of LS therapy,
the seizure frequency remarkably decreased. A follow-up
EEG showed the disappearance of hypsarrythmia, but
diffuse polyspikes remained. She had no adverse effects.
The patient is now 1 year old and free from convulsions.
Our experience of this case suggests that LS therapy might
be a new option for the treatment of refractory seizures in
WS.

528
Abstracts
FP-H-113
A clinico-electroencephalographic study of children
suffering from febrile seizures plus
K. Kobayashi a , Y. Ohtsuka a , Y. Nishio b , M. Fujiwara b ,I.
Ohmori a , T. Akiyama a , T. Ogino a , H. Yoshinaga a ,N.
Murakami a , K. Nakano a , Y. Watanabe a , T. Nakahori a ,E.
Oka a
a Department of Child Neurology, Okayama University
Medical School, Okayama; b Department of Pediatrics, JA
Fuchu General Hospital, Hiroshima, Japan
Purpose: FS 1 are attracting attention because of their
related genetic abnormalities, and are defined as FS continuing
beyond 6 years of age or those associated with afebrile
seizures earlier than 7 years. However, general clinical
pictures of children suffering from FS 1 have not yet been
thoroughly clarified. We tried to elucidate their clinical and
electroencephalographic characteristics as compared with
those of children with only FS. Subjects and methods: By
reviewing the clinical records of the pediatric neurology
clinic in the JA Fuchu General Hospital, we found 27
patients with FS 1 (Group FS 1 ) and 51 with only FS
(Group FS), and studied their family history, clinical
pictures and EEG findings. Results: Family histories of
seizures were noted in ten patients (37.0%) of Group FS 1
and in 23 (45.1%) of Group FS. In Group FS 1 , 14 patients
(51.9%) had FS occurring beyond 6 years of age, 12 (44.4%)
had afebrile seizures, and the remaining one (3.7%) had
both types of seizures. Two also had seizures induced by
TV/video-games, and another suffered from absences. The
observed epileptic EEG abnormalities were diffuse and/or
focal, and were noted in 11 patients (40.7%) of Group FS 1
and 12 (23.5%) of Group FS. There were no statistically
significant differences regarding the above findings within
Group FS 1 or between the two groups. Conclusions: The
clinico-electroencephalographic characteristics of the children
with FS 1 were diverse, and their rate of positive family
history was no higher than that of children with only FS.
FP-H-114
A case of a 15-year-old patient with intractable epilepsy
who frequently showed gelasma after the introduction of
clobazam
T. Iwasaki, H. Miura, W. Sunaoshi, N. Hosoda, K. Takei, F.
Katayama
Department of Pediatrics, Kitasato University School of
Medicine, Kanagawa, Japan
This is a case of a 15-year-old boy patient who has severe
mental retardation and spastic paraplegia due to fetal
distress and hypoxic brain damage at the time of birth.
The patient developed West syndrome at the age of 7
months. He was diagnosed with undetermined epilepsy
more recently. His intractable epilepsy had not been
controlled well with the administration of nitrazepam and
zonisamide for 5 years. We added clobazam (CLB) to this
combination therapy. CLB was marketed 2 years ago in our
country for the first time. After the introduction of CLB, his
intractable tonic spasms disappeared. But so-called gelastic
seizures appeared frequently before falling asleep. Sleep
disturbance became troublesome subsequently. The gelastic
seizures manifested as laughing with a stiff face and a wry
mouth. As side effects of CLB, gelastic seizures have not
been reported previously, although irritability or sleep
disturbance have been recognized.
FP-H-115
The electro-clinical features of Landau-Kleffner
syndrome
K.Yu. Mukhin, A.S. Petrukhin, A.A. Kholin, L.Yu.
Glukhova, N.V. Mikhalitchenko
Paediatric Neurology Clinic of Russian State Medical
University, Moscow, Russia
Purpose: To summarise the seizure semiology, clinical
and EEG findings in LKS. Methods: Clinical examination,
EEG, sleep EEG, video-EEG monitoring, neuroimaging
were recorded in the investigation of five patients with
LKS. Results: We investigated four boys and one girl
aged 6–10 years (medium – 7.8 years) affected by LKS.
The epileptic seizures, presented in three children, began
between 2.5 and 6 years of age (medium – 4 years) with
atypical absences (two cases) and simple partial pharyngooral
seizures (one case). Two of five patients had no
seizures. We have observed the following types of seizures:
atypical absences (all three patients with seizures) accompanied
by atonic drop attacks – in one patient, simple partial
with secondary generalization before awakening (two
patients). All the children developed sensory and motor
aphasia, and cognitive dysfunction with hyperactivity.
Two presented autistic disorder with aggressive behaviour.
The EEG investigation revealed fronto-temporal (three
patients) or centro-temporal (two) sharp-slow wave and
‘rolandic-like’ spike focuses right (one), left (one) unilateral
and right (two), left (one) unilateral with diffuse sharp-slow
wave patterns. Both regional and diffuse sharp-slow wave
patterns increased in non-REM sleep. Secondary bilateral
synchrony mechanism were evident in all cases: continuous
diffuse spikes and waves during slow sleep but less than in
ESES – two patients and ESES in three patients. MRI was
normal in four cases. Antiepileptic drugs used in our group
were valproic acid (Depakin), carbamazepine (Finlepsin),
suxilep, lamotrigine, clobazam, and sulthiame. The seizures
have a favourable outcome, but the aphasia is usually resistant
to therapy. Conclusion: We think that the LKS is a form
of partial epilepsy of cryptogenic origin with a large spectrum
of clinical features. EEG findings often show secondary
bilateral synchrony and continuous spikes and waves
during slow sleep progressing to ESES.

Abstracts 529
FP-H-116
Health-related quality of life in children with epilepsy:
development and validation of a self-report- and a
parent proxy respondent measure
G.M. Ronen, D.L. Steiner, P. Rosenbaum
Canadian Pediatric Epilepsy Network, McMaster University,
Hamilton, Ontario, Canada
Health-related quality of life (HRQL) includes the
patient’s own experiences, preferences, values, and their
perceptions of their symptoms and functioning. HRQL
broadens the range of outcomes that are measured and
expands the professional’s awareness of issues that concern
patients. We used qualitative and quantitative research
methodologies sequentially to develop a self-report- and a
parent proxy HRQL measure for pre-adolescent children
with epilepsy. Modified focus groups, run separately with
children with epilepsy and their parents, facilitated exploration
of HRQL issues. Textual analysis helped us understand
the HRQL components and identify 31 domains clustered
into five major themes. Items for the scale were extracted
from the raw data. We created a scale formatted with alternative
paired options of forced responses and used factor
analysis to reduce the number of items. Internal consistency
was measured with Cronbach’s alpha; test-retest reliability
with the Pearson Correlation, and construct validity with
seven hypotheses. A total of 381 children with active
epilepsy, ages 6–15, and their parent(s) completed separately
a 67-item questionnaire. We chose five subscales
from the factor analysis with five items per subscale. Children
and parents could discern differences and report differentially
between the various aspects of their HRQL. Testretest
was only satisfactory for parents and children 8 years
and older. Internal consistency, construct and discriminative
validity were acceptable for all subscales. Our data provide
solid psychometric properties for user-friendly HRQL
measures for parents and children 8 years and older. The
measures subscales encompass HRQL dimensions identified
and judged most important by children with epilepsy
and their parents.
FP-H-117
Evaluation of 76 patients with infantile spasms:
epidemiologic and clinical data
Ş. Uçar a ,Ö.F. Aydin b ,N.Şenbil b ,Ü. Ertan a , Y.K.Y. Gürer 2
Dr. Sami Ulus Children’s Hospital, a Department of Pediatrics
and b Pediatric Neurology, Ankara-Turkey
Infantile spasms (IS) is an epileptic syndrome of early
infancy with poor prognosis which is not controlled by standard
antiepileptic drugs. Medical records of 76 patients
diagnosed with IS between 1995 and 2001 were reviewed.
Forty-two of them were males (55.3%) and 34 were females
(44.7%). The mean onset of infantile spasms was
5.88 ^ 0.53 months. The mean admission age was
8.79 ^ 0.54 months. Spasms were seen mostly at 4–6
months (50%) and 80.3% started before the aged of 1.
Seventy of 76 cases were symptomatic (92.1%) and six
were cryptogenic (7.9%). In the symptomatic group prenatal,
perinatal and postnatal causes were 42.9, 34.3 and
17.1%, respectively. No etiological cause was found in
5.7%. Hypoxic ischemic encephalopathy was the most
common of the prenatal and perinatal causes (48.58%).
Flexor spasms were seen most frequently (59.2%), mixed
spasms were less frequent (31.6%) and extensor spasms
were the least frequently seen (9.2%). A total of 35.5% of
the cases had other kinds of seizures before IS. Spasms
disappeared before the age of 2 in 63.2% of the cases.
EEG findings improved with ACTH treatment. Early treated
patients showed better responses to the treatment. No significant
difference was found in neurodevelopmental and
mental improvement between the pre and posttreatment
states. The patients who were on ACTH or ACTH 1
pyridoxine treatment showed more complete response
compared to the patients on the other alternative treatments.
Patients with tuberous sclerosis showed better response to
vigabatrin. A total of 34% of the 57.9% patients with remission
showed relapse. Relapses were less in the early treated
group. Relapses responded to treatment poorly. Mortality
rate was 2.6%, 25% of the patients developed other kinds
of epilepsies after IS, which were mostly LGS.
FP-H-118
Teaching paediatric and adolescent epilepsy at advanced
level in Slovenia – example of a small country
I.M Ravnik a , P. Wolf b
a Ljubljana, Slovenia, b European Epilepsy Academy, Slovenia
Rationale: In Slovenia (2 million inh), the practice of
child neurology started by studying and treating seizure
disorders (Jeras, 1954), evolving into full-scope child
neurology. General paediatricians have been offered update
courses, and special courses including paediatric and
adolescent epilepsy (PAE) have been organized for paediatricians
interested in neurology. Advanced epilepsy
courses, due to few trained specialists and small audiences,
could not be similarly established. The small team, still
developing, mostly educated abroad, participates with
interested adult neurologists and organizes advanced
courses/workshops for child neurologists, neurophysiologists
and neurologists, with foreign and local experts to
compensate for the lack of formal training in epileptology.
What would be the best way to go in future? Method: (1)
Analysis of post-graduate teaching on epilepsy via
programmes organised by the Slovenian ILAE Chapter,
Slovenian Medical Society’s Epileptology Section, and
local Epi-club in collaboration with European Epilepsy
Academy (Eurepa), and established bilateral (e.g. Slove-

530
Abstracts
nian – French) co-operation. (2) Analysis of questionnaires
on gaps in knowledge in PAE care in Central Eastern
European countries (CEEC). Results: Different answers
could be obtained from students depending on their
previous knowledge. All university post-graduate courses
(paediatrics, child neurology, neurophysiology, child
psychiatry) do offer basic knowledge of PAE. Advanced
students claim more knowledge in neuroradiology, paediatric
EEG and electro-clinical characterisation of PAE
syndromes. Clinical workshops (on classification of epileptic
syndromes, drug treatment, assessment of drug trials,
epilepsy management, EEG, neuropsychology of epilepsy,
severe epilepsies, comprehensive epilepsy care, epilepsy
and handicap) in collaboration with foreign centres and
Eurepa’s trainers have been well attended. Assessment:
clinical work with case-studies and real-life ‘know-how’
added to theoretical work is favoured by the students.
The CEEC questionnaire (Ravnik et al., Epilepsia 1999)
on comprehensive PAE care disclosed domains in which
‘knowledge and expertise were felt to be missing’: presurgical
assessment, surgery (6/7 countries); social work,
functional imaging (5/7); remedial teaching (4/7); neuroradiology,
neuropsychology, child and adolescent psychiatry,
clinical psychology (3/7); while only 2/7 listed EEG
and 1/7 neuropharmacolgy. Quite a few of the above do not
belong to the domain of child neurology in a narrow sense.
The same study showed that in many CEEC, child neurologist
was in charge of the whole bulk of problems encountered
in comprehensive epilepsy care. The perceived ‘lack
of knowledge’ may indeed correspond to lack of paramedical
staff and/or lack of knowledge enabling effective
communication between child neurologist and other
professionals. This may have to be considered in planning
education of child neurologists on PAE care. Conclusions:
Experience gathered in post-graduate teaching of PAE
through years by the local team networking with international
centres of excellence, and including audience from
the neighbouring countries, has proved very useful. Plans
are underway to formalize some of the already tested
educational projects into a curriculum adapted to the
demands of European Epilepsy Academy offering future
child neurologists in the region high level training opportunities
in spite of non-existing formal epileptological
training in their countries.
FP-H-119
Main side-effects of valproic acid drug treatment for
pediatric patients with epilepsy
V.M. Studenikin, O.I. Maslova, V.I. Shelkovsky, S.V.
Balkanskaya, M.Z. Karkashadze
Division of Psychoneurology, Scientific Center of Child
Health (Russian Academy of Medical Sciences), Moscow,
Russia
Background: Valproic acid (VA) drugs are currently used
world-wide as the most popular medicines for seizure
control in the management of partial and generalized
seizures in pediatric and adult patients, but unfortunately
valproates are also known to induce some undesirable
side-effects. Goal: Therefore, the aim of our study was to
find out the incidence of various undesirable effects in
pediatric patients with epilepsy, undergoing therapy with
VA. Method: For this purpose we have analyzed case
histories of 383 epileptic infants and children (aged 2
months–15 years) hospitalized in the neurological clinic in
1995–1999, and receiving VA as mono-or polytherapy.
Results: Most side-effects observed can be divided into
two main categories: hepatotoxic in 26.9% of cases (varying
from raised hepatic enzymes to biliary dyskinesia, mild
reactive pancreatitis, etc.) and hematological changes
(leukopenia with accompanying platelet decrease) 6% of
patients. The latter led to bleeding diathesis in three pts.
Such various side-effects as excessive weight-gain, alopecia,
decreased calcium in serum, and cognitive deficit were
registered, as well, but less frequently. Only rarely VA sideeffects
could be clearly categorized as dose-related, resulting
from idiosyncratic or hypersensitivity reactions.
Conclusion: Our data support the hypothesis that, aside
from common gastrointestinal tract (GIT) side-effects,
administration of VA drugs may lead to thrombocytopenia
of heteroimmune origin, and another possibility is that liver
dysfunction or medicinal hepatic damage develops with
resulting platelets depression. These immunological considerations
remain to be debated.
FP-H-120
Neurovisualisation techniques in diagnostics of central
nervous system dysgenesis with symptomatic epilepsy in
pediatric patients
O.I. Maslova, L.M. Kuzenkova, V.M. Studenikin, S.Y.
Ishkhanova, E.I. Stepakina
Division of Psychoneurology, Scientific Center of Child
Health (Russian Academy of Medical Sciences), Moscow,
Russia
Background: Progress in modern radiology substantially
improves the arsenal of diagnostic methods in neurology.
Seemingly, the diagnoses of CNS dysgenesis and seizure
disorders benefit from the new investigational techniques
most of all. Goal: Combined utilization of neurovisualisation
methods in the determination of brain dysgenesis,
extent and specificity, in children, was the aim of our
study. Method: CT, MRT and SPET scans were used in a
group of 35 patients, hospitalized with cerebral dysgenesis
originally postulated on the basis of typical EEG-verified
epileptic activity (all children aged 1–15 years). Results:
The described group of patients showed the following most
important findings: (1) hypoplasia of cerebral hemispheres
(42.8%); (2) intracranial hemorrhages (37.1%); (3) destruction
of cortical lamina (31.4%); and (4) false porencephalic

Abstracts 531
cysts in white matter of the brain (28.5%). Pathological
vascularisation of brain structures (varying from mild to
profound) was only evident with SPET scans (in 68.5%
of cases observed). Conclusion: CT and MRT scans
allow the demonstration of structural and morphological
cerebral problems, but establishment of adequate clinical
long-term prognoses can only be expected, when the
above-mentioned diagnostic routines are combined with
SPET. The latter method not only objectively (quantitatively)
verifies topographic vascularisation of cerebral
structures, which is of critical interest in pediatric patients
with congenital brain anomalies, but its prognostic value
seems to be second to none, when investigating the cases
of cerebral dysgenesis in pediatric patients.
FP-H-121
Experience in management of Rasmussen syndrome in
pediatric patients
V.I. Shelkovsky, V.M. Studenikin, O.I. Maslova, O.V.
Globa
Division of Psychoneurology, Scientific Center of Child
Health (Russian Academy of Medical Sciences), Moscow,
Russia
Background: Rasmussen syndrome (chronic progressive
focal encephalitis) is a rare neurological form of epilepsy,
described over 40 years ago, with only about 50 verified
pediatric cases reported worldwide. Goal: Our aim is to
describe and to share our own experience in therapeutic
approach and management of Rasmussen syndrome in
pediatric patients. Method: We have analyzed case
histories of four pediatric patients, hospitalized and receiving
treatment in our clinic since 1996. Results: The ages of
patients with Rasmussen syndrome (three females and one
male) varied from 18 months to 14 years and 9 months.
The earliest onset of the described condition was registered
at the age of 9 months, and the latest took place at
the age of 11 years and 8 months. In two cases (both of
them young children) myoclonic paroxysms were evident
with secondary generalization (tonic propulsive paroxysms
with absences), while in two other cases classic partial
paroxysms were seen with myoclonic seizures development
after the period of 2–3 months. Anticonvulsant
drugs such as valproic acid, carbamazepine and phenobarbital
were used, but Rasmussen syndrome proved to be
refractory (the number of paroxysms varied from 5 to 30
daily). In one case complete remission (presently 3 years)
was achieved in a 25-months old female patient after
acyclovir intravenous administration (for herpes type I
generalized infection). Conclusion: Our hypothesis is that
Rasmussen syndrome results from an autoimmune pathological
pathway, which can possibly be altered by using
immunosuppressive drugs, but we lack evidence for this
concept.
FP-H-122
Dynamics of EEG data in pediatric patients with various
epilepsy types under valproic acid treatment and
monitoring in blood
A.V. Anikin, N.Y. Semenova, V.I. Shelkovsky, O.I.
Maslova, V.I. Shelkovsky, S.V. Balkanskaya
Scientific Center of China Health, Russian Academy of
Medical Sciences, Moscow, Russia
Background: Monitoring of VA blood concentration is
currently viewed as disputable, compared with EEG
dynamics in patients with epilepsy under treatment with
this anticonvulsant class. Goal: The aim of our study was
to register correlations between EEG patterns and VA blood
concentrations in children with epilepsy under valproate
treatment for optimization of the therapy. Methods: Ninety
patients (aged 2 months–16 years) with various types of
epilepsy were observed. Digital EEG (‘DG Examiner’,
‘10–20’ system, standard technique) and VA blood monitoring
(until 80–120 mcg concentration achievement) were
incorporated. Positive clinical effect was judged by
complete seizure control or decrease in attacks, with EEG
evidence of reduction or abatement of epileptiform signs.
Results: Typical epileptic seizures and spikes, spike-andslow
wave EEG patterns were evident in 83% of cases,
generalized paroxysms in 32% and focal abnormalities in
42% of cases (in 9% of the latter secondary generalization
was present). Patients with idiopathic epilepsy had better
clinical results and EEG patterns as compared with cases
of symptomatic epilepsy. Correlation was registered
between the VA concentration in blood and EEG specific
activity. In addition, repetitive studies revealed increases in
voltage and discharges in 19% of patients with localized
epileptic activity. Conclusion: Our data seemingly show
that the use of valproic acid blood concentration monitoring
in combination with adequate EEG control is considerably
more reliable in pediatric patients with generalized epileptic
activity, than in cases of epileptic partial seizures. Treatment
effectiveness was considerably higher when increased
VA blood concentrations were seen.
FP-H-123
The clinical characteristics of febrile seizures and
outcomes in children
T. Galia
Almaty ‘480063, Aksai-4’ –101, app. 18, Kazakhstan
The purpose of this research was the study of clinical signs
and outcomes of febrile seizures in children. We observed
179 (75.8%) children with simple and 57 (24.5%) with
complex febrile seizures. Simple febrile seizures were characterized
as generalized tonic-clonic in 43.6% (78), clonic in
25.7% (46), atonic in 19% (34), tonic in 11.7% (21) of children.
Relapses offebrile seizures in this group, were observed

532
Abstracts
in 46.4 (83) children. Simple febrile seizures remitted favorably
in 78.2% of cases. In 21.8% the development of epilepsy
was observed: absence (15), grand mal (19), Lennox-Gastaut
syndrome (four), infantile spasms (one). The spasms of
complex febrile seizures began with a turn of the head and
eyes to the side in 10.5% (six); with nystagmus followed by a
turn of the head and eyes to the side in 7% (four); with focal
motor paroxysms, in 36.8% of children, followed by generalization.
Other seizures observed were myoclonic-12.3%
(seven), tonic-clonic 59.6% (34), tonic 10.3% (23). In 3.5%
(two) children a visual and epigastric aura was observed
before the beginning of an attack. In 64.9% (37) cases there
was evolution to epilepsy: symptomatic partial 22.8% (13),
grand mal 21.1% (12), temporal lobe 7% (four), myoclonic
epilepsy-5.3% (three), infantile spasm-8.8% (five). In 35.1%
(20) cases remission of disease was observed. Therefore the
features of the clinical signs of the febrile seizures were
reflected in the outcomes of the disease.
FP-H-124
Recurrent febrile seizures induced brain damage in
developing rats
Z.-X. Yang a , J.-M. Wang b , G.-P. Zhou a , X.-Z. Chang a ,J.
Qin a
a Department of Pediatrics, Beijing University First Hospital,
Beijing, China; b Department of Pediatrics, Shangqiu
First People’s Hospital, Shangqiu, Henan, China
The present study is to establish a FS model and to
explore the FS induced brain damage in developing rats.
FS in Wistar rats was induced ten times, once every 2
days, through a warm (44.58C) water bath. The rats were
randomly divided into two groups: 37.08C water bath group
(n ¼ 10), and 44.58C water bath group (n ¼ 40), The latter
was subdivided into non-FS (FS ¼ 0, n ¼ 10) and FS (FS
$6, n ¼ 22) groups. On HE stain, the disarrangement of
hippocampal CA1 and CA2 neurons in FS group was
observed. The cellular polarity was not clear, and vacuolization
appeared. No obvious loss of hippocampal neurons in
FS group was observed on thionine stain. Whereas neuronal
numerical density (Nv, counted by means of a stereological
method, the dissector system) in hippocampal CA1 in FS
group was significantly decreased (P , 0:01) compared
with that in normal and non-FS groups. The ultrastructural
abnormalities of the hippocampal neurons were observed
under the electron microscope. In FS rats, the mitochondrial
volume was markedly decreased, the matrix condensed, the
ridge obscured or disappeared, and vacuoles formed in some
mitochondrias. Mild to moderate dilation of Golgi apparatus
was also demonstrated. The results indicated that there were
many similarities between warm water-induced FS in developing
rats and febrile seizures in children. This model is
suitable for the research on the pathogenetic mechanisms
and brain damage in FS. Recurrent FS may cause damage
and loss of hippocampal neurons in the developing rats.
(This work was partly supported by a key clinical project,
2001-03, from the Ministry of Public Health of China).
FP-H-125
The syndrome of facial hemangioma – cerebral cortical
and vascular dysplasia: a rare neurocutaneous
syndrome associated with epilepsy
H.B. Szliwowski, A. Aeby, P. David, G. Rodesch, P. Van
Bogaert
ULB-Hôpital Erasme, Brussels, Belgium
Patients with extensive facial hemangioma may present
abnormalities of the cerebral arteries ranging from minor
vascular dysplasia to agenesis of major arteries. Some of
them present associated brain malformations which affect
mainly the cerebellum (Dandy-Walker malformation, cerebellar
hypoplasia or atrophy). We report a patient in whom
facial hemangioma was associated with vascular cerebral
dysplasia and frontal polymicrogyria. This 13-year-old girl
had a history of congenital facial hemangioma which
progressively extended over her nose and forehead during
the first year of life, and then spontaneously regressed.
Neurological examination showed mild mental retardation
and a discrete congenital left hemiplegia predominating in
the upper limb. Seizures were present from the age of 11
years and were refractory to valproate and carbamazepine.
EEG showed interictal right rolandic spikes and generalized
seizures (atypical absences and tonic seizures). Cerebral
MRI showed polymicrogyric-like changes affecting the
right frontal lateral cortex and the parasagittal frontal
cortices, and hypoplasia of the corpus callosum. At cerebral
angiography, the pericallosal arteries appeared tortuous and
presented focal dilatations. This is, to our knowledge, the
second reported case of supra-tentorial cortical dysplasia
associated with facial hemangioma and vascular cerebral
malformation. The fact that the polymicrogyria extended
beyond the vascular territories supplied by the dysplasic
arteries suggests that polymicrogyria and vascular dysplasia
are expressions of the same pathogenic process at different
stages of gestation. This case illustrates that the neurocutaneous
syndrome of hemangioma-cerebral dysplasia must be
suspected in children presenting with epilepsy and facial
hemangioma.
FP-H-126
Benign infantile familial convulsions and paroxysmal
choreoathetosis: report of first cast with homozygous
linkage to chromosome 16p12–q12
V. Humbertclaude, A. Poubertie, F. Rivier, J. Rochette, P.
Szepetowski, B. Echenne
Service de Neuropediatrie, Chu Saint Eloi, Montpellier,
France
We report the first case of homozygous linkage to chro-

Abstracts 533
mosome 16p12–q12 associated with benign infantile familial
convulsions and paroxysmal choreoathetosis. A boy
presented two clusters of seizures at 3 and 4 months of
age. Seizure was characterized by left or right head and
eye deviation, hypertonia of one upper limb and tonicclonic
generalization. Ictal EEG showed right temporoparietal
rhythmic spikes followed by generalization. Cerebral
MRI was normal. Six isolated seizures recurred
between 6 and 9 months. At 25 months of age. Paroxysmal
choreoathetosis (PC) appeared, characterized by unilateral
choreic movements, followed by generalized athetosis.
Attacks occurred spontaneously or after repeated movements.
Ictal and inter-ictal video-EEG were normal. Ictal
ECD SPECT showed bilateral basal ganglia and mild left
temporal hyper perfusion. Daily attacks of PC persisted
despite multiple therapy. The grand-aunt of the boy and
her daughter had presented benign infantile convulsions.
No other case of PC was noted. The parents of the boy
were consanguineous. Genetic analysis showed homozygous
linkage to the chromosome 16p12–q12. This homozygous
status may explain the unusual phenotype, with
repeated seizure and severe early-onset PC.
FP-H-127
Quality of life in children with epilepsy – Indian scenario
S. Aneja, N. Prakash, V.R. Nigam
Department of Pediatrics, Lady Hardinge Medical College,
New Delhi, India
Objective: To study the QoL profile in children with
epilepsy. Methods: A prospective (nested control) study
was done among children (age 6–15 years) attending
epilepsy clinic who were on AED for minimum of 6 months.
Children with associated motor/cognitive deficits were
excluded. Evaluation was done by interviewing parents
with part open ended and part structured pretested questionnaire.
It included responses to measure effect of epilepsy on
physical, psychological, social and school domains. Each
item was rated on a five point likert scale. Results: Ninety
five children (males 56; females 39) were studied. Complex
partial seizures (37%) were the commonest type followed by
generalized seizures (25%). Sixty one percent had infrequent
seizures. The structured questionnaire showed good testretest
reliability (r ¼ 0:9), internal consistency (a ¼ 0.9)
and interparental agreement (r ¼ 0:9) QoL was most
affected in the school domain with 71.5% parents perceiving
problems in school. Behavioural and psychological problems
were felt to be affected by 65.2% of parents. Social limitations
were perceived by fewer parents (40%) and physical
domain was least affected (10%). The effect of Seizure severity
and seizure frequency on QoL was studied after adjusting
for age, sex, socioeconomic status and IQ. It was seen that
patients with frequent seizures and severe seizures had lower
scores on QoL. Conclusion: Epilepsy had significant effect
on schooling and behaviour of children with epilepsy.
Management protocol of epilepsy should include complete
assessment of behaviour and psychological functions.
FP-H-128
The risk factors and outcome of children with
intractable status epilepticus
P.A.M. Kunju
Division of Pediatric Neurology, Medical College Trivandrum,
Kerala, India
Objectives: (1) To identify the risk factors; (2) the
immediate outcome; and (3) to assess the neurological
and mental outcome at the end of 1 year in children
with intractable SE. Setting: Paediatric Intensive Care
Unit, medical college. Design: Prospective Study. Methodology:
160 children with intractable generalized SE
during 1998–2001 were studied. The subjects were classified
into 1. those with previous history of epilepsy and 2.
those with no prior history of epilepsy. Children who
recovered were followed up at 6 monthly intervals had
cognitive assessment by a clinical psychologist and were
grouped under two categories: (1) those with neurological
impairment, (i.e. IQ , 80) and or having neurological
sequelae; and (2) those who were normal. A total of 160
cases (56 were infants, 40 between age 1–6 years and 64
belonged to 6–12 years, 86 males and 74 females) were
studied. Sixty-two (38.75%) belonged to the first group
and 98 (61.25%) to the second group. The causes of
previous epilepsy in the 62 were cerebral palsy, meningitic
sequelae, cerebral malformations, metabolic and idiopathic.
Twenty-eight (45.16%) fell in the idiopathic
group and 21 were having non-progressive encephalopathy
(33.87%). Two had heterotopias, one lissencephaly, one
agenesis of corpus callosum, one tuberous sclerosis, one
Sturge-Weber syndrome and one metabolic disorder,
(1.61%), Gauchers disease. Among the patients with
previous epilepsy, problems with anti-epileptic drugs
were the most prevalent, i.e. 36 out of 62 patients
(58.06%). Twelve out of 62 were precipitated by systemic
infections. In the remaining 14, no precipitating factor
(22.58%) was noticed. There were 98 SE with no previous
epilepsy. Of these 65 were having intracranial infections
(66.32%). Twenty-two had prolonged febrile seizures
(22.44%) two had metabolic problems (2.04%) One was
subdural hematoma in a hemophiliac patient (1.02%).
Immediate outcome of SE: Out of the 160 SE 18 died
(11.4%), 14 out of 18 deaths (77.77%) were due to infections
of the CNS. Two (11.11%) were due to metabolic
causes, one was a case of intractable hypoglycemia, and
the other a case of Gaucher’s disease. The remaining two
were children with previous history of epilepsy. They had
more than two episodes of SE. There was history of abrupt
withdrawal of antiepileptic drugs. Sixteen out of the 18
had seizures lasting for .2 h (88% of cases), five had
raised intracranial tension (27%), three had hypotension,

534
Abstracts
(16.6), two had respiratory insufficiency (11.1%), one had
hypoglycemia and one, uraemia (5.5%). Outcome at the
end of 1 year: Out of the 60 children with previous
epilepsy, 56 had neurological impairment (93.6%).
Among the 82 with no history of prior epilepsy, six developed
neurological impairment (6.25%) and 76 (93.75%)
were normal. Relative risk 14.75%. Conclusions: (1)
Most common cause of SE were acute symptomatic
cases with the previous epilepsy. (2) Important contributing
factors for mortality in SE were seizure duration .2 h
and underlying cause. (3) High association was noted
between children with previous epilepsy and mental retardation.
FP-H-129
Clinical analysis of antiepileptic therapy with
individuated-dose valproate in children
J. Wu, Y.-Q. Zhong, X.-L. Xie, W.-G. Hu, M. Wu
Children’s Hospital of Chengdu, Chengdu, Sichuan, China
Objective: The valproate is a kind of wide AEDs, which is
used widely. To evaluate the efficacy of individual dose
VPA in treatment of 61 epilepsy children, 29 were boys,
as well as 32 girls. Method: Nine months ,14 years of age
children with epilepsy in outpatient treated by single-drug
VPA from August 1999 to October 2001 were retrospectively
analysed. Results: The children with general seizure
or partial were 42 and 19, respectively. Their therapeutic
dosage was from 11.25 , 48.49 mg/kg per day, and average
dosage was 22.92 ^ 7.83 mg/kg day. After 5 , 9 days
taking VPA, the valley blood-drug-concentration of VPA
were from 24.5 , 163 mg/l, Its average blood-drug-concentration
was 69.75 ^ 20.75 mg/l. Conclusion: VPA has good
therapeutic effect on epilepsy, which does better for general
seizure than partial seizure. The target dosage in different
children change greatly, and there are a lot of individual
variations. Meanwhile we should select anti epileptic
drugs according to the epilepsy type. In treatment epilepsy,
it is necessary to monitor the constant concentration of VPA
after five half eliminate time.
FP-H-130
Effects of recurrent audiogenic seizures on hippocampal
structure and seizure behavior of P77PMC rats
S.-G. Zhao, X.-R. Wu
Pediatric Neurology, Department of Pediatrics, Peking
University, First Hospital, Beijing, China
To investigate the functional role of hippocampal mossy
fiber (MF) sprouting in the pathophysiological mechanisms
of initiation and propagation of epilepsy, we examined
hippocampal MF synaptic reorganization and changes in
the hippocampal neurons of AGS-prone P77PMC rats at
various stages in the course of recurrent seizures using
Timm’s method of sulfide silver staining and Nissl staining.
Also, we observed the effects of recurrent audiogenic
seizures (AGSs) on the changes of seizure behavior of
P77PMC rats. Our results showed that frequent recurrent
AGSs not only can cause neuronal loss in the CA1 region
of the hippocampus and hippocampal MF sprouting into
the inner molecular layer of dentate gyrus in P77PMC rats,
but can also decrease the latency of IV/V grade AGSs, and
increase the duration of AGSs. Our findings suggest that
hippocampal MF sprouting and neuronal loss are not only
present in limbic seizure, but are also seen in AGS, which
are initiated in brainstem and rapidly generalize. In AGSprone
rats, recurrent AGSs can cause MF synaptic reorganization
and neuronal loss in the hippocampus, and
enhance seizure susceptibility of P77PMC rats. During
the course of recurrent AGSs, enhanced seizure susceptibility
was observed before hippocampal MF sprouting.
This finding may indicate that anatomical changes may
be preceded by functional changes characterized by
elevated excitability in the epileptic brain.
FP-H-131
Clinical and neuro-psychological analysis of epilepsy
patients caused by Japanese encephalitis
D. Ding, Z. Hong, B. Zhou
Institute of Neurology, Fu Dan University, Hua Shan Hospital,
Shanghai, China
Objective: To investigate the morbidity, clinical symptoms,
and brain functions of epilepsy patients caused by
Japanese encephalitis. Methods: Eighty-five patients who
suffered from Japanese encephalitis during 1973–1994
were followed and evaluated using neurological examination,
MMSE, intelligence and memory measurements, and
activity of daily life (ADL). Results: Ten patients were
found to have epilepsy with diverse types, including nine
cases of GTCS and one case of absence petit mal seizures.
Another two cases died of GTCS before evaluation. Totally
12 cases (14.4%) were evaluated as epilepsy, among which
nine cases had seizures beyond 1 year and within 17 years.
Therefore the morbidity of lately occurring epilepsy is
10.6%. The cumulative morbidity of epilepsy occurring
within 17 years after discharge was 16.1% calculated by
survival analysis. Among ten epilepsy patients evaluated,
two cases, six cases and eight cases were confirmed as
MMSE abnormal, mental retardation, and memory defect,
respectively. Four cases were considered as disability
because of the abnormal ADL scores. Conclusion: Epilepsy
with higher morbidity and worse prognosis is one of the
sequelae caused by JE virus infectious during the childhood.
The prevention and control of JE should have certain impact
to decrease the morbidity of epilepsy.

Abstracts 535
FP-H-132
The clinical and EEG characteristics of infancy epilepsy
from temporal lobe origin
J.-P. Liang, F.-C. Cai
Department of Neurology, Children’s Hospital, Chongqing,
China
Introduction: The clinico-pathological data indicated
Temporal lobe epilepsy may manifest differently in infants
and older children. The concept of complex partial
seizures, which may be useful in older children, is difficult
to apply to infants, since it is often not possible to assess
impairment of consciousness in this age group. The aim of
study was to analyze the clinical manifestations, EEG, CT
scan and MRI in a cohort of 32 infancy epilepsy from
temporal lobe origin (IETLO). Materials and methods:
Thirty-two cases with IETLO including 19 males and 13
females were subjected to the study. The onset of IETLO
was between 2 and 34 months with an average of 20
months, and 25 cases were followed up for 1–6 years
with the examinations of EEG, Videotapes, CT and MRI.
Results: IETLO was an age-related epileptic syndrome
characterized by the clinico-electrical features in infants
aged less than 2 years different from those in elder children.
The seizure semiology shown atypical presentations
including initial motionless stare, behavioral arrest or
reduction with possible impairment of consciousness in
27 cases, presenting not react to visual threat or loud
noise during seizures, and that all spontaneous purposeful
activities were interrupted; autonomic features and oroalimentary
or simple manual automatisms in 16 cases;
tonic, versive or myoclonic seizures were observed in 13
cases, which may not indicated a localized origin of the
seizures. Most of seizures lasted for more than 1 min. The
interictal EEG indicated that the epileptogenic discharges
found in infants were less often than in elder children with
single or multiple foci. The ictal EEG discharges were less
focal and variable, even falsely lateralized, and the patterns
were not very distinct, including focal attenuation of beta
activity or sleep spindles, focal slowing and unilateral electrodecremental
events, etc. Video-EEG monitoring was
important in identifying the seizure patterns and impairment
of consciousness. CT scans shown structural cerebral
lesions in 5/6 cases and MRI gave more information of
brain lesions in 11/14 cases, which were more valuable
in localization of lesions than EEG. Conclusion: IETLO
is an age-related epileptic syndrome characterized by the
clinico-electrical features as follows: motionless stare,
behavioral arrest or reduction with possible impairment
of consciousness, autonomic features and oro-alimentary
automatisms; convulsive seizures were prominent; duration
of seizures was more than 1 min. The intericatal discharges
were less found while ictal discharges were less focal and
distinct. Video-EEG monitoring was critical to identify the
seizure patterns and impairment of consciousness. Neuroimagings
are more important in finding the structural cerebral
lesions.
FP-H-134
Efficacy and safety of topiramate for pediatric epilepsy
in clinical practice: data from a postmarketing
surveillance study
Y. Roh a , Y.S. Hwang b
a Department of Pediatrics, Chosun University Hospital,
KwangJu, Seoul; b Department of Pediatrics, Seoul National
University Hospital, Seoul, Korea
Objectives: To evaluate the efficacy and safety of topiramate
in pediatric patients with epilepsy. Methods: TPM
was treated in 561 patients who were taking one or more
antiepileptic drugs or no medication. Topiramate dose was
increased gradually from 0.5 to 1 mg/kg per day weekly or
biweekly. After 16 weeks since the patients started having
TPM, the antiepileptic efficacy was measured. All adverse
events reported during this study period were gathered.
Results: Their mean age was 8 years old (range 3 , 13
years old). A total of 62% of the patients were boys. The
median number of monthly seizures at baseline was 4.5
(mean ¼ 41). The mean duration of epilepsy was 5 years
(^4). Fifty-seven percent of patients were diagnosed idiopathic
or cryptogenic epilepsy and 43% symptomatic
epilepsy. Forty-seven percent of patients had other neurological
disorders like as mental retardation or hemiparesis.
Carbamazepine (52%), vigabatrin (52%), and valproate
(50%) were the most common concomitant drugs. The overall
seizure free rate in 16 weeks from topiramate treatment
was 35%. The .50% seizure reduction rate was 70%.
Seizure free rate in Lennox-Gastaut syndrome (LGS) or
infantile spasm was 18%. Seizure free rate in patients
with topiramate monotherapy was 67%, which was much
higher in patients with combination therapy. Seizure free
rate by monthly baseline seizure frequency was 58% in
less than two times, 27% in three to nine times, 14% in
10–30 times, and 17% in more than 31 times. Seizure free
rate by duration of epilepsy was higher in shorter duration;
70% in less than 1 year and 20% in 11–13 years. A total of
69% of parents with the pediatric patients rated the efficacy
of topiramate good or very good. Seventy percent of medical
doctors rated the efficacy of topiramate good or very
good, 20% fair and 10% poor. The adverse events of TPM
were somnolence (6.0%), weight loss (5.0%), anorexia
(3.7%), and lethargy (3.0%). Conclusions: Topiramate had
high .50% seizure reduction rate (70%) and seizure free
rate (35%) in various seizure types and showed remarkable
seizure free rate (18%) in LGS and Infantile Spasm. The
shorter history of seizure duration was, the higher seizure
free rate of topiramate showed. Drop out due to adverse
effects was 2.8%. This study suggests that topiramate is
the first rank antiepileptic drug in its efficacy and is very
effective for pediatric epilepsy regardless of seizure types.

536
Abstracts
FP-H-135
Efficacy of topiramate monotherapy in pediatric patients
with newly diagnosed epilepsy
K.S. Lee
Department of Pediatrics, ChungNam National University
Hospital, DaeJun, Korea
Objectives: To investigate efficacy and safety of topiramate
as initial therapy in pediatric patients with newly diagnosed
epilepsy. Methods: Among pediatric patients were
brought to the hospital emergency room due to seizure, 36
patients with newly diagnosed epilepsy were recruited for
this study. Topiramate was given to them for 16 weeks and
CBZ and/or VPA was added if seizure was not controlled
with topiramate within 6–8 weeks. Seizure reduction rate
was evaluated as well as adverse events at end point of
16th week. Results: Among 36 patients there were 19 patients
with secondarily generalized seizure (GS), nine with
complex partial seizure (CPS), six with generalized tonic
clonic seizure (GTCS), one with myoclonic seizure, and
one with tonic seizure. Mean age of the patients was 4.6
years and mean duration of epilepsy was 0.7 year (8.4
months). Mean seizure frequency was 35 times per month
and mean weight was 18 kg. Eight patients had other neurological
conditions such as CP or developmental delay. Five
out of 36 patients were added on CBZ and/or VPA in 6–8
weeks since topiramate treatment. Twenty-six patients
(72%) were seizure free and all of them were on topiramate
monotherapy and no seizure free patients were in CBZ and/or
VPA added group. Seizure free rate by seizure type was
100% in GTCS, 78% in CPS, and 63% in secondarily GS.
Three out of 36 patients withdrew due to adverse events or
lost follow-up. Conclusion: Topiramate was highly effective
in controlling seizures of pediatric patients with newly diagnosed
epilepsy, showing 72% of seizure free rate. Topiramate
was effective in various seizure types and considered
‘good or very good’ by more than 80% of parents and investigator.
There were few serious adverse events and basically
all adverse events were mild.
FP-H-136
Efficacy of topiramate for pediatric patients as
replacement therapy of vigabatrin
S.J. Chung
Department of Pediatrics, KyungHee University Hospital,
Seoul, Korea
Objectives: Although many clinical studies of topiramate
(TPM) have been reported, its effect on Asian children was
not evaluated yet. Clinical efficacy of TPM was assessed on
Korean epileptic children whose seizures were not controlled
with more than two kinds of AEDs even at maximum doses.
Methods: A total of 104 patients received add-on therapy of
TPM and 96 patients completed the study. Eight patients
were dropped out at 4th week due to adverse reactions of
TPM. The trial consisted of 8 week baseline phase, 10–16
weeks of titration phase, and 8 week stabilization phase.
Seizure history, adverse events, and drug compliance were
observed at patients visit every 2 weeks. TPM dose was
increased by 1 mg/kg every 2 weeks up to 6 mg/kg, or in
some cases, up to 9 mg/kg. Mean age of the patients was 11
years old (range 2–16 years old) and male patients were 62
(64.5%). A total of 61 (64.0%) patients were on vigabatrin
(VGB) and 65 (68.0%) on carbamazepine. Concomitant
AEDs including VGB were reduced gradually after stabilization
period. Clinical efficacy was evaluated by change in
seizure frequency, i.e. disappearance of seizure (seizure
free), significant improvement (more than 75% of reduction
of seizure), improvement (more than 50% of reduction of
seizure) and no improvement (less than 50% of reduction
of seizure). Results: Out of 96 patients, 65 patients (67.7%)
became seizure free, 22 patients (22.9%) showed more than
50% seizure reduction, nine patients (9.4%) showed less than
50% seizure reduction. When the efficacy was evaluated by
seizure type; 15 (83.3%) showed seizure free and one (5.6%)
showed significant improvement out of 18 patients with
generalized seizure. Three (25.0%) showed seizure free
and 5(41.7%) showed significant improvement or improvement,
while four (33.3%) showed no improvement out of 12
patients with LGS. Forty-seven (71.2%) showed seizure free
and 16 (24.2%) showed significant improvement or improvement
out of 66 patients with partial seizure. TPM showed
higher seizure free rate in less number of concomitant
AEDs: 85.7% (12/14) in one AED, 70.8% (17/24) in two
AEDs, 65.5% (19/29) in three AEDs, 57.1% (13/21) in four
AEDs, and 50.0% (4/8) in five AEDs. Seizure free rate by
duration of epilepsy was 81.8% (9/11) in less than a year,
80.0% (28/35) in 2–4 years, 65.4% (17/26) in 5–7 years, and
45.8% (11/24) in more than 8 years. In the efficacy by baseline
monthly seizure frequency, TPM showed higher seizure
free rate in lower seizure frequency: 41 (93.2%) out of 44
patients whose frequency of seizure was less than once a
month. Fourteen (58.3%) out of 24 patients with one to
three seizures/month, four (50.0%) out of eight patients
with four to five seizures/month, and six (30.0%) out of 20
patients with more than six seizures/month. Seizure free rate
by seizure type was highest in generalized seizure and lowest
in LGS. Global assessment was excellent from 66 (69%) of
patients or physicians, good from 15 (16%), fair from eight
(8%), and poor from seven (7%). Conclusion: It is concluded
that TPM can replace VGB and/or other AEDs without
seizure aggravation or serious side effects.
FP-H-137
Topiramate in pediatric subjects with inadequately
controlled epilepsy: multicenter trial
Y.J. Woo, M.H. Lee
Department of Pediatrics, ChonNam University Hospital,
KwangJu, Korea

Abstracts 537
In order to evaluate the efficacy and safety of topiramate
as adjunctive therapy for uncontrolled pediatric seizures,
multicenter add-on topiramate trial was conducted in
Korea. Methods: Twenty study sites in Korea enrolled
patients. Pediatric epilepsy patients, aged 2–16 years old,
with uncontrolled seizures with conventional antiepileptic
medications were included in this study. After receiving
written informed consent, patients were enrolled in a 4-
week baseline phase, followed by a 24 week trial phase
which consisted of a 12 week titration and a 12 week
stabilization period. Topiramate was added to each
patient’s background antiepileptic drugs at an initial dosage
of 1 mg/kg and titrated to target dosage of 9 mg/kg per day.
Study visits were scheduled at 4-week interval. Results:
Data consisted of 184 patients showed that 152 of 184
patients completed this study. Among 184 patients there
were 24 patients with simple partial seizure, 71 with
complex partial seizure, 53 with secondarily generalized
seizure, and 36 with LGS. During the entire period, the
proportion of patients with 50% or higher reductions in
seizure frequency was 59.2%. Among them 12 patients
(6.5%) were seizure free. During the 12-week stabilization
period alone, the proportion of patients with 50% or higher
reductions in seizure frequency increased to 60.9% and 30
of those patients were seizure free, indicating an increase
of seizure free proportion (16.3%) during the stabilization
period. The median percent seizure reduction was 62.2%
during the entire period and 67.1% during the stabilization
period. There were no significant differences in median
percent seizure reduction among patients with different
epilepsy duration, seizure type, and number of concomitant
antiepileptic drugs. Eighteen patients withdrew from the
study due to lack of efficacy, loss of follow-up, poor cooperation
of parents, and adverse reactions. The most
common reported adverse events were anorexia, somnolence,
mental slowing, gastrointestinal disturbances, and
difficulty with concentration or attention. Most reported
adverse events were mild or moderate in severity. In fact,
there were only eight patients who withdrew from this
study due to adverse reactions. Conclusions: TPM was
safe and effective as adjunctive therapy in the treatment
of uncontrolled pediatric epilepsy patients with simple or
complex partial seizures with or without secondary generalization
and LGS.
FP-H-138
Clinical review of severe myoclonic epilepsy in infancy
H.C. Kang, O. Dambajamts, H.D. Kim
Department of Pediatrics, Inje University, Sang-gye Paik
Hospital, Seoul, Korea
Rationle: Severe myoclonic epilepsy in infancy (SMEI)
seems to be probably more common than realized, because
it is often overlooked. In addition, the etiology is unknown
and the prognosis is very grave despite of newly developed
anticonvulsants. This study is intended to give a help to
suspect and management with reviewing our experiences
about SMEI. Methods: From April 1995 to April 2002,
subjects were selected by as following criteria; frequent
generalized or focal febrile clonic seizures before the 1st
year of life, subsequent or previous afebrile seizures of
various types including myoclonic seizures, progressive
delayed development from a certain period. We reviewed
retrospectively clinical features such as etiology, change of
seizure types, treatment, prognosis, electrophysiologic
features and MRI findings. Results: Among eighteen
patients enrolled to this study, ten patients were male and
eight were female. The mean age of seizure onset and
duration of seizure were respectively 5.1 months (SD ^ ,
2.8) old and 69.9 months (SD ^ , 32.6). Five patients
(27.8%) had familial tendency of seizure and four
(22.2%) had photosensitivity. With related disease, mitochondrial
cytochrome complex IV deficiency was
confirmed in one patient. In seven patients (38.9%), seizure
had been started with febrile illness and the others with
afebrile seizure. Onset of febrile seizure was from 2 to
11 months old (mean ^ SD, 7.8 ^ 3.0). Those features of
beginning seizures were focal clonic in nine patients
(50.0%), generalized clonic in five (27.8%) and focal
with generalized clonic in four (22.2%), as well subsequent
seizures were atonic in one (1.9%), atypical absence in
6(33.3%), complex partial in 16 (88.9%), partial with
secondary generalization in six (33.3%), generalized clonic
in one (5.6%), generalized tonic clonic in ten (55.6%) and
myoclonic in 18 (100.0%). Myoclonic seizure had been
started from 7 to 48 months old (mean ^ SD,
26.5 ^ 12.1) and persisted in 14 (77.8%) patients. There
were afebrile or febrile status epilepticuses in nine patients
(50.0%). Initial neuroimaging studies were normal in all
patients but subsequently diffuse atrophy and encephalomalatic
change could be seen each in one patient. EEG
features of 14 patients who could have been serially
followed, were initially normal, but diverse abnormal findings
including background abnormalities and epileptiform
discharges have been founded with followed. Duration of
follow up to appearance of EEG abnormalities was from 6
to 86 months (mean ^ SD, 28.2 ^ 21.0). All patients were
intractable to anticonvulsants. Ketogenic diet was introduced
in 13 patients, one patient has been seizure-free
for 7 months, one patient has been reduced seizure
frequency about more than 90%, and six patients about
more than 50%. Among seventeen patients who could be
observed for more over 12 months, 15 (88.2%) had
progressive delayed development, mildly in four (22.2%)
patients, moderately in seven (38.8%), and severely in four
(22.2%). Conclusion: SMEI has progressive and diverse
clinical appearances with grave prognosis. Mitochondrial
cytopathy has to be considered with mechanism of SMEI
and ketogenic diet could be effective to SMEI intractable
to antiepileptic drugs.

538
Abstracts
FP-H-139
Behavioral and psychological problems in pediatric
epilepsy: HOPE study
I.J. Seol a , H.D. Kim b
a Department of Pediatrics, HanYang University College of
Medicine; b Department of Pediatrics, InJe University Sanggye
Paik Hospital, Seoul, Korea
Purposes: Health outcome of pediatric epilepsy (HOPE)
study aimed to reveal the incidence of various behavioral
and psycho-social disturbances in pediatric epilepsy patients
and to identify the factors related to various behavioral and
psycho-social disturbances. Method: A total of 827 patients
were enrolled from 37 centers during the 6-month study
period. Five questionnaires were given to patients and
their parents who visited epilepsy clinic during the study
period; Korean version of child behavior check list (K-
CBCL), Yale children’s inventory (YCI), family environmental
scale (FES), children’s depression inventory (CDI),
and Piers-Harris children’s self-concept scale. A set of five
questionnaires was filled out by each patient or its parent.
Parents checked out K-CBCL, YCI, and FES and patients
checked YCI and Piers-Harris scale. In case which patients
were too young to understand the questions, YCI and Piers-
Harris scale were excluded but other three scales were
checked by their parents. All scales were analyzed based
on etiology, duration of epilepsy, seizure frequency, number
of AEDs, and presence of complications. Results: Male
patients was 56% and the enrolled patients were 18 years
old and under. Children aged between 6 and 12 years old
was the most with 59%. Based on etiology idiopathic
epilepsy was 78%, crytogenic was 8% and symptomatic
was 14%. Ratio of partial and generalized epilepsy was
65% versus 35%. In terms of number of antiepileptic
drugs (AEDs) 2% of patients did not take any AEDs and
66% of patients had been taking one AED. A total of 32% of
patients had been taking two AEDs and more. Duration,
seizure frequency and number of AEDs were related to
poor results in K-CBCL and YCI. FES showed no significant
difference in all categories. The longer the duration was
the higher CDI score was. In comparison with normal children,
Piers-Harris scale was lower in children with epilepsy.
Conclusions: Various kinds of psycho-social and behavioral
problems are frequently associated with pediatric epilepsy
especially in cases of older age, more frequent seizures,
prolonged duration of treatment and/or using multiple
AEDs.
FP-H-140
Comparative analysis of handedness and footedness in
children with epilepsy
S.R. Ahn, S.O. Nam
Department of Pediatrics, College of Medicine, Pusan
National University, Busan, Korea
Purpose: Study for handedness and footedness of patient
with epilepsy is rare. A few studies suggest that left handedness
is more in children with epilepsy. We analyzed correlation
of handedness and footedness of children with epilepsy
according to the cause of epilepsy and site of brain lesion.
Methods: Subjects were 130 epileptic patients who visited
pediatric ambulatory clinic of Pusan National University
Hospital from June 2001 to August 2001. Controls were
130 children with no history of convulsion or neurologic
problem. We let check them by carrying out and inquiring
ten items about use of hand and foot. We defined as left or
right handedness and footedness by carrying out more than
five items dominantly. We analyzed age, type of seizure,
cause of epilepsy and site of brain lesion in symptomatic
group. Results: In 130 epileptic patients, left handedness
and left footedness were 20.0 and 15.7% which are significantly
higher than 4.0 and 5.6% in control group (P , 0:05).
But there was no significant difference between idiopathic
epilepsy group and control group as 8.9 and 9.3%. However
left handedness in symptomatic epilepsy group was 45.0%
and left footedness was 31.4%, which were significantly
higher than those of control group (P , 0:05). According
to the site of brain lesion in symptomatic group, all patients
with abnormality in left hemisphere showed left handedness,
but only 57% of the patients showed left footedness. In the
patients who have abnormality in both hemisphere or diffuse
brain lesion, left handedness was 25.9% and left footedness
was 36.8%. Concordance rate of left handedness and left
footedness was 80.0% in control group, 87.5% in idiopathic
epilepsy group and 76.9% in symptomatic epilepsy group.
Conclusion: Left handedness and footedness are more
common in epileptic patients than normal control group.
This is mainly due to high proportion of left handedness
and left footedness in symptomatic epileptic patients.
FP-I
Neuromuscular Disorders
FP-I-001
Assessment of the respiratory function in Duchenne
muscular dystrophy patients
A. Lissoni
Valduce Hospital, Como, Italy
Objective: The study is aimed at defining the diagnostic
value and the prognostic reliability of several methods of
respiratory assessment in relation with the progression of the
ventilatory failure in DMD patients. The timely diagnosis and
definition of respiratory insufficiency level arethe basic stepto
start the non-invasive ventilatory support treatment. Methods:
The results of respiratory functional tests carried out on more
than200DMDpatientsina16yearsperiodhavebeenanalysed
and grouped on the basis of the subjects’ age, in a continuum
between the ages of 11 and 31. Mean values and standard
deviations of VC, VC%, MVV, MVV%, Pimax, Pemax,

Abstracts 539
PaO 2 and PaCO 2 were calculated for each age group and
graphs were plotted to show the respective trends. Results:
The diagnostic- prognostic significance of the different tests
changes at different ages: the most appropriate tests as well as
those less significant are identifiable on the basis of the disease
progression. Conclusions: The respiratory assessmentof these
patients is particularly justified from age of 11–12 years on;
between 11 and 20 years VC, VC% and Pimax values are
useful to determine the residual possibilities of the ventilatory
pump. The blood gas analysis, already known to be useful
after the age of 17–18, becomes a guiding criterion in respiratory
function testing after the age of 20. The study of sleep
patterns by oxymetry and transcutaneous capnometry is advisable
after the age of 13 and compulsory after the age of 15.
FP-I-002
Follow up of children with corticospinal tract
involvement in Guillain-Barre syndrome (GBS)
N. Barisic, L. Brcic, R. Likic
Department of Pediatrics, Zagreb Medical School, Clinical
Medical Center Zagreb, Zagreb, Croatia
GBS is characterized by areflexia.Hyperreflexia is
reported in AMAN. We present 13 children with GBS at
the age 14 months–12 years with hyperreflexia or positive
Babinski sign in initial (5/13) or in early recovery period (10/
13) and 2/13 in both periods. The children were examined
clinically and electromyoneurographically 2–5 times successively
during 1–7 years of follow up. Cerebrospinal fluid
protein content was increased initially in seven patients without
pleocytosis. Meningitic syndrome was present in 7/13
children. Compound muscle action potentials (CMAP) in
range 0.1–1 mV (mean 0.39 mV) and motor conduction
(CMNV) ranging 15–33.6 m/s (mean 26.07 m/s) were
decreased in nine children in the initial phase. Neural potentials
and F waves were absent initially in 11 patients. Distal
latency was prolonged in nine patients. On follow up
improvement of CMAP occurred in six children. Improvement
of CMNV was registered in four children. Outcome was
excellent in 7/13 in the period 1 month–7 years. In 1/13
remained spastic monoparesis, while five patients manifested
slight peroneal gait. Hyperreflexia appeared usually in the
recovery period suggesting involvement of upper motor
neurons or spinal interneurons occurring both in GBS of
demyelinating and axonal type in children.
FP-I-003
Motor neuron disease (MND) in a girl associated with
neuropathy, mitochondrial abnormalities and
centromeric deletion of survival motor neuron (SMN)
gene on chromosome 5q13
N. Barisic, J. Sertic, L. Pazanin
Department of Pediatrics, Zagreb Medical School, Clinical
Medical Center Zagreb, Zagreb, Croatia
Childhood SMA is the most common MND. Homozygous
centromeric survival motor neuron (SMNc) gene deletions
are rarely described in SMA but are present in 36% of
lower MND cases. We present a 12-year-old girl of normal
psychomotor development. At the age of 8, she developed
progressive walking difficulties. On examination, peroneal
gait, left leg hypotrophy, radicular pain, absent triceps sure
jerks and bilateral Babinski sign were registered associated
with extensive right leg angiokeratoma. Brain CT and MRI
spinal scan were normal. Clinical and electromyographical
progression of neurogenic lesion and decreasing of motor
nerve conduction occurred during 4 years, followed by
involvement of left upper extremity and development of
cavus foot on the right. Cerebrospinal fluid, immunologic
tests and alpha-galactosidases activity (Fabry disease) were
normal. Increased vascular resistance without stenotic
abnormalities was recorded on both legs by Doppler ultrasonography.
DNA analysis revealed SMNc deletion. Muscle
biopsy showed neurogenic atrophy and accumulation of
abnormal mitochondria. Sural nerve biopsy showed
decreased number of myelinated axons with scarce onion
bulbs. SMNc deletion probably acts as factor of increased
susceptibility for MND. Although SMN gene deletion
detection is useful in atypical SMA it might be coincidental
finding in MND associated with clinical features of other
pathogenetic origin.
FP-I-004
Early onset progressive chronic inflammatory
demyelinating polyneuropathy (CIDP) associated with
CNS demyelination and inflammatory myopathy
N. Barisic, L. Pazanin, Z. Hutinec, L. Sojat-Cvitanovic, M.
Trbojevic-Cepe
Department of Pediatrics, Zagreb Medical School, Clinical
Medical Center Zagreb, Zagreb, Croatia
Chronic inflammatory demyelinating polyneuropathy
(CIDP) is a chronic disorder, which manifests usually
with monophasic or relapsing course. We present a boy,
now at the age of 6, with progressive generalized muscle
weakness since the age of 2, developing after viral infection.
On examination, generalized hypotonia and hypotrophy of
small muscles, peroneal gait, positive Gowers’ sign,
areflexia, and right abducens palsy were registered. Electromyoneurography
showed severe neurogenic lesion, low
compound muscle action potentials, slowed motor nerve
conduction and myopathic pattern in proximal muscles.
Increased protein content was obtained in the cerebrospinal
fluid. Muscle biopsy showed neurogenic atrophy. Sural
nerve biopsy revealed demyelination and onion bulbs.
Inflammatory perivascular CD 3 positive infiltrates were
present in both biopsies. Brain MRI showed cortical atrophy
with hyper intensities of the white matter and gray matter
hypo intensities. Increased serum anti-G M1 (IgM) and anti
G D1b (IgG) antibodies were registered. Very long chain fatty

540
Abstracts
acids, B 12 , folic acid, lactate, and immunologic test were
normal. He rapidly improved on IVIG and methylprednisolone
treatment, while upon tapering off the steroids
complete deterioration occurred. Demyelination might
develop in the central and peripheral nervous system associated
with inflammatory myopathy in patients with
progressive course of CIDP.
FP-I-005
Acute inflammatory polyradiculoneuropathy in
childhood: clinical features, electrophysiological studies
and treatment
S.B. Yahmed, I. Turki, N. Gouider-Khouja, N. Miladi, F.
Hentati
National Institute of Neurology, Tunis, Tunisia
Acute inflammatory polyradiculoneuropathy (AIP) is the
most common form of acute polyneuropathy in Tunisia
since 1992 when acute anterior poliomyelitis was eradicated.
Thirty-six children with acute inflammatory polyradiculoneuropathy
were analysed from 1974 to 2001. The
mean age of onset was 6 years. There was a predominance
of male 2:1. One half had a prodromal illness within a 4-
week period before the onset of AIP. The illness affected the
upper respiratory tract in 27% of patients and gastro-enteritic
infection in 3% of them. Immunization was implicated in
10% of patients. Weakness, particularly of the lower limbs,
was the initial complaint in 89% of patients. In three patients
paresthesias were present without detectable weakness. A
rise in the level of cerebrospinal fluid protein in the absence
of a pleiocytosis was found in 98% of patients. Electrophysiological
studies showed marked slowing of motor conduction
velocities along with prolonged distal latencies
consistent with demyelination in 54% of patients. Axonal
involvement was identified in 17% of patients. Supportive,
symptomatic treatment was the mainstay of therapy until
1995. The last 6 years, a high-dose gammaglobulin (0.4 g/
kg per day for 5 days) was given for the first days of the
disease and has been shown to reduce the duration of the
hospitalization, to avoid respiratory insufficiency and to
limit the extension of the paralysis
FP-I-006
Genetic diagnosis in childhood Duchenne muscular
dystrophy
H. Cai, L.-W. Wang, G.-Z. Xu, J.-X. Wu, Y.-Y. Li, Y.-L.
Zhu
Capital Institute of pediatrics, Beijing, China
Objectives: DMD is the most common hereditary neuromuscular
disease in children. Its incidence is 1:3000 live
born boys whose abnormal gene is on the X chromosome
at the Xq21 locus and is one of the largest gene identified.
Because the genealogical research of childhood DMD have
not been widely reported in our country, our study aimed at
exploring the genealogical gene of the disease as well as the
relationship between the gene deletion and the level of lysosomal
enzymes, establishing the pediatric gene laboratory in
the north area. Methods: By using the method of the PCR,
blood samples from 30 DMD patients and their parents were
screened for the genes deleted. Serum lysosomal enzymes
were detected with biochemical auto analyzer. Results:
There were significant differences in the levels of lysosomal
enzymes between DMD and normal children (P , 0:01).
The positive rate of the gene deletion was 56.7% (17),
among which the positivity rate of No. 55 was 64.7%
(11), the No. 48 was 52.9% (nine), No. 45 was 35.2%
(six), No. 44 was 17.6 (30), No. 12 was 11.8% (two), and
each of Nos. 8, 17, 19 was 5.9% (one). The positivity rate of
monogenic deletion was 47% (eight), multigenic deletion
was 52.9% (nine), and the genes deleted in parents was 0.
Conclusion: (1) Levels of lysosomal enzymes in DMD are
obviously higher than that in healthy children, and
decreased with the severity of DMD. (2) PCR assay was a
convenient and sensitive method for detecting the genes
deleted in DMD. Our result corresponds to the literature
that the rate of multigenic deletion among these patients
are 47%. (3) The result of our research is important for
studying antenatal diagnosis further.
FP-I-007
Mutations in the skeletal muscle a-actin gene in patients
with nemaline myopathy
Y.-J. Jong a , C.-Y. Yuo b , B.S.-C. Mak c , M.-Y. Chung d , S.-P.
Lin e , C.-C. Huang f
a Department of Pediatrics and Clinical Laboratory,
b Department of Biology, Kaohsiung Medical University,
Kaohsiung; c Department of Pediatrics, Taichung Veterans
General Hospital, Taichung; d Department of Pediatrics,
Chang Gung Memorial Hospital, Kaohsiung; e Department
of Pediatrics, Mackay Memorial Hospital, Taipei; f Department
of Pediatrics, National Cheng Kung University Hospital,
Tainan, Chinese Taipei
Namaline myopathy (NM) is a clinically heterogeneous
condition characterized by the presence of abnormal rodlike
structures in the muscle and ranging in severity from
neonatal onset with early death to adult onset with slow
progression. Recently mutations in the skeletal muscle a-
actin (ACTA1) gene have been found in up to 30% of the
patients with NM. In order to compare the frequency and
position of mutations in the ACTA1 gene in Taiwanese
patients with NM with those in Western countries, we
analyzed the ACTA1 gene for mutations in the six Taiwanese
patients with NM by sequencing PCR products of
genomic DNA containing the coding exons 2–7. We identified
two different heterozygous missense mutations in two
out of the six patients. Both mutations, Tyr69Asn and
Gly197Ser, have not been reported before in patients with

Abstracts 541
NM. In addition, these two mutations gave rise to restriction
digestion variants that were not found in DNA from all
controlled individuals analyzed. We also demonstrated by
DNA sequencing or restriction endonuclease digestion that
the two ACTA1 mutations identified in the patients were de
novo mutations. In conclusion, our results show that the
frequency of the ACTA1 mutation in Taiwanese patients
with NM is similar to that in patients of Western countries.
The identification of two novel mutations in the ACTA1
gene also reflects the genetically heterogeneous nature of
NM.
FP-I-008
Long-term corticoid treatment in Duchenne muscular
dystrophy
B.F. Reitter a , R. Korinthenberg b , M. Stötter c , U. Schara d ,R.
Lindemuth e
Departments of Pediatrics, Universities of Mainz a , Freiburg
b ,Tübingen c , Bochum d , and Homburg/Saar e , Germany
A total of 125 boys with Duchenne muscular dystrophy
have been treated openly and daily with Deflazacort, 0.75
mg/kg resp. Prednisone, 0.9 mg/kg (N ¼ 5). Checks at 3
months intervals: internal and neurological status, timed
motor function tests, blood: cell count, CRP, ALAT,
ASAT, CK, Gluc, Ca, P, bAP, triglycerides, electrolytes;
additionally after 6 months: osteocalcin in urine, ophthalmolog.
control; add. after 12 months: manual muscle test
(twice), joint mobility, bone densitometry, X-ray hand,
ultrasound abdomen, muscles, heart, ECG, spirometry.
Treatment started above age 5 years, has lasted 2 months
to 8:3 years, and was stopped at loss of ambulation (la)
(N ¼ 22, age 7–14 years). Seven boys older than 14 years
are still walking, some still able to hop on one foot. There
was no obvious relation between age at start, duration of
med and la. Effects and side effects varied considerably. In
more than 75% of patients the decline of motor abilities
diminished. Apart from la, medication was stopped only
due to unaccepted weight gain (N ¼ 14), progressing cataract
(N ¼ 1, cataracts total, N ¼ 27), bone fractures (N ¼ 3,
total, N ¼ 7) due to osteopenia. Those who gained weight
above two SD, did so in the 1st year and remained at
approximately the same percentile (PC). Growth fell
below the 3rd PC in all. No increased frequency or severity
of infections were noted. In no case dilative cardiomyopathy
developed. Summarizing, the positive effects outweigh
negative ones in part of the patients; a strict protocol
allows judging the individual balance within 1 year of
treatment.
FP-I-009
Study of the pathgenesis of 31 GBS cases
B. Li, Y.-P. Yang
Shen Zhen Shi Children’s Hospital, Shen Zhen, China
Objective: We measured the serum antibodies IgG and
IgM to ganglioside G M1 in 31 patients with Guillain-Barre
syndrome, in order to study the pathogenesis of GBS. Methods:
We measured the serum antibodies IgG and IgM to
ganglioside G M1 by enzyme-linked immunosorbent assay,
in 31 acute-phase and recovery patients with Guillain-
Barre syndrome (AIDP 23, AMAN eight), with two control
groups (non-GBS neurological diseases and normal
subjects). Results: The results revealed that the serum levels
of anti-G M1 IgG and IgM with GBS were much higher than
those of the other control groups (P , 0:05). Their cerebrospinal
fluid levels also were higher than those of normal
groups (P , 0:05). Conclusion: The finding indicates the
immunological damage caused by gangliosides is an important
factor in GBS, especially in AMAN.
FP-I-010
Genetic heterogeneity of the axonal form of autosomal
recessive Charcot-Marie-Tooth disease (ARCMT2)
Group 1: M.A.M. Salih a , M. Kabiraj b , M. Al-Rayess c ,T.
Maisonobe d , M.H.S. Al-Turaiki e , F.J. Al-Shammary f , J.A.
Urtizberea g , D. Grid h . Group 2: H. Azzedine, A. Brice, E.
LeGuern
Group 1: a Division of Pediatric Neurology, Department of
Pediatrics; b Clinical Neurophysiology Unit; c Department of
Pathology, College of Medicine, Department of Clinical
Laboratory Sciences, College of Applied Medical Sciences,
King Saud University, Riyadh; e The JCRPO, Riyadh, Saudi
Arabia, d Service de Neuropathologie, Hopital de La Salpetriere,
Paris; g AFM, Evry and Genethon, Evry, France.
Group 2: INSERM U289, Federation de Neurologie, Hopital
de La Salpetriere, Paris, France
The axonal autosomal recessive phenotype of Charcot-
Marie-Tooth disease (ARCMT2) is relatively frequent in
the Arabian Peninsula and North Africa because of the
high rate of consanguinity. The first locus for ARCMT2
was recently mapped to chromosome Iq21–q21.3 in a
Moroccan family and a mutation in Lamin A/C gene was
reported in Two Algerian families, whereas another locus
was mapped to chromosome 8q21.3 in a Tunisian family
who had an associated pyramidal tract involvement. The
corresponding gene, GDAP1 was identified in three
families from Spain. We describe three consanguineous
families who have eight children (four males and four
females; aged 16 months–13 years) affected with
ARCMT2. Onset was at birth (with foot deformities) in
the first two families (six males and two females) and 6–
7 months in the third family (two females). All patients had
delayed motor development. Cognitive development was
normal in six children belonging to the first two families
who had distal muscle weakness associated with scoliosis
in three of them. The two girls in the third family were
mentally retarded, had epilepsy, showed distal and proximal
muscle weakness and were bed-ridden. In seven exam-

542
Abstracts
ined children, the peroneal motor nerve conduction velocity
ranged between 40.4 and 59.5 m/s and muscle
compound action potential (CAP) was reduced (0.35–
0.56 mV) in five of them. The peroneal CMAPs could
not be recorded in another two patients. The sural sensory
CAP was absent in five cases and abnormal in another two.
The two already known loci for axonal ARCMT were
tested in these three families. These loci were excluded
except the 8q13 locus for the first family. The sequencing
of the coding region of GDAP1 is in progress for this
family. These data further demonstrate the genetic and
phenotypic heterogeneity of the ARCMT2.
FP-I-011
Infantile neuroaxonal dystrophy (INAD). Clinical
spectrum of eleven Brazilian cases, diagnosed through
conjunctival biopsy
S. Rosemberg, D. Fujiwara
Santa Casa of São Paulo Medical School, Department of
Pediatrics, Neuropediatrics Division, São Paulo, Brazil
INAD is a rare neurodegenerative disease whose metabolic
defect is unknown. A recent review disclosed only 44
reported cases between years 1990 and 2000. The diagnosis
is based on the finding of spheroid bodies of peripheral
axons on ultrastructural level. We studied 11 patients diagnosed
through conjunctival biopsies. There were six females
and five males. All came from unrelated families. Consanguinity
was present in six cases. The age of onset varied
between 6 and 24 months (mean: 15 m). The ages at the time
of the diagnosis varied between 20 months and 6.5 years.
The time elapsed between onset and diagnosis varied from 4
months and 6.5 years (mean: 2 years 8 months). The disease
started insidiously, with initial arrest followed by loss of the
psychomotor development. Only one patient achieved independent
walk. At admission, all patients were profoundly
demented. Only a few had some visual contact. The major
neurological sign concerned a severe global hypotonia particularly
prominent in the inferior limbs. In five cases, the
patients presented a ‘frog position’ being almost paraplegic.
Tendon reflexes were hypoactive or absent in seven cases
and brisk in four. Babinski sign was found in all patients. All
patients but one presented brisk, rapid, irregular movements
of the eyeballs. Head circumference was normal even in
older patients. CSF was always normal. Funduscopic
exam disclosed optic atrophy in ten patients. EMG
(n ¼ 6) showed denervation in five. Cranial TC or MRI
revealed cerebellar atrophy in all cases. Two patients died
at ages 6 and 8 years. In summary, a child presenting with
slowing of psycho-motor development beginning under 2
years, severe hypotonia mainly of the lower limbs, abnormal
ocular movements, optic atrophy, denervation and cerebellar
atrophy is highly suspected of having INAD and an
ultrastructural exam of the nerve terminals should be
performed.
FP-I-012
Peripheral neurophysiological and neuropathological
studies in Charcot-Marie-Tooth atrophy disease in
children
J.-L. Lu
Department of Neurology, Beijing Children Hospital, Beijing,
China
Objective: Charcot-Marie-Tooth atrophy disease (CMT)
is one of the most common hereditary neuropathies in children.
The diagnosis will be difficult because of lacking the
available and specific genetic tests. In this study, the authors
characterized the clinical features of CMT in children and
analyzed the value of the neurophysiology test and the sural
nerve biopsy in the diagnosis and classification of different
subtypes of CMT. Methods: Fifteen patients with CMT were
investigated during the years 1991–1999. Their clinical and
experimental data, including nerve conduction studies,
somatosensory and brainstem auditory evoked potentials
and nerve pathology study were analyzed. Results: All 15
patients (eight males and seven females) were characterized
by progressive weakness of four or two extremities, the
course of the disease varying from 1 to 11 years; three of
15 patients had a family history of CMT. The patients were
classified into CMT type I (hypertrophic type) and CMT
type II (neuronal type) according to the neurophysiological
and pathological criteria. The following features were found
in CMT type I patients: the average age of onset at 2.2 years
of age, all extremities involvement in seven, pes cavus in
eight, the appearance of stork leg in five, sensory abnormalities
in three. Five patients showed a decreased nerve
conduction velocity (12–38 m/s); six patients showed
demyelination in their sural nerves (moderate changes in
five and severe in one), onion bulb formations were found
in two patients. CMT type II was found in seven patients
with the following features; average age of onset at 7 years,
all extremities involvement and reduced amplitudes of
compound muscle active potential without conduction slowing.
The sural nerve biopsy in three patients demonstrated a
chronic axonal neuropathy. Conclusion CMT can be classified
into two subtypes. The peripheral neurophysiology test
is a reliable method to diagnose and classify CMT, while the
sural nerve biopsy may further support the diagnosis and
confirm the subtype classification.
FP-I-013
Clinical and genetic profile of spinal muscular atrophy in
Oman
R. Koul
University Hospital, BW-1, Children ward one, Alkhod,
Oman
Oman is one of the Gulf countries with a population of
about 1.5 million. In last 10 years about 60 patients of spinal

Abstracts 543
muscular atrophy in children were seen, majority being
Werdnig-Hoffmann disease. There was positive family
history in large number of cases. The genetic work up was
started in last few years. A detailed clinical and genetic
work up will be presented.
FP-I-014
Outcome of Guillain-Barre syndrome in children: Pre
and post intravenous immunoglobulins (IVIG) era
R. Koul
University Hospital, BW-1, Children ward one, Alkhod,
Oman
Oman is one of the Gulf countries with a population of
about 1.5 million. Forty-one children were prospectively
followed from 1992 till December 2001. All received
IVIG 400 mg per kg per day for 5 days. Eight children
before 1992 did not receive IVIG and were picked up retrospectively
from medical records. There was not much difference
in age group, sex distribution and onset of weakness in
both the groups. There was a significant difference in other
parameters. The hospital stay was prolonged in preIVIG
group (mean of 45.4 days) as compared to post IVIG
group (20.4 days). Three out of eight (37.5%) required
ventilation in preIVIG group while 19.5% in post IVIG.
Residual neurologic deficit was 43 % in preIVIG as
compared to 5% in post IVIG group. One child died in
preIVIG group (12.5%) and there was no mortality in the
post IVIG group though the number of patients was five
times larger. IVIG has definitely improved the outcome in
Guillain-Barre syndrome.
FP-I-015
Juvenile onset dentatorubral pallidoluysian atrophy:
follow-up MPI findings
T. Nakayama, H. Oguni, M. Funatsuka, M. Osawa
Department of Pediatrics, Tokyo Women’s Medical University,
Tokyo, Japan
Purpose: Dentatorubral pallidoluysian atrophy (DRPLA)
is a rare autosomal dominant neurodegenerative disorder
that is mostly prevalent in Japan. Although the clinical,
neouropathological and neuroradiologic findings of adult
onset DRPLA have been well described, they are poorly
understood in childhood onset DRPLA. In this study, we
analyzed the MRI findings, including sequential MRI
changes with age, to reveal the relationship between clinical
symptoms and neuroradiologic findings in childhood onset
DRPLA. Subjects: The subjects were four patients with
childhood onset DRPLA diagnosed by molecular biological
method. The expansion number of CAG repeats ranged
from 68 to 71. The age of onset of clinical symptoms ranged
from 6 to 11 years, with intellectual deterioration in two and
seizures in two. The follow-up period was from 22 to 24
months with a mean of 18.5 months. Method: MRI scan was
performed with T1, T2, FLARE method with 7-mm slices.
The axial, coronal and sagittal planed were recorded.
Results: A total of 16 MRI imaging studies were performed,
ranging from 3 to 9 in each patient. Progressive atrophy of
the cerebrum and cerebellum, and enlargement of the
lateral, third, and fourth ventricles were observed, which
correlated with the progression of dementia and ataxia.
Restoration of the red nucleus was not observed. Conclusion:
The degree of progressive cortical atrophy and enlargement
of the ventricles roughly corresponded to the degree
of dementia and ataxia. None of our patients showed involvement
of the basal ganglia, which may be related to the
lack of involuntary movement in our cases.
FP-I-016
Myasthenia gravis in Chinese children: Hong Kong
perspective
C.-W. Fung, B.H.-Y. Chung, V.C-N. Wong
Department of Paediatrics, The University of Hong Kong,
Queen Mary Hospital, Hong Kong, China
This study aims to analyze the clinical features, investigations,
treatment strategies and outcome of Chinese children
with myasthenia gravis (MG) in Hong Kong. A
comparison was made between Chinese and Caucasians.
Fifty-seven children with MG were identified in our unit
from 1992 to 2002. The records were reviewed. Out of the
57 children, the female to male ratio was 6 to 4. Fifty
patients (88%) had ocular MG while the remaining ones
had generalized MG. The mean age of onset of the disease
was 4.1 years old. Only four patients (7%) had identifiable
precipitating factors such as respiratory tract infections.
Majority of the patients presented with unilateral or bilateral
ptosis. Anti-acetylcholine receptor antibodies were positive
in 29 patients (52%). All the 57 children received anticholinesterase
initially. Only 13 of them (23%) required addition
of steroid therapy. Four patients (7%) required
intravenous immunoglobulin therapy and thymectomy was
performed in six patients (10%). Thirty-one children
(54.4%) entered into remission. Among this group, nine
(29%) had relapse. In conclusion, considerable differences
were observed among Chinese and Caucasians in the clinical
presentations and outcome of this disease.
FP-I-017
Nerve conduction studies in Guillain-Barre syndrome of
children
Q. Zhang, L. Wang
Children’s Hospital, Chonqing University of Medical
Sciences, Chongqing, China
Objective: To investigate the nerve conduction changes in
GBS patients, the nerve conduction records of 162 children

544
Abstracts
with GBS from 1994 to 2001 were retrospectively studied.
All patients came from rural area or suburbs of southwest
China, including 110 males and 52 females. The onset of
disease was from 9 months to 16 years (43 cases were less
than 3 years; 119 cases were older than 3 years). Results:
The nerve conduction studies suggested that 44 patients
(27.1%, group A) were categorized as acute inflammatory
demyelinating polyneuropathy (AIDP); 77 patients (47.6%,
group B) were recognized as acute motor axonal neuropathy
(AMAN); 41 patients (25.3%, group C) were classified as
AMSAN. The electrophysiologic abnormalities were
detected within the 1st week in 88 of 162 cases (54.3%).
Among them, 22 cases (25%) were less than 3 years, 66
cases (75%) were older than 3 years. Three nerve conduction
patterns detected in two different age groups were similar.
Thirty-four patients were followed up for a period from
1 month to 2 years. Five of 34 (14.7%) still had the nerve
electrophysiologic abnormalities, among them four cases
(11.8%) were in group A, 17 cases (50%) in group B, and
eight cases (23.5%) in group C. Conclusions: This study
suggested that AMAN was the most common type in children
with GBS, it is also not easy to recover; nerve conduction
detection was valuable for GBS early diagnosis, even to
infant and child patients.
FP-I-018
Secondary merosin deficiency CMD linked to
chromosome 19 in two Tunisian families
C. Triki, N. Louhichi, S. Rouissi, M. Méziou, C. Mhiri, H.
Ayadi, F. Fakhfakh
Department of Neurology and Laboratory of Human Biological
Molecular, Sfax, Tunisia
Congenital muscular dystrophy (CMD) is a heterogeneous
group of muscular disorder characterized by autosomal
recessive mode of inheritance. The CMD have been classified
according to the involvement of the brain and the eyes. It
is apparent that defects in different genes are responsible for
each type. We have studied two cases affected with CMD and
belonging to two Tunisian families. These patients have
benefited from complete clinical investigation. Immunohistochemical
and Western blot analyses on muscles biopsies
was performed using antibodies against human merosin 80
and 300 fragments. Linkage analysis was undertaken for
members of two consanguineous families using microsatellite
markers spanning LAMA2 (6q22), FCMD (9q31), MEB
(1p32), CMD 1B (1q42) and FKRP (19q13.3) loci. Clinical
investigation of the two patients showed severe motor delay,
mental retardation and calf hypertrophy. Cranial MRI
showed white matter abnormalities in two patients associated
with cerebellar cysts and vermis hypoplasia in one case.
Immunohistochemical and Western blot analyses revealed
a partial deficiency of merosin in the two cases. DNA analysis
excludes linkage to chromosomes 6, 9 and 1 and showed a
linkage to chromosome 19q13.3.
FP-I-019
Genetic and immunohistochemical studies of congenital
muscular dystrophy
M. Yoshioka a , T. Toda b , I. Nishino c
a Section of Pediatric Neurology, Kobe City Pediatric and
General Rehabilitation Center for the Challenged, Kobe;
b Division of Functional Genomics, Osaka University Graduate
School of Medicine, Osaka; and c National Institute of
Neuroscience, National Center of Neurology and Psychiatry,
Tokyo, Japan
The CMD are a heterogeneous group of autosomal
recessive disorders presenting in infancy with muscle
weakness, contractures, and dystrophic changes on skeletal-muscle
biopsy. Recently, a selective deficiency of
highly glycosylated alpha-dystroglycan (a-DG) became
apparent in several forms of CMD, including FCMD,
muscle-eye-brain disease, CMD with mutations in the
fukutin-related protein gene and myd mouse. A-DG is a
peripheral membrane protein linking the extracellular
basal lamina to the cytoskeletal proteins. In Japan, most
CMD patients have FCMD, characterized by severe CMD
associated with brain and eye malformations. Here, we
analyzed three Japanese patients from two families with
FCMD-phenotype. Full mutational analysis of the fukutin
gene, however, revealed neither 3 kb insertion (Japanese
founder mutation) nor point mutation. RT-PCR analysis of
RNA isolated from lymphoblasts of a patient revealed a
normal expression of the FCMD transcript. As classification
of CMD should be based on the genetic background,
our present cases might be a new variant of CMD. Immunohistochemical
analysis of dystrophic muscle in one
patient revealed a selective deficiency of a-DG, but not
beta-DG, merosin, sarcoglycan or dystrophin. These findings
raise the possibility that the abnormality of a-DG is
integral to the pathology in this variant of CMD.
FP-I-020
Benign acute childhood myositis
F.M. Aynacı, M.Çakır, Ş. Yayla
Karadeniz Technical University, Faculty of Medicine, Dept
of Child Neurology, Trabzon, Turkey
Six children, one girl and five boys, aged 3–9 years,
were admitted with benign acute childhood myositis during
January–February 2002. They presented with an acute
onset of symmetrical calf muscle pain and tenderness,
weakness and inability to walk a few days after upper
respiratory tract infection. Serum creatine kinase and
aspartate aminotransferase were increased all cases. Viral
studies included throat swab for nasopharyngeal aspirate
was done in one case and found to be negative for influenza
A and B. Full clinical recovery was achieved within
12 h–3 days.

Abstracts 545
FP-I-021
Persistent toe-walking, pain and stiffness as leading
symptoms of hereditary neuropathy with liability to
pressure palsies (HNPP)
T. Lönnqvist, H. Pihko
Hospital for Children and Adolescents, University of
Helsinki, Finland
Although molecular determination of the chromosome
17p11.2 deletion in conjunction with electrophysiology are
powerful tools in the diagnosis of hereditary neuropathy with
liability to pressure palsies (HNPP), this neuropathy is underdiagnosed,
especially in children. Most patients present with
mononeuropathies related to mild pressure trauma, but some
present with chronic generalized neuropathy. Clinically
atypical forms of HNPP have been described including
CMT-like polyneuropathy, chronic recurrent inflammatory
demyelinating polyneuropathy, and respiratory failure with
proximal muscle weakness. We present an atypical HNPP
case with very persistent, asymmetric secondary toe-walking
together with pain and stiffness especially in the legs. Our
patient is a previously healthy boy, the only child of healthy
parents, who came to our hospital because of secondary toewalking
at the age of 3.5 years. He had walked normally
without support since 12 months of age, but after an episode
of limping of his left leg for a week at the age of 3 years, he
started to walk on his toes. The clinical examination revealed
an alert boy, whose psychomotor development was normal,
but who persisted to walk on toes. His calves were thick, the
ankles were rigid, and the deep tendon reflexes were exaggerated
in the lower extremities with positive Babinski sign
in the left. A central nervous system process was excluded by
normal brain and spinal MR image, peripheral neuropathy
with normal EMG and muscle dystrophy with EMG and
normal serum concentrations of creatine kinase and transaminases.
Physiotherapy was started with plaster cast treatment
in both legs. In spite of the treatment, and especially
when physiotherapy was discontinued, our patient preferred
toe-walking and suffered from muscle pain and stiffness in
the mornings. Hard exercise in the previous day worsened the
pain. During the follow-up the symptoms and findings were
asymmetric being more severe in the left. At the age of 7
years, when the boy started to go to school, a new symptom
appeared: his hands became weak, when he exercised writing.
At the same time his father complained of numbness in
his hands after hard work. He also told that his sister had had a
brachial plexus nerve entrapment, which was cured without
any treatment in a couple of months. This information after 5
years follow-up made us to perform DNA tests, which
revealed 17p11.2 deletion in the patient, his father and his
paternal aunt and grandmother. To conclude it is important to
remember possibility of HNPP in young patients with persistent,
asymmetric toe-walking added with muscle pain and
stiffness.
FP-I-022
Study of radial nerve conduction in children recording
with surface electrode
L. Wang, F.-C. Cai
Children’s Hospital, Chongqing University of Medical
Sciences, Chongqing, China
Objective: To explore the reliability recording radial
nerve conduction with surface electrode in children and to
investigate the norms and the maturation course of radial
conduction in children. Methods: (1) A comparative study in
40 children with different ages at same radial nerve for the
reliability to record radial motor nerve conduction with
surface and also needle electrodes; and (2) maturation
course and norms of motor and sensory conduction of radial
nerve recorded by surface electrode on the basis of data of
128 children aged 0–14 years old. Results: (1) A clear
pattern of complex motor active potential (CMAP) was
recorded by surface electrodes as by the needle electrodes
in all of the tested children regardless their age. The value of
motor conduction velocity (MCVs) and latencies were
highly consistent by both methods. (2) With surface recording,
CMAPs were well presented in all age groups including
neonate. F waves could be obtained also in all subjects if
moving recording electrode to the proximity of upper extremity.
However it was hard to record a sensory nerve potential
(SNP) of radial nerve in infants younger than 6 months
of age, but the incidence of SNPs were increased to 50%
with age before 3 years old and still less than 90% in older
children; (3) a series of norms of radial nerve conduction
tested with surface electrode in children, including MCV,
SCV, distal latencies, amplitudes and duration of CMAPs
and SNAPs were obtained. A two-thirds level of MCV as
the young adult had been reached at birth, there were no
significant difference between the levels of MCV in 3 years
old group and in the older age groups. As MCVs, a similar
maturational course for SCV was demonstrated. Conclusion:
Motor nerve conduction of radial nerve could be satisfyingly
recorded with surface electrodes, instead of needle
electrodes, in children at any age. It could be the most
advantage to promote its clinical use in pediatric population
due to their non-injury operation.
FP-I-023
Physiological and pathological studies in Charcot-
Marie-Tooth disease in children
J.-L. Lu, H.-S. Wu, Z.-Q. Lang
Department of Neurology, Beijing Children’s Hospital,
Beijing, China
Objective: Charcot-Marie-Tooth disease is one of the
most common hereditary neuropathies in children. The
diagnosis can be difficult due to the lack of availability of
specific genetic tests. In this study, we characterized the

546
Abstracts
clinical features of this illness in children and analyzed the
value of neurophysiology and sural nerve biopsy in the
diagnosis and classification of different subtypes. Method:
Fifteen patients with CMT were investigated between 1991
and 1999. Their clinical and experimental data, including
nerve conduction studies, somatosensory and brainstem
auditory evoked potentials and nerve pathology study
were analyzed. Results: All 15 patients (eight male, seven
female) were characterized by progressive weakness of four
or two extremities, the course of disease varying from 1
through 11 years; three of the patients had a family history
of CMT. The patients were classified into CMT type I
(hypertrophic type) and CMT type II (neuronal type)
according to neurophysiological and pathological criteria.
The following features were found in CMT type I patients:
average age of onset at 2.2 years of age, all extremities
involved in 7, pes cavus in eight, the appearance of stork
leg in five, sensory abnormalities in three. Five patients
showed a decreased nerve conduction velocity (12–38 m/
s); six patients showed demyelination in their sural nerves
(moderate changes in five and severe in one), onion bulb
formations were found in two patients. CMT type II was
found in seven patients with the following features: average
age of onset at 7 years, involvement of all extremities in
three, pes cavus in five and stork leg in three patients; all
patients showed reduced amplitudes of compound muscle
active potentials without conduction slowing. Sural nerve
biopsy in three patients demonstrated a chronic axonal
neuropathy. Conclusion: CMT is characterized by chronic
progressive weakness and atrophy in all or lower extremities
and it can be classified into two subtypes. The neurophysiological
and pathological features were corresponded with
chronic demyelinating neuropathy in type I and axonal
neuropathy in type II. Electrophysiological test is a reliable
method for diagnosing and classifying different subtypes.
Sural nerve biopsy provides objective evidence in making
adefinitive diagnosis.
FP-I-024
The study of the value of SEP for diagnosis in pediatric
paralysis
N. Xiao, X.-H. Lan, C.-G. Feng, L. Wang
Department Neurology, Children’s Hospital, University of
Medical Sciences, Chongqing, China
Objective: To explore the diagnostic value of somatosensory
evoked potentials (SEP) for children with paralysis.
Method: Comparing the normal data of SEP along median
and posterior tibial nerves in 180 healthy children aged 0–14
years old, the SEP of 335 patients aged from 2 months to 14
years old with extremity paralysis were analyzed and
compared. Results: Among the 266 subjects with spastic
paralysis, abnormal SEP was present in 230/266 cases
(86.5%). The latent period records of spinal and/or scalp
SEP peak were showed delay or complete absence, but
their peripheral potentials were still within normal range.
The SEP abnormalities were higher in 92.6% (25/27) of
spinal diseases cases and 86.3% (139/161) of cerebral
palsy cases. Both were more than those in other CNS
diseases. In the cases with intracranial lesions, the abnormal
SEP in supratentorial lesions were more common than that in
postfovea lesion. In the 69 children with flaccid paralysis,
20.3% (14/69 patients) presented abnormal peripheral potentials
of SEP. Comparing the abnormalities rate of SEP, there
was no significant difference between the two age groups,
older and younger than 3 years old. Conclusion: The results
of the study indicate that SEP was very important to determine
the possible site of lesions pathologically particularly
for spinal and cortical lesions. It was suggested that SEP was
also a valuable diagnostic test for the paralysis in infants.
FP-I-025
Evaluation of the reasons of delayed diagnosis in
Duchenne muscular dystrophy patients
S. Kurul, E. Dirik
Dokuz Eylül University Faculty of Medicine, Department of
Pediatric Neurology, Izmir, Turkey
Duchenne muscular dystrophy is a severe X-linked recessive
neuromuscular disease that causes death in the second or
third decade. The clinical manifestations are not recognized
until 3–4 years of age. The early diagnosis of this incurable
disease allows potential benefits including the avoidance of
distressing diagnostic delays, enabling the family to plan for
future pregnancies and prenatal diagnosis, and the offer of
physiotherapy at an early stage. This study has shown that the
diagnosis continues to be delayed. The failure to recognize
the walking problems by families and doctors in affected
boys has been found to contribute to the late diagnosis of
this disorder. We conclude that all clinicians involved in
the assessment of children including general practitioners,
orthopedists and pediatricians should recognize the common
patterns of presentation of children with Duchenne muscular
dystrophy and either refer to a paediatric neuromuscular
diseases center or measure creatine kinase level.
FP-I-026
A clinical description of cases of acute flaccid paralysis
reported in Malaysia over a 5 year period of surveillance
from 1997 to 2001
T.G.S. Thomas c , M.T. Arif a , H. Ihmi b , S. Ali b , T.B. Khoo c ,
D. Kurup b , M. Sinniah b
a Chairman and b Members, Poliomyelitis eradication expert
review committee, Ministry of Health, Malaysia; c Paediatric
Neurology Clinic, Paediatric Department, Penang
Hospital, Penang, Malaysia
Objective: The nation-wide surveillance for acute flaccid
paralysis (AFP) was implemented in Malaysia in 1995 and

Abstracts 547
further intensified in 1996 as part of the World Health
Organisation (WHO) certification process for polio eradication
in the Western Pacific region. Clinical data on AFP
cases during a 5-year surveillance period from 1997 to
2001 were collected and analysed. Results: 517 cases of
AFP were reported during the surveillance period; the
overall rate of AFP was 1.38 per 100 000 children below
15 years old. The major clinical diagnoses associated with
AFP were Guillain-Barre syndrome (29.4%), central
nervous system infection (16.2%), transverse myelitis
(10.6%), non-polio enterovirus infection (6.2%), and hypokalaemic
paralysis (5.2%). There was an excess of CNS
infections and non-polio enterovirus infection due to enterovirus
71 infections occurring in 1997 and in the year
2000. There were no cases of poliomyelitis due to wild
poliovirus, based on the WHO virological classification.
Three cases were ‘polio compatible’ and 61 cases
(11.8%) remained unclassifiable as ‘non-polio AFP’.
There were no cases attributed to vaccine-associated
paralytic polio. Conclusion: These data suggest the absence
of circulation of wild poliovirus in Malaysia from 1997 to
2001. The Malaysian AFP surveillance mechanism has
improved over the years though there was still a need to
improve on the adequacy of stool specimens collected and
post-paralytic clinical follow-up.
FP-I-027
A case of opsoclonus-myoclonus syndrome: a molecular
observation on pre-and-post treatment of high dose
steroid therapy
K. Oishi a , H.J. Okano b , T. Tokoro a , M. Kurauchi c ,H.Ota a,c
a Department of Pediatrics, Jikei University School of Medicine,
Tokyo; b Department of Physiology, Keio University
School of Medicine, Tokyo; c Department of Pediatrics,
Kanagawa Prefectural Atsugi Hospital, Atsugi, Japan
Introduction: Opsoclonus-myoclonus syndrome (OMS)
is a rare disorder with abnormal eye movement, cerebellar
ataxia and myoclonus. The onset is acute and the outcome
is variable depending on the severity of the disease and the
response to therapies. The pathogenesis is still unknown.
However, the clinical response to immunotherapy and the
expression of autoantibodies suggest that dysregulation of
the immune system is involved. Objective: To investigate
the pathogenesis and the efficiency of the treatment, we
analyzed the expression level of autoantibodies in our
patient. Methods: A 2 year-old female of OMS was treated
with high dose steroid (30 mg/kg of intravenous methylprednisolone)
for 3 days. Sera were obtained before and
after the therapy, and were used for Western analysis.
Mouse tissue lysates were used for this assay. Results:
The treatment was successful, and all of the symptoms
disappeared in 2 months. She has been well with several
episodes of minor recurrence on a low dose of steroid.
Autoantibodies were detected in serum from the patient
before therapy. Their targets were 75 and 85 kD proteins
in the cortex, cerebellum, and midbrain. The expression
level was dramatically decreased by the therapy. Conclusion:
Autoantibody was detected in our patient and their
expression levels were decreased with high dose steroid
therapy. This coincided with an improvement in clinical
symptoms. This suggested that high dose steroid therapy
was effective in reducing the expression of autoantibodies
and monitoring
FP-I-028
Focal critical illness neuropathy in children
M.J. Tan, R.H. Kandler, P.S. Baxter
Sheffield Children’s Hospital, Western Bank, Sheffield,
United Kingdom
We describe a hitherto unrecognised association of focal
neuropathy with critical illness. Four children requiring
intensive care for critical illness developed clinical and electrophysiological
features of focal neuropathy. Signs have
persisted with some partial recovery in three. Two had a
generalised polyneuropathy as well, which recovered.
None had deletions in the CMT1a region on chromosome
17. Patient 1 (girl 2 years) had right peroneal nerve palsy
diagnosed 6 months after culture positive meningococcal
septicaemia which required ventilation for 10 days, complicated
by focal skin necrosis, encephalopathy, a generalised
movement disorder and a right hemiparesis. She had a deep
necrotic lesion over the head of the right fibula. Patient 2
(boy 6 years) had right peroneal nerve palsy diagnosed 5
months after type C meningococcal septicaemia, requiring
ventilation for 15 days and complicated by DIC, renal failure,
focal skin necrosis, and central motor signs. Patient 3
(girl 14 years) had right peroneal nerve palsy diagnosed
after resolution of right calf compartment syndrome requiring
fasciotomy, complicating type C meningococcal septicaemia.
She also developed adult respiratory distress
syndrome and polyneuropathy, requiring ventilation for 2
months. Patient 4 (girl 13 years) had left peroneal and tibial
nerve palsies following acute focal myositis associated with
influenza A infection, which also caused severe asthma
requiring ventilation for 5 days. She had electrophysiological
evidence of a generalised axonal polyneuropathy which
recovered. We suggest that peripheral nerves already at risk
from critical illness neuropathy are more susceptible to
damage from local pressure.
FP-I-029
Spinal muscular atrophy type I, hypertension and
neuropathy: a case report
M. Sahin, D. Dayangac, G. Haliloglu, H. Erdem, H.
Topaloglu
Hacettepe Children’s Hospital, Department of Pediatric
Neurology, Ankara, Turkey

548
Abstracts
Spinal muscular atrophy (SMA) is an autosomal recessive
disorder characterized by progressive loss of motor neurons
of the spinal cord. There are three types defined according to
age of onset of symptoms, clinical course and different clinical
severity. Homozygous mutations or deletions in telomeric
copy of survival motor neuron gene located on
chromosome 5q are found in more than 90% of the patients
in the classical form. SMA type I begins in the first few
months of life, muscle weaakness is predominantly proximal
and the facial muscles are spared. The criteria for differentiating
SMA type I from non-5q-linked diseases include
involvement of extraocular muscles and diaphragm and
pronounced facial weakness. A 2-month-old male infant
was admitted to our hospital with respiratory insufficiency
and hypotonia. There was first degree consanguinity. He had
signs of congestive heart failure, bilateral metacarpophalangeal
contractures, tongue fasciculations, abdominal breathing,
hypotonic posture, and absent deep tendon reflexes. His
blood pressure was 160/85 mmHg. Congenital hypomyelinating
neuropathy, hereditary sensorymotor/autonomic
neuropathies were included in the differential diagnosis
besides SMA type I. Molecular analysis revealed that SMA
gene deletion was positive and EMG showed severe and
diffuse motor demyelinating neuropathy with secondary
axon loss, mild sensory polyneuropathic involvement and
active neurogenic process. SMA gene deletion, axonal
neuropathy and hypertension were reported in two families
before. In our patient, presence of severe demyelinating
neuropathy, SMA, contractures and hypertension can be a
different clinical entity.
FP-I-030
Merosin-positive congenital muscular dystrophy:
characteristics of 59 patients. Hacettepe experience
G. Haliloglu, B. Talim, E. Demir, M. Yetuk, G. Kale, H.
Topaloglu
Hacettepe Children’s Hospital, Department of Pediatric
Neurology, Ankara, Turkey
CMD are a heterogenous group of autosomal recessive
disorders characterized by early-onset muscle weaakness,
hypotonia, joint contractures and a myopathic and/or a
dystrophic pattern at musscle biopsy. We present a total
of 59 patients (29 girls, 30 boys) diagnosed with merosinpositive
CMD with an age range of 7 months–21 years.
These patients are subgrouped in four according to clinical
course; severe (n ¼ 18) and mild (n ¼ 14) and presence of
distal laxity (n ¼ 15) and rigid spine syndrome (n ¼ 12).
Clinical presentation, physical examination findings, creatine
kinase (CK) levels, muscle ultrasonography (US), electrophysiologic,
muscle biopsy, cardiac evaluation and
neuroimaging findings are all documented taaking defined
subgroups into consideration. Clinical findings included
decreased intrauterine movements (10/59), neonatal hypotonia
(21/59), consanguinity (40/59), delay in motor milestones
(49/59), contractures (26/59), mental retardation (6/
59), and increased CK levels (17/59). Muscle US showed
grades 3–4 echogenicity in 90–95% of the patients. EMG
was performed in about 50% of the patients and only two
were neuropathic. Cardiac evaluation showed diastolic
dysfunction in some of the patients. However, none of
these patients were symptomatic. Neuroimaging findings
were normal in 21/23 and showed cortical atrophy in 2/23.
CMD represents clinically and genetically heterogeneous
group of disorders. Defining subgroups based on clinical
presentation and physical examination findings will help
further to classify and define molecular aspects of the
disease.
FP-I-031
A novel variant of spinal muscular atrophy with
progressive myoclonic epilepsy
G. Haliloglu, H. Erdem, Z. Akcören, Y. Renda, H.
Topaloglu
Hacettepe Children’s Hospital, Department of Pediatric
Neurology, Ankara, Turkey
SMA is a clinically and genetically heterogeneous
disease characterized by loss of motor function and muscle
atrophy due to anterior horn cell degeneration. The most
common variant is chromosome 5 linked proximal SMA,
ranging in severity from congenital onset and infantile death
to onset in adult life. Genetically separate variants with
different distribution of weakness and/or additional features
such as central nervous system involvement have been
described. A rare variant with associated myoclonic
epilepsy and lower motor neuron disease has been
previously described in three families. We report four
patients from two additional families affected by a
syndrome characterized by severe and progressive myoclonic
epilepsy and proximal weakness, tremor and lower
motor neuron disease proven by electrophysiologic and
muscle biopsy findings. Extensive metabolic investigations
including tests for neuronal ceroid lipofuscinosis, lysosomal
storage diseases and mitochondrial disorders were negative.
Genetic analysis excluded the SMN gene. This study
presents two families who differ from the patients defined
in the literature before and confirms that the association of
myoclonic epilepsy and motor neuron disease represents an
entity genetically separate from chromosome 5 linked SMA.
Metabolic and genetic defect remains unknown.
FP-I-032
Respiratory capacity course in patients with infantile
spinal muscular atrophy
C. Ioos, B. Estournet-Mathiaud, A. Barois, S. Mrad, D.
Leclerc-Richard
Department of Pediatric Neurology, Hôpital Raymond
Poincaré, Garches, France

Abstracts 549
We present the retrospective study of respiratory course
in patients with infantile SMA. We report 36 patients with
SMA type I for onset of symptoms before age 3 months (I
pure form); 43 patients with SMA type I for onset of symptoms
between 3 and 6 months (I bis form); 135 patients with
SMA type II for onset of symptoms between 6 and 18
months; 12 patients with SMA type III for onset of symptoms
after age 18 months. In patients with SMA type I pure,
75% of patients are dead. One third of patients had tracheotomy,
42% of them are dead. Death is due to ventilatory
insufficiency and bulbar symptoms. In patients with SMA
type I bis, 52% of patients needed nasal nocturnal ventilation
(NNV). Fifty-nine % of patients had tracheotomy.
Twenty-three % of patients are dead. In patients with
SMA type II, 39% of patients needed NNV, 16% of patients
needed tracheotomy. Four % of patients are dead. In patients
with SMA type II, we compared three groups: patients born
before 1975, between 1975 and 1985, between 1985 and
1995. This shows that earlier respiratory management
allows to protect the lung function for more a long time,
and reduces the need for tracheotomy. In patients SMA type
III, respiratory impairment is moderate, and begins during
second decade of life.
FP-I-033
Congenital myasthenic syndromes: a clinical study of 23
children
C. Ioos, L. Viollet, A. Barois, B. Estournet-Mathiaud
Department of Pediatric Neurology, Hôpital Raymond
Poincaré, Garches, France
Congenital myasthenic syndromes (CMS) constitute a
heterogeneous group of disorders in which the safety margin
of neuromuscular transmission is compromised. CMS are
classified as presynaptic, synaptic or post-synaptic according
to the site of the primary defect. We report the clinical
findings of 23 children with CMS. Age of onset vary from
1st day of life to the age of 9. The first clinical features are
more frequently swallowing difficulties (48% of cases) and
respiratory distress (44% of cases), then ptosis (26% of
cases) and fatigability of limb muscles (22% of cases).
Ptosis is a frequent clinical symptom (83% of cases), as
well as swallowing difficulties (61% of cases). Ophthalmoparesis
is present in 52% of cases, and occurs during the
course of the pathology. The muscle weakness predominates
on cervical muscles and limb-girdle muscles in 78%
of patients. In 55% of them, pelvic-girdle muscles are more
severely affected than scapular-girdle muscles. There is an
improvement of weakness and fatigability during childhood
in 74% of patients, stability in 17% of them, and worsening
in 9% of patients. There is a restrictive respiratory insufficiency
in 87% of patients, with stability or improvement in
90% of them. Twenty-two patients are treated. Seventyseven
% of them respond favorably to cholinesterase inhibitors.
This response to treatment is incomplete and needs
high doses reaching 4 mg/kg per dose £ 4/day for pyridostigmine,
and 2 mg/kg per day in four or five divided doses
for ambenomium. Morphologic studies in routine histochemical
studies show no abnormality or type 1 fiber
preponderance isolated or combined type 2 fibers atrophy.
FP-I-034
Merosin-deficient congenital muscular dystrophy: lack
of specificity of proton magnetic resonance spectroscopy
associated to MRI in eight patients
C.C. Leite a , M.T.C. Lacerda a , M.O.R. Costa a , M.G.
Otaduya a , L.G. Ferreira b , M.B.D. Resende b , M.S.
Carvalho b , S.K.N. Marie b , U.C. Reed b
Departments of
a Radiology and b Neurology, School of
Medicine, São Paulo University, São Paulo, Brazil
To evaluate the extensive brain white matter changes in
merosin-deficient CMD we performed proton magnetic resonance
spectroscopy (1h-MRS) associated to MRI in eight
patients (seven with total and one with partial merosin deficiency).
MRI included axial T1, T2, Flair and diffusion
weighted images (DWI). Enhanced T1-weighted images
were obtained in axial, coronal and sagittal planes. The 1 H-
MRS consisted of STEAM (stimulated echo acquisition
mode) sequence in frontal and parieto-occipital white matter
and parieto-occipital gray matter. Metabolite ratios (N-acetylaspartate,
choline and myo-inositol) were studied relatively
to creatine and water. The apparent diffusion
coefficient (ADC) was measured for the same regions.
ADC and 1 H-MRS results were compared to a control. All
patients presented involvement of supratentorial white
matter that predominated in frontal lobes; one patient had
diffuse involvement, and the external capsules were involved
in all, while internal capsules, corpus callosum, optic radiation,
cerebellum and brain stem were not affected. Lesions
were hypointense on T1, hyperintense on T2 and Flair and
did not enhance after gadolinium. No characteristic pattern in
the metabolite ratios relatively to creatine was seen, but by
measuring metabolite ratios relatively to water, important
dilution of metabolites was observed for the white matter,
compared to normal. ADC was increased only in the white
matter. It was a non specific finding consistent with the metabolite
dilution observed by 1 H-MRS, and correlated with the
demyelination observed on MRI.
FP-I-035
Congenital muscular dystrophy: report of two atypical
cases
H.V. Linden Jr., M.B.D. Resende, L.G. Ferreira, M.S.
Carvalho, S.K.N. Marie, U.C. Reed
Department of Neurology, School of Medicine, São Paulo
University, São Paulo, Brazil
We report two atypical cases of CMD. Case 1, a 9-month-

550
Abstracts
old boy, presented from 7 months of age a progressive flaccid
paralysis and loss of head control. The child acquired
head control and was able to sit unsupported at respectively
2 and 6 months of age. A 5-fold increased creatine kinase
serum level and abnormal myogenic pattern on electromyography
were detected. Muscle biopsy revealed marked
dystrophic pattern with no signs of inflammatory changes.
Merosin, sarcoglycan and dystrophin immunostaining were
normal. After 12 months any improvement was observed.
Case 2, a 4 year-old girl, manifested from birth severe
muscle hypotonia, muscular weakness and elongated face.
Her maximal motor ability was walking with support from 3
years of age. MRI revealed diffuse leucodystrophic pattern
and creatine kinase level was 2.5-fold increased. Muscle
biopsy showed marked dystrophic pattern and total absence
of merosin immunostaining. The girl became able to walk
independently at 5 years and 9 months. At 6 years and 5
months she gradually developed worsening of muscle weakness
and of walking again needing support. Deflazacort 0.75
mg/kg per day was started and strong improvement with
return to the anterior walking ability was noted. After 9
months walking persists stable and no side effects developed
except mild weight gain. Subacutely severe course as in
merosin-positive Case 1 and benign course plus additional
improvement under steroid therapy in totally merosin-deficient
Case 2 represent unusual clinical phenotypes to our
knowledge not yet described for the correspondent subtypes
of CMD.
FP-I-036
Juvenile myasthenia: clinical findings, treatment and
prognosis
B. Anlar, A. Deǧerliyurt, S. Aysun, H. Topaloglu, M.
Topçu, G. Turanlı, D. Yalnizoǧlu, E. Özdirim
Hacettepe University, Department of Pediatric Neurology,
Ankara, Turkey
Clinical and laboratory data of 27 patients being followedup
with juvenile (autoimmune) myasthenia are presented.
Mean age of onset is 10.1 (1.5–15) years. The F:M ratio is
7:1 in disease starting after age 9, and 2.7:1 before age 8.
Ocular weakness is the most common initial complaint: it
was present in 85.2% of patients, and as the only symptom
in 62.9%. Among the 17 patients who presented with ocular
weakness only, 3 developed systemic or bulbar weakness in 4
months–2 years, but 14 remained restricted to ocular muscles
in average 6.5 years’ follow-up. Myasthenic crisis was seen
only in six female patients with pubertal onset. In general,
33% of patients had mild, 44%, moderate, and 22%, severe
disease. Acetylcholine receptor antibodies were present in
82% of all cases, and in 71% of those with ocular symptoms
only. The rate of seropositivity did not differ between patients
in remission or those with moderate to severe symptoms.
Treatment was done with acetylcholineterase inhibitors in
all patients. In addition, 18 patients received glucocorticoids.
Those who experienced myasthenic crisis were treated with
intravenous immunoglobulin or plasmapheresis. Thynectomy
was performed in ten patients: five of them have been
in remission for 3–11 years, and four had severe symptoms
and myasthenic crisis within 4 months–8 years after surgery.
These results illustrate the clinical profile of juvenile
myasthenia with predominantly ocular disorder and good
response to treatment in the majority of cases.
FP-I-037
Immunoreactivities of GDNF and its receptors in
various neuromuscular disorders
N. Murakami a , R. Sakuta a , T. Murai a , T. Nagai a , I. Nonaka b
a Dokkyo University School of Medicine, Koshigaya Hospital,
Koshigaya;
b National Center of Neurology and
Psychiatry, Kodaira, Japan
Objective: Glial cell-line derived neurotrophic factor
(GDNF) belongs to multifunctional cytokines and is a
member of motor neuron survival factors. It has been
reported that it is expressed in diseased muscles including
amyotrophic lateral sclerosis (ALS) and DMD. However, its
function in muscle remains unknown. To clarify how GDNF
functions in muscle, we investigated immunoreactivities of
GDNF and its receptors, GDNF receptor alfa-1 (GFR alfa-1)
and receptor re-arranged during transfection (Ret) in various
neuromuscular disorders. Methods: We examined muscle
biopsies from ten patients with, five with Werdnig-Hoffmann
disease (WHD), five with ALS, five with myotonic dystrophy,
five with myotubular myopathy (MM) and five normal
controls. We stained these frozen sections with anti-GDNF,
GFR alfa-1 and Ret antibodies. Results: GDNF-positive
immunoreactivity was observed on subsarcolemma or sarcolemma
of atrophic fibers in WHD and ALS. On the other
hand, GDNF-positive reactivity was found in cytoplasm of
regenerating fibers in DMD. GFR alfa-1 and Ret-positive
immunoreactivities were observed in regenerating fibers
but not in atrophic ones. Conclusion: (1) GDNF may be
expressed in atrophic fibers as a target-derived trophic factor,
resulting in inducing reinnervation in motor neuron diseases.
(2) GDNF could be related with the process of muscle regeneration
because GDNF-Ret system is described to be
concerned with cell signaling of cell proliferation.
FP-I-038
Autonomic function in spinal muscular atrophy
H. Arai a , Y. Tanabe b , Y. Kohno c
a Department of Pediatrics, National Chiba Hospital;
b Department of Neurology, Chiba Children’s Hospital;
and c Department of Pediatrics, Graduate School of Medicine,
Chiba University, Chiba, Japan
Purpose: SMA is a degeneration disease of the anterior
horn cells of the spinal cord, classified into three types based

Abstracts 551
on the age of onset. Scoliosis, joint contracture, pneumonia
and metabolic acidosis have been known as complications
of the disease. However, to our knowledge, there is no
extensive study regarding autonomic function in SMA.
The purpose of this study is to investigate autonomic function
for improving care in SMA. Patients and methods: Six
patients (three males and three females) with SMA aged 2–
24 years old (three in type 1, two in type 2, one in type 3)
and nine healthy age-matched controls were studied by
sympathetic skin response (SSR), hunting reaction, heart
rate variability (RRIV) and oral glucose tolerance test
(OGTT). SSR was performed by electrical and auditory
stimulation, and recorded from the both palm. Hunting reaction
was performed that right index finger into iced water
examined skin temperature. RRIV at rest was examined.
OGTT was checked blood pressure and pulse rate for 2 h
after oral glucose intake. Results: In two patients the difference
of SSR amplitude between right and left palm was
higher than in controls. RRIV in a patient was higher than
in controls. One/6 case had hypotension after glucose intake
with OGTT. Three/6 cases were abnormal in hunting reaction.
Conclusion: It was suggested that there was autonomic
dysfunction in some patients. Careful repeated evaluation of
the autonomic function should be required in SMA.
FP-I-039
Schwartz-Jampel syndrome: clinical documentation of
six families
H. Özyürek a , S. Nicole b , E. Demir a , B. Fontaine b ,H.
Topaloǧlu a
a Hacettepe University Faculty of Medicine, Department of
Pediatric Neurology, Ankara, Turkey; b INSERM U546,
Faculte de Medecine, Pitie-Salpetriere, France
Schwartz-Jampel syndrome (SJS; chondrodystrophic
myotonia) is a rare autosomal recessive disorder characterized
by atypical facies with blepharospasm, dwarfism, bone
and joint abnormalities, and myotonia with an EMG
pattern of continuous muscle fibre activity. According to
the age of onset, two different types are distinguished.
Type 1 (SJS1) is the most frequent form, and usually
accompanied with moderate bone dysplasia. Type 2
(SJS2) is more severe, and manifested by severe skeletal
abnormalities and feeding difficulties. Several other
features, such as mental retardation or immunodeficiency,
have been sporadically associated with SJS. A positional
cloning approach allowed identifying the gene responsible
for SJS1. The SJS1 locus was localized on chromosome
1p34–p36.1 and was found to be genetically homogeneous.
Thereafter, some of us demonstrated that loss of function
mutations in the gene encoding perlecan were responsible
for SJS1. Perlecan is the major heparan sulfate proteoglycan
of basement membrane. Recent data suggest that perlecan
is necessary for the clustering of acetylcholinesterase
at neuromuscular junction, suggesting that the SJS1 myotonia
results from a deficiency in acetylcholinesterase. By
contrast, there is no linkage between SJS2 and the SJS1
locus, and the genetic basis of SJS2 is still unknown. We
present seven patients diagnosed with SJS1 from six unrelated
families. Our follow-up period is 5–15 years. Consanguinity
was present in all. First complaints were noticed
between 1 and 2 years of age in all of our cases. The main
clinical findings in our series included characteristic
appearance of facies; blepharospasm, ptosis, and orospasm,
shortness in stature, contactures of joints, and severe
myotonia. The mental status of the children was normal.
EMGs consistently revealed continuous myotonic activity
without any cessation in sleep. There were bone changes in
the plain films (especially acetabular dysplasia). In one
case coxa valga and in the other coxa vara deformity had
been corrected by surgery. We used carbamazepine as an
antimyotonic agent, and a mild relief was observed in the
myotonic component. Genetic linkage analysis showed that
all our patients were linked to the original SJS1 locus.
Subsequently, mutations were detected in two of the
families. The results of the others are pending. In conclusion,
we analysed the natural history, clinical, radiological
and genetic aspects in seven children with SJS. As far as
we know this is one of the largest series reported.
FP-I-040
Critical illness myopathy associated with prolonged
neuromuscular blockade in a 22-month-old female
P. Drigo a , L. Rigon a , A.M. Laverda a , E. Pegoraro b
b Department of Paediatrics and a Department of Neurological
and Psychiatric Sciences, University of Padua, Padua,
Italy
Clinical conditions characterized by quadriplegia developing
after prolonged stays in the Intensive Care Unit (ICU)
are reported with increasing frequency in pediatric age as
well as in adults. Their etiology may be neurogenic,
myogenic or correlated with neuromuscular transmission
blockade; forms with a combination of these factors have
also been described. We report on a 22-month-old girl
hospitalized at the ICU for sepsis and respiratory deficiency,
with no prior neuromuscular pathologies, intubated for 14
days and treated with high-dose methylprednisolone,
pancuronium for 48 h and vecuronium for 8 days. On
suspending the curarization, the child revealed quadriplegia
with positive deep tendon reflexes. Serum creatine kinase
levels were normal; the electromyogram showed signs of
myogenic damage. Testing with neostigmine showed a valid
return to spontaneous mobility of the four limbs. Muscle
biopsy 18 days after extubation revealed modest, non-specific
signs of myopathy, with a prevalence of type I fibers and
no alteration of the myofibrillary structure or selective loss
of the thick myosin filaments. A progressive improvement
was observed, with complete resolution of the weakness
within 7 weeks of extubation. We conclude that the patient’s

552
Abstracts
quadriplegia was due to a myopathic component (critical
illness myopathy) overlapping the pharmacologicallyinduced
neuromuscular transmission blockade, exacerbated
by the use of corticosteroids and the concomitant renal deficiency
and plasma acidosis. This blockade lasted longer,
however, than is usually reported in the literature.
FP-I-041
Role of genetic analysis in definition of nosological
diagnosis in pathology of peripheral motoneuron in
pediatric patients
L.G. Khachatrian, V.M. Studenikin
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific Center of Child Health (Russian Academy of
Medical Sciences), Moscow, Russia
Background: Clinical features of spinal cord anterior horn
cell lesions Include palsy, hypotonia, reduced or absent
reflexes, muscular weakness and delay in motor milestones.
Aim: To determine the role of molecular genetic analyses in
nosological diagnostics of neuron pathology. Methods: Case
histories of 28 pediatric patients (aged 3–18 months) with
clinical Signs of spinal cord anterior horn lesions were
analysed. Diagnostic algorithm included electroneuromyography
(ENMG), CT and MRT scan of brain, molecular
genetic analysis. Results: All patients were divided into
two groups. There were 19 patients with SMA: 13 cases
with type 1 SMA (Werdnig-Hoffman disease), six cases
with intermediate type 2 (Fried-Emery disease). Group 2
consisted of nine patients with myelodysplasia. All patients
had the same clinical features and similar ENMG pattern, as
well (poor interference pattern, fibrillation potentials, F-
wave fall-out, and reduced M-reply amplitude). Twentyfour
percent of Group 2 patients had ventriculomegaly
according to MRT scan results. Typical deletion in chromosome
5 (locus 11.2–13.3 in survival motor neuron) were
revealed by molecular genetic method. Besides, in six most
acute cases with type 1 SMA defect of neuronal apoptosis
inhibitory protein was detected. Conclusion: Molecular
genetic analysis is indicated as necessary investigational
method in pathology of motoneuron. This technique allows
to establish the diagnosis reliably and also to determine the
prognosis and the extent of required treatment.
FP-J
Neuro-Oncology
FP-J-001
A rare case of primary diffuse leptomeningeal
gliomatosis in a child: presentation, imaging, and results
of temozolomide therapy
L. Dom a , E. Duval b , D. De Surgeloose c , I. Neetens d ,E.
Michiels b , P.P. De Deyn a
Departments of a Neurology and Child Neurology, b Paediatrics,
c Neuroradiology and d Pathology, Algemeen Ziekenhuis
Middelheim and Koningin Paola Kinderziekenhuis,
Antwerp, Belgium
We report the case of a 12-year old girl with diffuse leptomeningeal
gliomatosis. One year prior to admission she was
diagnosed elsewhere with communicating hydrocephalus
treated by ventriculo-peritoneal shunting. In the course of
the next year school results deteriorated and she presented
multiple episodes of headache, confusion and infantile behaviour
once associated with convulsions. She was admitted to
our hospital due to another of these episodes. Except for her
behaviour her neurological examination was normal. Urgent
CT scan of the brain showed stabilised hydrocephalus.
Cranial MRI showed multiple nodular hyper intensities on
the cerebellar and cerebral surface with minimal meningeal
contrast enhancement. Spinal MRI disclosed leptomeningeal
thickening and gadolinium enhancement from C1 to the
cauda. In addition there was a non-enhancing hypointense
lesion at C7. Analysis of CSF disclosed mononuclear pleocytosis
(13/mm 3 ), raised protein value (447 mg/dl), normal
glucose concentration and no oligoclonal bands. Cultures
and serological tests were negative. Biopsy of the cerebellar
arachnoidea and surface showed a grade 2 mixed astrocytic
and oligodendrocytic leptomeningeal gliomatosis compatible
with a diagnosis of diffuse leptomeningeal gliomatosis.
Diffuse leptomeningeal gliomatosis (DLG) is a rare condition
characterized by diffuse infiltration of the leptomeninges
by glioma. In primary DLG, there is no parenchymal tumour
of the brain or spinal cord. It is considered to arise from
heterotopic glial cells. About 25 cases have been reported
so far. It has to be differentiated from diffuse leptomeningeal
seeding from a parenchymal tumour. This may require postmortem
examination. In our case we considered the possibility
of a primary intraparenchymal spinal tumour at C7.
However the lesion is non-enhancing on spinal MRI which
is a strong argument against an intramedullary neoplasm.
Neuroimaging features in this case are furthermore remarkable
for the absence of intracranial gadolinium enhancement
and the nodular lesions on the cerebellar surface. These characteristics
have only been described once by S. Kastenbauer
et al. Therapy was initiated with vincristine and carboplatin.
Despite this, her condition deteriorated and repeat cranial and
spinal MRI disclosed progression of the disease. Her therapy
was switched to temozolomide in November 2001. Results of
this therapeutic follow-up will be presented. To my knowledge
it is the first case of primary diffuse leptomeningeal
gliomatosis in childhood treated with temozolomide.
FP-J-002
EEG in diagnosing childhood brain tumor – do we still
need it?
I. Prpić, E. Paučić-Kirinčić, A. Sasso
University Children Hospital ‘Kantrida’ and Medical
Faculty of University of Rijeka, Rijeka, Croatia

Abstracts 553
EEG as a technique is simple, painless and can be easily
repeated, but it is not conclusive in diagnosing brain tumor.
With brain CT and/or MR, there is no doubt; the diagnosis
of brain tumor is conclusive. But, these relatively advanced
neuroimaging techniques are still not widely accessible for
every patient. In order to estimate role of EEG in diagnosing
childhood brain tumor we retrospectively analyzed
EEG findings of 39 children with brain tumor, treated at
the Department of Pediatrics ‘Kantrida’ of University
Hospital Center Rijeka. Group consisted of 18 boys and
21 girls; medium age was 6.8 years. Brain tumor was
confirmed by CT or MR. In 22 children tumor was located
infratentorial and in 17 children supratentorial. The average
days from the first symptoms to final diagnosis were 73
for infratentorial tumors and 32 days for supratentorial
tumors. EEG, performed immediately after admission,
was pathological (delta focus/spikes/spike-wave) in 18
children (46.1%), non-specific (diffusely dysrhythmic) in
15 children (38.6%), and normal in six children (15.3%).
Normal EEG was found only in children with infratentorial
located tumor. Pathological EEG was found in all children
with supratentorial tumor location except two. Almost all
children (13 from 15) with non-specific EEG had infratentorial
tumor. Our results lead to a conclusion that EEG is
suitable for the assessment of ‘tumor suspects’. If there is a
doubt in the mind of the clinician, and CT or MR are not
quite accessible, follow-ups EEGs on regular monthly
intervals can assist in timing appropriate further neuroimaging
procedures. The EEG usually might show an increasing
abnormality as the pathological process advances and
still has a role in diagnosing childhood brain tumor.
FP-J-003
A case of spinocerebellar degeneration as a late
manifestation after Langerhans cell histiocytosis
Y. Nakamura a , M. Ito a , M. Miyao a , K. Nihei a ,Y.
Tsunematu b
Departments of
a Neurology and b Hematology/Oncology,
National Center for Child Health and Development, Japan
Central nervous system complications associated with
neoplasm are well known; paraneoplastic syndrome with
cerebellar degeneration and encephalomyeloneuritis caused
by autoantibodies to neural tissue, as have been described in
neuroblastoma and other malignancies. Langerhans cell
histiocytosis (LCH) is the cryptogenic disease characterized
by proliferation of the monoclonal histiocyte which is the
same as the Langerhans cell in the epidermis. Diabetes insipidus
and anterior pituitary dysfunction are familiar CNS
complications because of proliferation and invasion of
LCH into the CNS. We report a case of spinocerebellar
degeneration as a late manifestation after LCH. In this
case, there was no family history of spinocerebellar degeneration.
Routine laboratory tests and cerebrospinal fluid were
normal. Neuroimaging studies included brain CT, MRI and
SPECT. Brain CT and MRI showed a global cerebellar atrophy
but no evidence of direct invasion into the CNS. He
received treatment with thyrotropin-releasing hormone
(TRH) (2 mg/day, 14 days), g-globulin (400 mg/kg per
day, 5 days) and high dose corticosteroid. Treatment with
TRH was effective but g-globulin and corticosteroid were
not effective. Recently, there are some reports of spinocerebellar
degeneration affected with LCH and we attempt to
hypothesize about the occurrence mechanisms and the treatment.
FP-J-004
Intracranial tumours in infants
H.K. Young, H.M. Johnston
Department of Neurology, Sydney Children’s Hospital,
Randwick, Sydney, Australia
Prognosis in infants with brain tumours has historically
been very poor. This study reviews 16 infants under the
age of 12 months with brain tumours, presenting to our
institution between 1988 and 1999. The aim was to
describe the clinical presentation, diagnosis and management
of these patients, and to establish if newer diagnostic
and treatment modalities have improved prognosis in terms
of survival and neurocognitive outcome. Methods: Charts
were reviewed retrospectively for age at diagnosis, time to
diagnosis, presenting features, location, histology, surgical
and adjuvant treatment, survival and neurocognitive
outcome. Results: Survival has improved. Three quarters
of the patients remain alive. Five year survival is 81%.
Five year progression free survival was 51% with a median
follow up time of 70 months. Five year survival for benign
tumours was 100%. None of those with malignant tumours
survived. Morbidity remains high. Eight/13 of survivors
had focal neurological deficits, 7/13 had epilepsy. Seven/
12 had significant cognitive disability. Conclusions: Future
treatment protocols should include formal analysis of
neurocognitive morbidity, functional outcome and quality
of life measures to provide accurate prognostic information
and to prepare families for early interventional programs.
FP-J-005
A clinical study on intracranial relapsing germinomas
by combined chemotherapy
G. Jia, S.-Q. Luo
Department of Neurosurgery, Beijing Tiantan Hospital,
Beijing, China
Objective: To study the clinical effects of intracranial
relapsing germinomas by combined chemotherapy. Methods:
From August 1993 to December 2000, we conducted
combined chemotherapy to ten patients with intracranial
relapsing germinomas. Pathological examination of the
specimens resected during operation was made in seven

554
Abstracts
out of ten patients. The diagnosis of the others was
confirmed by the result of radiotherapy and examination
of HCG in blood. Metastasis of intracranial relapsing germinomas
was found in seven patients. The drugs we used are
vincristine (VCR), methotrexate (MTX), Pinyangmycin and
pervasive developmental disorder (PDD). Four days are
considered as one course of treatment. A repeat course
was taken after 4 weeks. CT and/or MRI are repeated 1
month after the second course. Local radiotherapy (2500–
3500 Gy) was taken with low or middle dosage 1–2 month
after chemotherapy. Results: All patient were followed up
for 1.5–7 years. They have resumed normal life or back to
school. Conclusion: The authors suggest that combined
chemotherapy should be conducted first once the diagnosis
of intracranial relapsing germinomas has been confirmed.
Local radiotherapy with low or middle dosage was
followed. Thus, it could eliminate the tumors and avoid
affecting the development of the children caused by large
dosage of whole brain and spinal axis irradiation
FP-J-006
Clinical significance of cerebrospinal fluid b2-
microglobulin in central nervous system leukaemia
R.-X. Zhang
Department of Pediatrics, Central Hospital of Yichang, Hu
Bei, China
Objective: To investigate the clinical significance of CSF
b2-microglobulin in the patients with central nervous
system leukemia (CNSL). Methods: We investigated 45
leukemia patients, including 31 patients without CNSL, 14
patients with CNSL. Thirty-one patients without CNS
disease and leukemia were taken as the controls. CSF b2-
microglobulin was detected with RIA. Results: b2-Microglobulin
levels in patients with CNSL (2.63 ^ 0.38 mg/l)
were significantly higher than either those without CNSL
leukemia (1.32 ^ 0.47 mg/l) or controls (1.32 ^ 0.13 mg/l)
(P , 0:01). There was no difference between CSF b2-
microglobulin levels in the patients without CNSL and
those in controls. Conclusion: b2-microglobulin can be
considered as an important index for diagnosis of CNSL.
FP-J-007
Primary non-Hodgkin lymphoma of the vertebral body
with spinal epidural invasion – case report
D. Plesca a , D. Dragomir a , D. Teleanu b , G. Iacob b
Department of Pediatric Neurology, ‘Dr. Victor Gomoiu’
Children’s Hospital; and b Department of Neurosurgery,
University Hospital, Bucharest, Romania
A rare case of primary non-Hodgkin lymphoma of the T6
vertebral body with spinal epidural invasion is reported.
Primary non-Hodgkin lymphoma is a rare malignant hematological
tumor. The tumor produced predominantly osteolytic
lesions, usually in the femur and pelvic bones, and very
occasionally presents with vertebral and spinal epidural
involvement. The case now reported was a 12-year-old
boy with primary non-Hodgkin lymphoma involving the
T6 vertebral body and with epidural invasion, who underwent
surgical treatment (laminectomy T6, T7 resection of
the epidural mass), radiotherapy and chemotherapy. The
patient was in complete remission at the follow-up (1 year
after diagnosis). Surgical treatment is only palliative, but
has several goals, e.g. obtaining a biopsy or improving the
neurological symptoms through decompression. Because of
its aggressive behavior, this type of lymphoma should be
treated by combined modality approach (surgery,
chemotherapy and radiotherapy).
FP-J-008
Analysis of clinical and histological characters of 33
cases brain tumours in children
T.N. Minh, N.T. Ung
Neurology Department, National Institute Pediatrics,
Hanoi, VietNam
Objective: Study of 33 children with brain tumours from
January 1998 to December 2001 at National Institute Pediatrics.
Methods: Diagnoses were based on clinical findings of
focal neurological signs and computer tomography. Results:
There were 52% boys, 48% girls. Patients (39%) aged from
3 to 5 years, 27% from 6 to 10 years, 34% from 11 to 15
years. The clinical findings were headache in 70% patients,
vomiting 36%, ataxia 18%, hemiplegia 9%; 24/28 patients
had papilloedema, 4/28 normal optic disk. Histological findings:
astrocytomas comprised 15%, medulloblastomas 48%,
choroid plexus papillomas 6%, oligodendrogliomas 6%,
teratomas 3%, and primitive neuroectodermal tumours
12%. Craniotomy was performed in 33 patients with radical
resection. The results of treatment showed high mortality
and morbidity rates.
FP-J-009
The changes of sICAM-1 and soluble L-selectin in blood
of children with acute lymphoblastic leukemia (ALL)
during high-dose methotrexate (HD-MTX)
chemotherapy
W.-Q. Geng, F.-H. Guan, Y. Hu, K. Tan
Shenya ng Women and Children’s Medical Health Care
Center, Shenyang, China
Objective: To study the mechanism that high-dose methotrexate
(HD-MTX) therapy prevents and treats CNSL.
Method: The serum intercellular adhesion molecule-1
(sICAM-1) and soluble L-selectin (sL-selectin) levels
were measured 1 h before and 24, 28, 32, 44, and 68 h
after the HD-MTX therapy by dual-antibody sandwich
ELISA in 30 children with ALL. The blood MTX levels

Abstracts 555
were monitored 24, 28, 32, 44, and 68 h after HDMTX
therapy in 12 of these cases. Results: There was no difference
between the serum sICAM-1 and sL-selectin levels at
24 h after HDMTX chemotherapy and those before the
HDMTX treatment (P . 0:05). After chemotherapy, the
serum sICAM-1 and sL-selectin levels decreased gradually.
The levels at 28 and 32 h after chemotherapy were significantly
decreased compared with that before chemotherapy
(P , 0:05), so did the levels at 44 and 68 h after chemotherapy
(P , 0:01). Conclusion: The decrease of the serum
sICAM-1 and sL-selectin levels could be another major
mechanism that HDMTX prevented and treated CNSL.
FP-J-010
High level of soluble L-selectin and prealbumin in the
cerebrospinal fluid of children with central nervous
system leukemia (CNSL)
W.-Q. Geng, F.-H. Guan, Z.-Y. Li
Shenyang Women and Children’s Medical Health Care
Center, Shenyang, China
Objective: To observe the changes of the levels of sLselectin
and prealbumin (PA) in CSF of children with
CNSL. Method: The levels of sL-selectin and PA were
determined in the CSF samples of 28 ALL children with
CNSL. CSF samples were obtained from 24 patients at 4–12
weeks before the diagnosis of CNSL, and from 20 of these
patients after treatment of CNSL. In addition, the levels of
sL-selectin and PA were also assayed in 18 ALL children
with complete remission at least a year. There were 12
children without malignant or meningeal disorders selected
as controls. Results: There was no significant difference in
CSF sL-selectin levels between the children with ALL without
evidence of CNSL and the controls. However, the CSF
levels of sL-selectin and PA in patients with CNSL were
significantly higher than those in controls at the time CNSL
diagnosed and 4–12 weeks before the diagnosis of CNSL.
After treatment and disappearance of the lymphoblasts in
CSF, the levels of sL-selectin and PA were decreased in the
majority of children who had CNSL. The levels of sL-selectin
in CSF remained high in six patients after the disappearance
of lymphoblasts in CSF, four of them recurred CNSL
within 12 weeks. Conclusions: Detection of sL-selectin and
PA in CSF may be helpful for the diagnosis of CNSL. The
levels of sL-selectin in CSF may be used as a predictor of
CNSL in children with acute leukemia.
FP-J-011
The clinical profile of pediatric leukemic patients with
neurologic complications in a tertiary hospital: a 5-year
retrospective study
D.M. Dionson-Gigataras
Philippine Children’s Medical Center, Quezon City, Philippines
The leukemias are the most common form of childhood
cancer. Due to advances in treatment and prolonged survival,
an increased incidence of leukemic neurologic complications
has been observed. Objectives: This study was
undertaken to document the spectrum of problems that
prompt neurologic referral in leukemic patients, to determine
the diagnosis associated with the common presenting
neurological symptoms, and to describe the population of
leukemic children who developed such problems. Methodology:
This paper reviewed the clinical profile of 48 leukemic
patients referred to the Neurology service of a pediatric
tertiary hospital from June 1996 to June 2001. Results: The
population was composed of 26 males and 22 females. Forty
percent of the study population belonged to the 4–6 year old
age group. Majority (91.7%) had ALL. The average time
interval from diagnosis of leukemia to the time of developing
neurologic symptoms was 24 months. The most
common symptoms that prompted referral to the neurology
service were headache (39.6%), altered mental status,
seizures and lower extremity weakness. Of the 19 patients
who presented with headache, 14 were diagnosed to have
CNS leukemia while three patients had intracranial hemorrhage.
For those with altered mental status 42.8% had intracranial
hemorrhage, 28.57% had infarction and 28.57% had
CNS leukemia. Among those with seizures, 46.15% had
intracranial hemorrhage, and 10% had CNS leukemia. Of
the 13 patients with lower extremity weakness, 53.84% had
radiculitis and 15.4% had GBS. Mortality in this study
population was 26.22%, majority of which was secondary
to intracerebral hemorrhage (16.4%).
FP-J-012
Epilepsia partialis continua associated with widespread
gliomatosis cerebri
E. Shahar a , U. Kramer b , D. Nass c , D. Savitzki a
a Child Neurology Unit and Epilepsy Service, Rambam
Medical Center, Haifa, b Epilepsy Service, Dana Children
Hospital, Tel-Aviv, c Department of Pathology, Sheba Medical
Center, Tel-Hashomer, Israel
We present herein an uncommon association of intractable
epilepsia partialis continua which was the main presentation
of widespread gliomatosis cerebri in two girls. Both
children had a preceding prolonged secondary generalized
seizure 2–4 months prior to the evolution of epilepsia partialis
continua (EPC). Patient 1 had a 6 month period of up to
80–100 daily clusters of left-sided twitching but remained
fully alert, accompanied with increasing left-sided pyramidal
weakness and sensory impairment. Surface and video-
EEG demonstrated prolonged right fronto-central epileptic
spike/wave discharges, corroborating with the left focal
myoclonic jerks. MRI demonstrated a wide demarcated
area of increasing signals over the right fronto-central
regions, with diffuse cortical-subcortical infiltration,
impinging on the left hemisphere. Patient 2 had recurrent

556
Abstracts
focal sensory seizures followed by focal motor events, along
with progressive hemiparesis, but remained alert, along with
focal epileptic activity. A streotactic brain biopsy in both
revealed gliomatosis cerebri (GC): diffuse widespread infiltration
of glioma cells with no constitution of a circumscribed
tumor mass. The first child is surviving after 2
years on combined treatment, the second perished.
Although very rare, GC should be taken into account in
the differential diagnosis of EPC, of which Rassmussen’s
encephalitis and focal cortical dysplasia are more common
etiologies in children, in order to facilitate a rapid diagnosis
and prompt current combined effective therapy for GC.
FP-J-013
Giant cell tumor in a child: Unusual location, rare
presentation but favorable outcome
H. Bibas-Bonet, R. Fauze, G. Lavado, R. Páez, J. Nieman
Hospital del Niño Jesús, San Miguel de Tucumán, Argentina
An 8-year-old female presented with a 3-month gradually
worsening right (R) otalgia, tinnitus, hearing loss, R facial
numbness, and diplopia. She was admitted with a 2-week
swallowing difficulties, voice change, and R shoulder pain.
Neurological examination revealed R abducens paralysis, R
facial palsy, R sensorineural deafness, R trigeminal hyperesthesia,
R shoulder weakness, right-sided tongue fasciculation,
and normal motor-sensory functions of limbs. Skull X-
ray revealed lytic lesions in R temporal petrous portion. CT
scan showed a destructive mass involving R greater wing of
sphenoid bone and temporal petrous apex. MRI demonstrated
a tumor arising from temporosphenoidal region,
infiltrating neither the brain nor the brainstem. No hydrocephalus
was seen. Biopsy revealed giant cell tumor osteoclastoma.
Posterior treatment consisted of radiotherapy.
Clinical improvement was noticed by second week. After
8-year follow-up, patient is well but with functional sequelae.
There was no MRI evidence of tumor growing. Giant
cell tumor is a primary bone tumor affecting rarely the skull
base. It is considered benign, but could show local aggressiveness.
Progressive involvement of cranial nerves culminating
in paralysis of all unilaterally, without sensory or
motor long tract disturbance, no intracranial hypertension,
and osteoclastic lesion in skull base on X-ray is described as
Garcin syndrome. It is usually resulting from malignant
tumor, sarcoma or nasopharynx fibrosarcoma invading
skull base. To our knowledge, a giant cell tumor presenting
as Garcin syndrome has not yet been reported.
FP-J-014
Gelastic seizures and hypothalamic hamartoma: do not
forget the Pallister-Hall syndrome
C. Arpino a,b , C. Galasso b , M. Marzio b , M. Verdecchia b ,P.
Curatolo b
a ‘E. Litta’ Rehabilitation Center for Developmental
Disabilities; b Pediatric Neurology, Tor Vergata University
of Rome, Rome, Italy
Gelastic seizures are often associated with hypothalamic
hamartoma. A detailed evaluation, through MRI, in patients
presenting gelastic seizures is recommended. In order to
exclude an autosomal dominant disorder known as Pallister-Hall
Syndrome (PHS), a thorough physical examination
in the presence of gelastic seizures and hypothalamic hamartoma
should be also recommended. We observed a 3-yearold
boy and a 21-year-old girl presenting with mental retardation,
behavioral disorders, and symptomatic localization
related epilepsy characterized by gelastic, partial motor,
autonomic seizures accompanied by staring and impaired
responsiveness, and drop attacks. Interictal EEG showed
bilateral and asynchronous sequences of slow spike and
wave complex (2.5–3 Hz) on fronto-temporal regions with
a secondary bilateral synchrony. MRI showed a non-enhancing,
midline hypothalamic mass with a diameter of 2 cm.
The girl had undergone surgery for central polysyndactyly
and a callosotomy to control seizures. The clinical picture
presented by these patients was suggestive of PHS, for which
the described phenotypic features include hypothalamic
hamartoma, central polydactyly, pituitary dysfunction and
visceral malformations. Seizures are occasionally reported
in PHS and are not currently included in the clinical spectrum
of the syndrome. Whether gelastic seizures in PHS are underdiagnosed
or their occasional occurrence is dependent on the
topographic localization of the hypothalamic hamartoma, as
suggested by a previous study, remains an open question. The
association between gelastic seizures, hypothalamic hamartoma
and malformations suggestive of PHS needs to be carefully
investigated in order to better define the clinical
spectrum of PHS and to plan genetic counseling in apparently
unaffected relatives.
FP-J-015
Spontaneous recovery of medulloblastoma in a girl with
Gorlin-Goltz syndrome
C.-W. Su a , K.-L. Lin a , J.-W. Hou b , S.-M. Jung c , E.-C. Zen d
a Division of Pediatric Neurology; b Division of Pediatric
Genetics; Department of Pediatrics, Chang Gung Children’s
Hospital; c Department of Pathology; d Division of
Oral Surgery, Department of Dentistry, Chang Gung
Memorial Hospital, Taoyuan, Chinese Taipei
The Gorlin-Goltz syndrome is characterized by nevoid
basal cell carcinomas, odontogenic keratocysts of jaws,
palmar and plantar pits, falx calcifications, and various
cancer susceptibilities attributed to a mutation in a tumor
suppressor gene. We presented an 11-year-old female with
Gorlin-Goltz syndrome. Medulloblastoma was diagnosed
when she was 4 years old. She received total excision of
the tumor and adjuvant therapy of chemotherapy and radiotherapy.
One year later, the tumor relapsed with CSF seeding.

Abstracts 557
She recovered spontaneously without any treatment, and she
did not develop basal cell carcinoma within 6 years followup.
It suggests that patients with Gorlin-Goltz syndrome tend
to have a better prognosis with regard to medulloblastoma,
and to diagnose the syndrome as early as possible will make
the management optimal.
FP-K
Genetics/Degenerative Diseases
FP-K-001
Cerebral dysgenesis associated with L1CAM mutations
L. Sztriha, R.M.W. Hofstra, Y.J. Vos, J. Johansen
Departments of Paediatrics and Radiology, FMHS, UAE
University, United Arab Emirates; and Department of
Medical Genetics, Faculty of Medical Sciences, University
of Groningen, The Netherlands
L1 neuronal cell adhesion molecule belongs to the immunoglobulin
superfamily. It plays a key role in axon outgrowth,
guidance and pathfinding during development of the nervous
system. The encoding gene, L1CAM is located near the telomereofthelongarmoftheXchromosome,intheXq28region.
Mutations in the L1CAM gene are responsible for an X-linked
recessive disorder with features of corpus callosum hypoplasia
or agenesis, mental retardation, adducted thumbs, spastic
paraplegia and hydrocephalus (CRASH syndrome). The clinical,
MRI and molecular genetic findings of two boys with
missense mutation in the L1CAM gene are presented. Patient
1 had delayed development, mental retardation, adducted
thumbs and spastic diplegia. Brain MRI revealed simplified
pattern of gyri with reduced volume of gray and white matter,
agenesis of the corpus callosum, dilated lateral ventricles and
hypoplasia of the cerebellar vermis. Transition of a G
(guanine) to T (thymine) was found at position 604
(604G ! T) in the L1CAM gene. This mutation results in
conversion of aspartic acid to tyrosine at position 202
(D202Y) of the L1 protein. Patient 2 presented with congenital
hydrocephalus. Three of his brothers with congenital
hydrocephalus died after birth. The proband showed delayed
development and mental retardation after shunt implantation.
Adducted thumbs and severe spastic diplegia were observed.
MRI revealed parietal-occipital hypoplasia with multiple
small gyri (polymicrogyria), agenesis of the corpus callosum
and fused thalami. Transition of a G (guanine) to C (cytosine)
at position 1243 (1243G ! C) was found in the L1CAM gene,
which led to conversion of alanine to proline at position 415
(A415P) in the L1 protein.
FP-K-002
The Fahr disease in a family
Q.-K. Huang, F.-J. Yang, J.-R. Wang
Department of Pediatrics, Shanxian Central Hospital,
Shanxian County, Shandong, China
Fahr disease is a hereditary disease that rarely occurs in
childhood characterized by calcium deposition in basal
ganglia. It has been considered an autosomal dominant/
recessive inheritance or chromosome disorder. We present
the clinical features and the inheritance pattern of six cases
of Fahr disease in a family. The proband was a 12 yeas old
Chinese girl, normally delivered from unrelated parents.
The initial onset of seizures occurred when she was 2
months old. The head CT showed small local calcification
in bilateral basal ganglia when she was 2 years old. Now she
is 12 years old and still has recurrent seizures, learning
disability and irritability, and CT scan shows diffuse
calcium deposition in bilateral basal ganglia, chromosome
analysis shows normal pattern. Proband’s sister developed
seizures when she was 2 months old. The head CT have
shown small local calcification in her bilateral basal ganglia
when she was 10 months old. Now she is 10 years and 10
months old and still have recurrent seizures, mental retardation,
and CT scan showed diffuse calcium deposition in
bilateral basal ganglia and hemicerebrum. Head CT scan
of the prodand’s father, uncle, aunt and grandfather all
have shown small local calcification in bilateral basal ganglia
10 years ago, but no significant change now. They have
no any clinical symptom and sign. Conclusion: (1) the mode
of transmission of the Fahr disease in this family is autosomal
dominant inheritance; (2) this pedigree shows a
hereditary disease of early onset, and the earlier the onset,
the faster the progression; and (3) the clinical features are
heterogeneous and head CT is an important examination in
the clinical diagnosis of Fahr disease.
FP-K-003
Angelman’s syndrome: Evaluation of clinical features,
EEG and therapeutic approach
J. Arcas, M. Cabrera, A. Martínez-Bermejo, D. López-
Pajares, V. López-Martín
Paediatric Neurology and Genetics, ‘La Paz’ University
Hospital, Madrid, Spain
Angelman’s syndrome (AS) is a severe neurobehavioural
disorder with a heterogeneous genetic aetiology. Epilepsy
develops in 90% and it is intractable in about 50% in children.
The aim of this study was to evaluate the early clinical
and EEG finding in a group of patients with AS to determine
whether there are any clinical or EEG features sufficiently
distinctive to be of value in early diagnosis, as well as the
best therapeutic approach. The charts of 13 patients with
clinical/genetic diagnosis of AS were reviewed. All patients
had been clinically, neurophysiologically (EEG data) and
genetically investigated. There were five males and eight
females, aged 13 month–19 years. Range age at diagnosis
13 months–15 years. All patients had severe speech impairment
and motor development was delayed in all patients but
one. Seizures were reported in 11 out of 13 cases. The onset
seizures age ranged 12 months–5 years. The most frequent

558
Abstracts
ictal patterns were atypical absences and myoclonic
seizures. EEG studies showed characteristic AS EEG
patterns in 12 out of 13 cases. The most effective antiepileptic
drugs were Benzodiazepines and VPA regardless of
seizure type. We conclude that motor and mental development
delay with severe speech impairment, associated to
characteristic EEG patterns, should help to identify patients
with AS at an early age. Moreover, the effects of GABAergic
drugs for epilepsy in AS may suggest that defective
genes of the g-aminobutyrate-A receptor subunit, are implicated
in the mechanism of epilepsy in this syndrome.
FP-K-004
A case report of mitochondrial myopathy
S.-P. Ying, S.-J. Wang
Department of Pediatrics, No. 3 Affiliated Hospital of
Baotou Medical College, Qingshan District, Baotou, Inner
Mongolia, China
Mitochondrial myopathies are rare hereditary diseases in
children. The prognosis in most cases is very poor. We
report one case of carnitine deficiency syndrome, which is
one type of mitochondrial myopathies. The patient was a 12
year-old girl. The main clinical features were chronic
progressive generalized weakness without muscle atrophy.
The weakness got worse after exercise such as chewing and
walking and improves following rest. The nervous system
examination is normal, without muscle pain or symptoms of
encephalopathy. The case had no positive family history.
Laboratory investigations showed lactate level increased
after exercise. EMG demonstrated a myopathy-type
abnormality. Muscle biopsy showed that fat drops could
be seen in many muscle fibers. After diagnosis, the case
was treated with carnitine 0.5 g three times daily. The
short-term curative effect is very good. After 2 weeks of
treatment, the muscle strength returned to normal. Up to
now, the case had been treated for one and a half years.
The weakness did not appear again.
FP-K-005
Cytogenetic and molecular-cytogenetic studies of Rett
syndrome (RTT): the investigation of 81 girls and four
boys
S.G. Vorsanova, Y.B. Yurov, V.Y. Ulas, I.A. Demidova,
V.O. Sharonin, A.D. Kolotii, A.K. Beresheva, V.S. Kravets,
I.V. Soloviev
Institute of Pediatrics and Children Surgery, Russian Ministry
of Health and National Research Center of Mental
Health, Russian Academy of Medical Sciences, Moscow,
Russia
Rett syndrome (RTT) is a severe neurodevelopmental
disorder with the incidence of 2.5% in mentally retarded
girls in Russia. We have performed cytogenetic studies of
85 patients (81 girls and four boys) with clinical picture of
RTT. Collections of DNA samples and fixed cell suspensions
of RTT patients and their parents were established for
extended molecular studies. MeCP2 testing in randomly
selected group of 30 RTT girls show that 84% of them
have different mutations in the MeCP2 gene. Among 85
patients: 81 girls with clinical picture of RTT had normal
female karyotype, two boys had normal male karyotype in
cells of blood (46, XY) and two boys had mosaic form of
Klinefelter syndrome (47, XXY/46, XY) in blood and
muscle cells. Eighty-one mothers and parents of RTT
girls had normal karyotype, three mothers of RTT patients
– with mosaic forms of Turner syndrome (45, X/46, XX)
and of trisomy X (47, XXX/46, XX), one mother – mosaic
karyotype (47, XX, 1mar/48, XXX, 1mar). We analyzed
chromosome X staining after incorporation of BrdU in
lymphocytes of 81 affected girls with clinical picture of
RTT. Specific types of inactive chromosome X with
unusual conformation of chromatin in long arm of the
chromosome X were found in 93% of RTT girls. This
cytogenetic technique was used for presymptomatic diagnosis
of RTT in five girls in affected families. We believe
that the phenomenon of altered chromatin conformation in
the inactive chromosome X could be used as laboratory test
for diagnosis of RTT. Supported by INTAS and COPER-
NICUS grants.
FP-K-006
FISH analysis of DNA replication of chromosome X loci
in Rett syndrome using interphase fluorescence in situ
hybridization
Y.B. Yurov, S.G. Vorsanova, A.D. Kolotii, I.Y. Iourov, P.
Sheinson, V.V. Monachov
Institute of Pediatrics and Children Surgery, Russian Ministry
of Health and National Research Center of Mental
Health, Russian Academy of Medical Sciences, Moscow,
Russia
RTT is a developmental disorder, affecting girls. It was
shown recently that RTT is caused by mutations in the
MeCP2 gene on chromosome Xq28, whose function is to
silence other genes. In RTT patients the defective MECP2
might fail to keep other genes ‘silent’. The targeting genes
directly involved in the pathogenesis of RTT are still
unknown. To identify the potential regions and loci with
altered transcription and replication status in active and
inactive chromosomes X at RTT we used molecular cytogenetic
approaches. We used interphase FISH with DNA
probes to determine the loci with altered replication pattern
in X-chromosomes at RTT. Large-insertion PAC clones for
Xptel and Xqtel, pseudoautosomal locus at Xq28 and
centromeric alphoid DNA were used. PAC clones 671D9
(MeCP2 gene) and 24.23.0 at telomeric region of the q-arm
(Xq28) escape inactivation in inactive chromosome X only
in some of the RTT patients. Therefore, this region could

Abstracts 559
contain the gene(s), abnormally transcriptionally active in
both chromosomes X. Our results indicate that interphase
FISH analysis of DNA replication patterns allows detecting
the targeting loci with altered order of replication and transcription
in RTT patients. These results support the hypothesis
that the disturbances in dosage compensation effect due
to aberrant activation of genes in inactive chromosome X at
RTT genes (bi-allelic expression instead of mono-allelic).
Our results indicate that MeCP2 itself could escape X-inactivation
and reduce the pathogenic effect of mutated allele at
RTT. Supported in parts by INTAS and COPERNICUS
grants.
FP-K-007
Differences in clinical picture of tuberous sclerosis
complex in patients with TSC1 and TSC2 mutations
S. Jóźwiak a , D. Domańska-Pakiel⁄a a , J. Kasprzyk-Obara a ,
D.J. Kwiatkowski b , S. Dabora b
a Department of Child Neurology, The Children’s Memorial
Health Institute, Warsaw, Poland; b Division of Experimental
Medicine and Medical Oncology, Brigham and Women’s
Hospital, Boston MA, USA
Tuberous sclerosis (TSC) is a relatively common neurocutaneous
syndrome caused by mutations in either of two
genes TSC1 and TSC2. We carried out a comprehensive
mutational analysis in large group of patients treated in The
Children’s Memorial Health Institute in Warsaw and correlated
mutation findings with clinical features. Eighty-six
patients were identified with mutations: 18 within TSC1
gene and 68 within TSC2 gene. Age of patients ranged
from 4 months to 51 years. Epilepsy was reported at an
earlier age (median age 0.5 versus 1.83 years) and occurred
more frequently (86.8 versus 55.6%) in TSC2 group. Incidence
of infantile spasms was much higher in TSC2
patients (64.4 versus 20%). In contrast, mental status of
patients with TSC1 mutations was normal in 64.8% of
those examined and only in 27.7% of patients with TSC2
mutations. Despite of younger age of examined in TSC2
group, skin lesions were more frequently reported in these
patients. Only periungal fibromas were more common in
TSC1 subjects. The incidence of calcified periventricular
calcifications on brain CT was similar in both groups.
There was a slight preponderance of TSC2 patients
among those with subependymal giant cell astrocytoma
(1/16 versus 7/64). Renal and liver angiomyolipomas and
cardiac rhabdomyomas were more frequent in TSC2
patients. None of examined TSC1 patients had retinal
hamartomas or pulmonary lymphangiomatosis. The more
severe clinical presentation of tuberous sclerosis in the
TSC2 group demonstrates the need for more frequent
inner organ screening in these patients than in those with
TSC1 mutations.
FP-K-008
Initial manifestations and follow-up studies of optic
pathway gliomas in children with neurofibromatosis
type 1 (NF-1) and without NF-1
S. Jóźzwiak a , E. Czyzyk b , J. Kasprzyk-Obara a
a Department of Child Neurology, The Children’s Memorial
Health Institute, Warsaw, Poland; b Provincial Hospital No.
2, Rzeszów, Poland
Optic pathway gliomas (OPGs) are frequent central
nervous tumors in patients with neurofibromatosis type 1
(NF-1). The main aim of our study was to compare initial
clinical manifestations and natural history of OPGs associated
with NF-1 from isolated OPGs. There have been 83
children with OPGs identified retrospectively by a review
of medical records: 51 children with N-1 and 32 children
with isolated gliomas. There was a slight preponderance of
female patients in NF-1 group (32/51 versus 19/51,
P ¼ 0:013), while isolated OPGs occurred equally in
boys and girls (16/32 versus 16/32). The median age of
children was 4.6 years for OPGs with NF-1 and 4.8 years
for isolated OPGs. OPGs commonly occurred at an early
age in children with NF-1, but there were no significant
differences. Decreased visual acuity at the time of diagnosis
was more common in children without than with NF-1
(18/19 versus 29/42, respectively, P , 0; 05), similar to
ophthalmoscopic abnormalities (optic atrophy and oedema)
(25/27 versus 30/49, respectively, P , 0:01). Children
without NF-1 had more often proptosis and nystagmus
(11/32 versus 10/51 and 7/32 versus 4/51, respectively).
Multicentric location was more common in NF-1 (34/51,
P ¼ 0:0001), whereas isolated chiasmal involvement
occurred more often in those without NF-1 (15/32). Radiological
signs of progression and deterioration of visual
function at later follow-up were more commonly noted in
children with isolated OPGs than with NF-1. The clinical
presentation of OPGs at the time of diagnosis and during
follow-up was more severe in children without than with
NF-1.
FP-K-009
Three novel SURF1 mutations in Japanese patients with
Leigh syndrome associated with cytochrome c oxidase
deficiency
E. Naito, Y. Ogawa, M. Ito, l. Yokota, T. Saijo, J. Matsuda,
S. Kitamura, Y. Kuroda
Department of Pediatrics, School of Medicine, University of
Tokushima, Tokushima, Japan
Leigh syndrome, a severe neurodegenerative disorder,
commonly is associated with cytochrome c oxidase deficiency.
Recent studies in Caucasian patients indicate that
SURF1 gene mutations can cause Leigh syndrome associated
with cytochrome c oxidase deficiency. When we

560
Abstracts
measured cytochrome c oxidase activity in cultured
lymphoblastoid cells from Japanese patients with typical
Leigh syndrome, six proved to have cytochrome c oxidase
deficiency. Three novel mutations of the SURF1 gene were
identified in three of these six patients with cytochrome c
oxidase deficiency. We found one homozygous mutation in
exon 5 of patient 1 (367AG del), and one heterozygous
mutation in each of exon 7 (C640T) and exon 8 (802del
26 bp insGG) of patient 2. We performed subcloning
analysis of exons 7–8 in patient 2 and determined that
each mutation was confined to one allele. Patient 3 showed
the same mutation as patient 1 (367AG del). Parents of
patients 1 and 3 are not consanguineous. Therefore, the
367AG del mutation might have a high incidence in
SURF1 mutations of Japanese patients with Leigh
syndrome. All mutations predicted loss of function of the
SURF1 protein: cytochrome c oxidase activity was
decreased to less than 20% of the control mean in all of
the patients. These results indicate that cultured lymphoblastoid
cells are useful for elucidating the etiology of
Leigh syndrome, and that loss of function of the SURF1
gene product can be responsible for Leigh syndrome associated
with severe cytochrome c oxidase deficiency in
Japanese patients.
FP-K-010
Mutation analysis of phenylalanine hydroxylase gene in
Northern Chinese
F. Song, L.-X. Liu, Y.-L. Yang, Y.-W. Jin, H. Wang
Capital Institute of Pediatrics, Beijing, China
Objective: To detect the mutation of phenylalanine
hydroxylase (PAH) gene in Northern Chinese. Methods:
By using PCR/SSCP and DNA sequencing, we studied the
mutation in exons 3, 5, 7, 10, 11 and 12 of PAH gene. A total
of 108 unrelated children with phenylketonuria from the
northern region of China were included in the analysis.
Result: Twenty-two mutations were identified in the above
exons. Eight mutations were detected in exon 7 and four in
exon 5. Among them there were twelve missense, two
nonsense, three splice and three silent, one insertion and
one deletion mutation. The mutations: I65T, G239D,
722delG, L255s, G346R, 1089-1099insC, IVS 1 2t . c,
Q160Q, L385L have not been previously described in the
China population. Conclusion: Because there are more
mutations in exons 7 and 5 than others, it is suggested that
the exons 7 and 5 is a mutant hot spot of PAH gene in
Chinese. The results obtained confirm the high heterogeneity
of the PAH gene and provide information about the
distribution of PKU mutations in the Northern Chinese
population.
FP-K-011
Polymorphism analysis of ATP7B related microsatellite
DNA haplotype in Wilson disease
X.Q. Liu, Y.F. Zhang, L. Wang, X.F. Gu, K.R. Bao
Department of Pediatrics, Xin Hua Hospital, Shanghai
Second Medical University and Shanghai Institute for
Pediatric Research, Shanghai, China
Objective: To explore the distribution and significance of
haplotype of microsatellite DNA (D13S314, D13S301 and
D13S316) closely related to ATP7B in normal population,
WD patients and heterozygotes. Methods: Using three well
characterized short tandem repeat markers of fluorescence
labeling (D13S301–D13S314–D13S316), localization and
sequence were analyzed with Genotype e software in 71
WD patients and 123 parents. Results: Based on the analysis
of haplotypes of D13 S314, D13S301 and D13S316, 19,
20, and 15 alleles were obtained in 71 patients with WD,
123 carriers and 54 normal persons respectively, size of
segments was 134–157, 128–156 and 136–154 bp. Heterozygosity
was 0.79, 0.82, and 0.23. There was a significant
difference in the distribution of alleles of D13S314,
D13S301 and D13S316 markers between WD patients
and normal subjects (P , 0:01); four alleles in D13 S314
marker, six alleles in D13 S316 marker. There were 81
haplotypes. 12-6-5, 15-10-5, 6-10-5 and 6-15-5; the
commonest haplotypes accounted for 5.22, 4.48, 4.48 and
3.37%, respectively, 12-8-5, 12-7-5 and 6-16-5 separately
accounted for 2.99%, 13-10-8,6-13-5,6-14-13, and 6-9-5
separately accounted for 2.24%. Conclusion: The complexity
of haplotype may be related to the complexity of mutation
spectrum of ATP7B. The haplotype analysis of
D13S314-D13S301-D13S316 may be an effective and
feasible method with important clinical significance for
the presymptomatic and prenatal diagnosis of Wilson
disease.
FP-K-012
Gas chromatography-mass spectrometry (GC/MS)
screening for inborn error of metabolism (IEM) in
patients with mental retardation of unknown cause
K.-M. Xu a , L.-W. Wang a , H. Cai a , T. Zhang a , C.-H. Zhang b ,
I. Matsumoto b
a Capital Institute of pediatrics Beijing of China; b MILS:
Matsumoto Institute of Life Science of Japan
Objective: To conduct screening of IEM by GC/MS in
950 infants with mental-motor retardation of unknown
cause. Method: All of the 950 patients’ urines were
collected on filter paper and sent to MILS in Japan
where the urinary compounds were analyzed using GC/
MS, and the chemical diagnosis for IEM was determined.
Results: Among 950 patients analyzed, 85 cases (9.0%) had
abnormal urinary profiles. There were 18 cases of phenylk-

Abstracts 561
etonuria (PKU), 16 cases of methylmalonic acidemia, 13
cases of galactosemia, four cases of lysinuria, two cases
each of glutaric aciduria, homoserinuria, and fructose-1,6-
diphosphatase deficiency, one case each of pyro-glutaminuria,
neuroblastoma, long-chain fatty acetyl CoA dehydrogenase
deficiency, multiple carboxylase deficiency,
maple syrup urine disease, propionic acidemia, ornithine
transcarbamylase deficiency and Lesch-Nyhan syndrome,
six cases of Fanconi syndrome and 14 cases of ketonuria.
Good outcomes were obtained in most of the abovementioned
patients after treatment with appropriate therapy.
Conclusion: (1) Screening for IEM should be
performed in infants with mental retardation of unknown
cause. (2) The GC/MS analysis method is an accurate,
sensitive and specific method. It is very important to
extend the range and type of screening of IEM.
FP-K-013
The clinical analysis of PKU: report of five cases with
atypical manifestations
G.-Z. Xu a , C. Hong a , L.-W. Wang a , Y.-L. Zhu a , C.-H.
Zhang b , I. Matsumoto b
a Capital Institute of pediatrics Beijing of China; b MILS:
Matsumoto Institute of Life Science of Japan
Objective: Classic PKU is caused by the complete or
near-complete deficiency of phenylalanine hydroxylase.
The excess phenylalanine and subsequent metabolites
disrupt normal metabolism and cause brain damage. Sometimes
the mental retardation is misdiagnosed as an atypical
syndrome and neglected by pediatricians, so that older
untreated children become hyperactive with purposeless
movements, rhythmic rocking and athetosis. This study
aimed to explore the clinical characteristics and prognosis
of the disease and diagnostic values of the urinary gas chromatography-mass
spectrometric (GC/MS) analysis. Methods:
All of the five patients’ clinical manifestations were
non-specific. Samples of urine were collected and sent to
MILS in Japan where the chemical compounds was
analyzed using GC/MS. Results: Among five cases, two
are diagnosed as autism, one spinal muscular atrophy, one
dwarfism, and one language barrier with albinism before
urinary analysis. The levels of phenylalanine, phenyllactate
and phenylpyruvate in urine were obviously elevated. The
diet and medical therapy was followed in four cases. One
case have had a relatively satisfactory outcome, one case
refused treatment, another 3 cases were recently diagnosed
and still on the follow-up. Conclusion: Much attention
should be paid to PKU with atypical signs such as autism,
cerebral palsy especially weakness of muscle strength,
dwarfism, language barrier with albinism. Early diagnosis
and immediately treatment contribute to the improvement of
the prognosis of PKU.
FP-K-014
Diagnoses and treatment of methylmalonic aciduria
(MMA)
L.-W. Wang a , K.-M. Xu a ,H.Cai a , C. Qian a , J.-X. Wu a , C.-
H. Zhang b , I. Matsumoto b
a Capital Institute of pediatrics Beijing of China; b MILS:
Matsumoto Institute of Life Science of Japan
Objective: Chemical diagnosis of inherited metabolic
diseases in patients with mental-motor retardation, poor
feeding, hypotonia, lethargy, convulsion and so on. Method:
All of the 950 patients’ clinical manifestations were nonspecific.
Urines were collected and sent to MILS in Japan
where the chemical compounds in the urine were analyzed
using GC/MS. Results: Among 950 patients analyzed, 85
cases (9.0%) with abnormal urinary profiles were found.
In these patients, 16 cases of MMA and one case of propionic
acidemia were found. They accounted for 22.4% of all
patients with abnormal urinary profiles (16/85). Good
effects were obtained in eight cases after treating with suitable
therapy, five did not improve, three died of late onset
lethal complications (one died of intracranial hemorrhage
owing to disorder of the platelet, and two died with coma
and convulsion associated with ketoacidosis, and hyperammomemia).
Conclusion: (1) Some patients with MMA could
be treated by medication and low-protein diet. (2) Clinical
manifestations of patients with MMA are non-specific, and
the diagnoses cannot be achieved by clinical findings only.
The GC/MS analysis method is accurate, sensitive and
specific. It is important to extend the range and type of
IEM screening.
FP-K-015
244insL, a novel mutation in methylmalonic aciduria in
Chinese patients
J.-X. Wu, H.-B. Chang, Z.-W. Liu, Y.-Y. Li, L.-W. Wang,
H. Cai, K.-M. Xu
Capital of Paediatrics Institute, Yabao Road 2, Beijing,
China
MMA is an autosomal-recessive disorder that results
from inadequate function of methylmalonyl-coA mutase
(MCM E.C. 5,4,99,2) nuclear-encoded, mitochondrial
enzyme that uses the vitamin B12 derivative, adenosylcobalamin
as a cofactor. It can be associated with fulminant
metabolic acidosis, widespread secondary aberrations in
systemic metabolic homeostasis, mental retardation, or
even neonatal death. To date, 49 different mutations have
been identified at the methylmalonyl-coAmutase (MUT)
locus on the short arm of chromosome 6, but there have
been no reports on MCM mutations in the Chinese population.
Genomic DNA was extracted from leukocytes of
MMA patients and normal children. DNA was amplified
by PCR for exons III, XII of the MCM gene using oligonu-

562
Abstracts
cleotide primers recognizing flanking intronic sequences
and cloned into pGEMT-easy vector. By sequencing the
PCR product and cloning, we demonstrated that a two
base pair insert in exon of the MCM patient in cDNA805
resulted in a frame shift mutation. 244insL(c805insTT) is a
novel mutation found in a compound homozygote. We also
detected several SNP sites in MCM exons III and XII of the
patient and controls. The positions in relation to different
MCM domains allow for an interpretation of the identified
mutations. This new mutation is the first reported in the
Chinese population.
FP-K-016
Proteomics: a new strategy to identify mitochondrial
diseases
J. Xie a , S, Techritz a , J. Klose b , M. Schuelke a
a Department of Neuropediatrics,
b Institute of Human
Genetics, Charité, Humboldt-University Berlin, Berlin,
Germany
Mitochondrial diseases can be caused by any defects in
the metabolic pathways located in the mitochondria.
However, there are several obstacles to the molecular diagnosis
in this disease group. These are: (1) the variety of
inheritance patterns (maternal, autosomal recessive, X-
recessive); (2) the large number of genes involved in mitochondrial
biogenesis; and (3) the similarity of the clinical
features. Based on current diagnostic methods, less than
20% suspected cases can be diagnosed at the molecular
level. We have therefore presently developed a new screening
method by which we hope to increase the identification
rate. We studied the mitochondrial proteins in healthy individuals
and in patients with mitochondrial diseases by twodimensional
protein-electrophoresis (2DE). Differences in
protein patterns between healthy and diseased individuals
direct the attention to disease-specific proteins and genes.
Mitochondria were purified from Epstein Barr Virus-transformed
lymphocytes by density gradient centrifugation. We
obtained a satisfactory yield of mitochondria from a small
cell volume of ca. 10 8 lymphocytes. In order to establish a
reference map of mitochondrial proteins we identified ca.
100 proteins by matrix-assisted laser desorption/ionisationtime
of flight (MALDI-TOF) mass spectrometry. The ensuing
reference map was compared to mitochondrial 2DE
maps of patients with mitochondrial diseases. Three of the
patients had mutations in their mitochondrial DNA
(7472inC; 3243A . G ¼ mitochondrial encephalopathy,
lactic acid stroke (MELAS)-syndrome; 8344A . G ¼
myoclonic epilepsy and red ragged muscle fibers
(MERRF)-syndrome) three others had biochemically
proven deficiencies of complex I, III and IV. We have identified
numerous deviating protein spots, which can provide
novel insights into the pathophysiology of mitochondrial
diseases.
FP-K-017
A family case of spongiform leukoencephalopathy
S. Yamashita, H. Iwamoto
Division of Child Neurology, Kanagawa Children’s Medical
Center, Yokohama Japan
Three members including an 11-year-old girl, 6-year-old
girl and 2-year-old boy among four siblings suffered from a
similar disorder. Their parents were cousins. Their clinical
courses included uneventful neonatal history, psychomotor
retardation and developmental arrest. The neurological
examination revealed that the elder two patients had muscle
hypotonia caused by peripheral neuropathy, and the younger
brother had the increased tendon reflexes and positive
Babinski sign. All had cerebellar ataxia, nystagmus and
slurred speech. Biochemical studies showed no evidence
of mitochondrial encephalopathy, leukodytrophy, amino
acid, or organic acid disorders. MRI showed similar
abnormalities in both the T1 and T2 sequences in the
white matter. The lesions involved cerebral white matter
sparing subcortical U-fibers, corpus callosum, internal
capsule, cerebellar white matter and brain stem longitudinal
fibers. Two patients were autopsied and the neuropathological
findings were similar. Vacuolar changes in the white
matter started immediately below the cortex. The deeper,
the white matter showed the more spongy alterations and the
larger number of vacuoles and astrocytes. The matrix tissue
was sparse and destroyed in the deep white matter with
infiltration by many macrophages. The electron microscopic
examination showed intralamellar splitting in the central
myelin sheaths. The cerebral cortical layers showed normal
structures without abnormal storage materials in the neuronal
cells. A genetic disorder is suspected. We discuss this
disorder in the relation to encephalopathy with vanishing
white matter.
FP-K-018
The neurochemical peculiarity in children with
neurocutaneous syndromes
O.V. Globa, E.G. Sorokina, O.I. Maslova, V.M. Studenikin,
V.I. Shelkovsky, V.G. Pinelis
Divisions of Psychoneurology and Membranology,
Research Institute of Pediatrics Scientific Center of Child
Health Russian Academy of Medical Sciences, Moscow,
Russia
The investigations in neurochemical processes – the
determination on autoantibodies (aAB) to glutamate
AMPA GluR1 receptors, cyclic GMP (cGMP) (ELISA)
and sialic acid (SA) (spectrophotometry) in blood serum
were performed in children with different neurological
diseases. To evaluate the changes in content of aAB the
ELISA, elaborated by Institute of the Human Brain, RAS,
was used. We examined 151 patients from 2 months to 16

Abstracts 563
years of ages: 101 with epilepsy (Epi), 15 with neurocutaneous
syndromes (NCS) (seven – tuberous sclerosis, five –
neurofibromatosis type 1, 2-Sturge-Weber syndrome, 1-
Bloch-Sulzberger syndrome) and 35 healthy children. The
content of aAB was significantly increased by 1.5 times in
patients with Epi in comparison with control group (CG) in
all age groups (P , 0; 05). In patients with NCS the aAB
level was by 2–2.5 times higher versus CG. A tendency for
the increased level of SA in children with Epi was revealed.
In patients with NCS the level of SA was significantly
increased to 1.2 times versus CG. These data indicated the
damage of neurons induced by glutamate receptors overstimulation
in children with seizures. In all age groups the
cGMP concentration, an indirect index of NO, was 5–7
times higher than in CG (P , 0:001). In patients with
NCS the cGMP level was higher by not more than three
times versus CG. The results show more expressed injury
of brain neuronal membranes in children with NCS than in
patients with Epi and damage of the cGMP synthesis in
patients with NCS compared to children suffering by Epi.
FP-K-019
Newly described multiple congenital anomalies in
methylenetetrahydrofolate reductase deficiency in an
Arab child
A.A. Al Tawari, N.A. Al Torki, L.C. Heberle, M.A. Al
Ajmi, J.E. Abdullah, N.A. Al Othman, M.M. Al Ajmi
Child Neurology Unit, Children Department, AL Sabah
Hospital, Kuwait
Methylenetetrahydrofolate reductase deficiency
(MTHFR) is a rare metabolic autosomal recessive inherited
disorder. The principal clinical features are neurologic
rather than metabolic such as hypotonia, developmental
delay, seizures, microcephaly, apnea and coma. There are
reports of association of MTHFR with neural tube birth
defects or with congenital cardiac malformations. We report
on an Arab child who is now 1 year old, presenting with two
different disorders caused by two different gene mutations.
Homocystinuria type III caused by methylenetetrahydrofolate
reductase deficiency and spondylocostal dystosis as
well as urogenital anomalies. The child showed improvement
after he was commenced on betaine therapy.
FP-K-020
Canavan disease in Kuwait
A.A. Al Tawari, Y. Habib, L.C. Heberele, M.A. Al Ajmi,
J.K. Abdullah, M. Boloshi, A. Moosa
AL Sabah Hospital, Children Department, Child Neurology
Unit, State of Kuwait
Canavan disease is a neurodegenerative autosomal recessive
disease associated with disorders in the synthesis or
catabolism of myelin due to deficiency of aspartoacyclase
caused by mutations in the gene for this enzyme. The gene
has been mapped to the short arm of chromosome 17. It is a
panethnic disease more prominent among Ashkanazi Jews.
We report nine children with Canavan disease from eight
families. The diagnosis was confirmed by the elevated N-
acetylaspartic acid in urine and brain imaging abnormalities.
The eldest one is 8 years and the youngest is 6 months,
one died at 3 years. We describe the clinical features, diagnostic
procedures and note the need to elucidate the gene
mutation among Arab Ethnic group.
FP-K-021
A family of episodic ataxia type 2: no evidence of genetic
linkage to the CACNA1A gene on chromosome 19p13
H. Hirose a , T. Arayama b , J. Takita a , T. Igarashi a ,Y.
Hayashi a , Y. Nagao a,c
a Department of Pediatrics, The University of Tokyo Tokyo,
Japan; b Kikkoman General Hospital and c Social Health
Insurance Medical Center, Japan
Episodic ataxia type 2 (EA2) is an uncommon autosomaldominant
disorder, which is characterized by bouts of vestibulo-cerebellar
ataxia, lasting from 15 min to days, and
interictal gaze-evoked nystagmus. EA2 has been reported
to be caused by mutations in the CACNA1A gene, located
on chromosome 19p13, which codes the pore-forming
Ca v 2.1 (formerly known as a1A) subunit of a P/Q-type
voltage-dependent calcium channel. In this report we
describe a family with episodic ataxia, clinically indistinguishable
from EA2, which was not caused by CACNA1A
gene mutation. The proband is a 10 year-old boy, who has
had six cerebellar ataxic attacks since 8 years. His attacks
occurred almost monthly, lasting for 2–3 days. He was treated
successfully with acetazolamide. His maternal grandmother,
mother and identical twin had similar attacks
manifesting at 50, 34 and 10 years, respectively. The symptoms
in his grandmother improved gradually without medication.
His mother and identical twin took acetazolamide
with a good response. We examined the CACNA1A gene on
this family but did not detect any mutations. Furthermore,
linkage analysis, using the SNP in exon 46 and the variable
number of tandem repeats of GT repeats in intron 40,
showed that there was no evidence of genetic linkage
between the CACNA1A gene and the symptomatic patients
in this family. This suggests that the cause of EA2 can be
heterogeneous, that is, defects of genes other than
CACNA1A might be the cause of EA2.
FP-K-022
Clinical research of X-linked adrenoleukodystrophy
H. Xiong, Y.-H. Zhang, J. Qin, J.-X. Xiao, C.-Y. Shi, S.-M.
Zhou, X.-R. Wu
Department of Pediatrics, First Hospital, Peking University,
Beijing, China

564
Abstracts
Objective: The clinical manifestations, biochemical
changes and genetic counseling aspects of X-linked adrenoleukodystrophy
(ALD) were analyzed. Methods: Clinical
features of 29 cases with ALD were summarized and
analyzed. Results: Among these 29 cases, the clinical
phenotypes include childhood cerebral type (22 cases),
adolescent cerebral type (four cases), adrenomyeloneuropathy
type (one case), Addison disease only (one case), and
asymptomatic or presymptomatic (one case). Nine had positive
family history. Pedigree investigation was consistence
with typical sex-linked recessive inheritance. There were 45
ALD patients in these 29 pedigrees. The neurological manifestations
varied among members of the same family. Nine
cases died during follow-up. The causes of death were
central respiratory failure or other complications associated
with ALD. Laboratory tests demonstrated abnormally high
plasma levels of very long chain fatty acids (VLCFA) in
ALD patients; MRI demonstrated symmetric butterfly-like
T1 low T2 high signals in the parieto-occipital white matter.
The involvement in the splenium of corpus callosum led to
the convergence of bilateral lesions. It can progress forward
and involves the posterior limb of internal capsule bilaterally
and the temporal lobe, and the brainstem inferiorly.
Conclusions: Atypical initial symptoms of ALD were
seizures. The MRI showed abnormal signal in the cerebellar
white matter. The disease can impair the normal development
of children; this was more pronounced in the childhood
cerebral ALD type. It tended to progress rapidly with
dementia, vegetative state or death. We can now do antenatal
examinations. Emphasis should be placed on antenatal
examination, in order to achieve early diagnosis and to abort
pregnancy if indicated.
FP-K-023
Detection of mutations in ALD protein (ALDP) gene by
PCR-DNA sequence analysis in Chinese
adrenoleukodystrophy patients
H. Xiong, H. Pan, Y.-H. Zhang, X.-R. Wu
Department of Pediatrics, Peking University First Hospital,
Beijing, China
Objective: X-linked ALD is a peroxisomal disorder characterized
by impaired peroxisomal beta-oxidation of
VLCFAs. The ALD gene is localized on Xq28, has ten
exons and encodes a protein of 745 amino acids. The mutations
in ALD gene encoding adrenoleukodystrophy protein
in Chinese ALD patients were identified. Methods: Genomic
DNA from 14 unrelated patients (and two patients’
parent) with X-linked ALD was extracted using standard
procedures from the peripheral blood leukocytes. So far,
exon 6 at the 3 0 end portion of ALDP gene were amplified
with the primers synthesized according to the published
flanking intronic sequences. The PCR products were further
analyzed by agarose gel electrophoresis and directly
sequenced. Results: By DNA sequencing analyses, four
PCR products were identified. One was deleted with one
C nucleotide in the intronic sequences before the exon 6,
maybe induce cut disorder. The missense-type mutations:
T1559A was found in one patient and his mother. One other
had a silent mutation: G1548A. Conclusion: Mutations in
ALDP gene were identified in Chinese ALD patients in the
mainland of China for the first time. No major gene deletion
or rearrangement was detected. Despite many mutations
having been identified in patients with these clinical phenotypes,
the genotype-phenotype correlations have not been
clarified. Furthermore, there were no phenotypic expression
changes caused by the subtle amino acid changes. These
data indicated that no obvious correlations exist between
the phenotypes of ALD patients and their genotypes,
suggesting that other genetic or environmental factors may
also be involved in determining phenotypic expression in
ALD.
FP-K-024
Clinical, biochemical and genetic diagnosis in a family
with atypical Fabry disease
Th. Kroepfl a ,K.Paul a , B. Plecko a , P. Kotanko b , E. Paschke a
a University of Graz, Department of Pediatrics, Graz,
Austria; b Krankenhaus der Barmherzigen Brueder Graz,
Department of Internal Medicine, Graz, Austria
Fabry disease (McKusick 301500) is an x-linked deficiency
of lysosomal a-galactosidase A (EC 3.2.1.22) leading
to the accumulation of glycosphingolipids in the
lysosomes. As it mainly affects the skin, eyes, kidneys,
heart and the nervous system, the patients usually suffer
from severe pain attacks in childhood, later followed by
angiokeratoma, renal dysfunction and severe cardiomyopathy.
In a 42 year-old male mild proteinuria was noticed at
the age of 29, leading to end stage renal disease and kidney
transplantation within few years. At the age of 40 a marked
non-obstructive cardiomyopathy became obvious.
Although the majority of typical symptoms like paresthesia,
hypohydrosis or lesions of the skin and the cornea were
lacking, Fabry disease was histologically suspected due to
the result of a kidney biopsy and was biochemical
confirmed when leucocyte a-galactosidase was shown to
be reduced to 1.5% of controls. A novel insertion of 6
bases in exon 7 of the a-galactosidase gene on chromosome
Xq22.11 was found in the DNA of the patient but not
in 40 DNA controls. The mutation was also present in a
sister with cardiomyopathy and her asymptomatic daughter
but not in a second sister without symptoms. Surprisingly
the enzyme level was normal in all relatives examined.
These results illustrate importance of a complete genotype-specific
diagnosis in suspected Fabry patients in
order to provide the benefit of novel therapies to all male
and female patients of this probably highly underdiagnosed
condition. Thus, our patient will be the first to undergo
ERT in Austria.

Abstracts 565
FP-K-025
Eighteen years follow-up of a boy with congenital
disorders of glycosylation (CDG) type IIa syndrome:
clinical aspects
P. De Cock, J. Jaeken
Department of Paediatrics, University Hospital Gasthuisberg,
Leuven, Belgium
Because of a cardiac murmur (VSD) and dysmorphic
features J. was transferred soon after birth to our department.
In the following years several diagnoses have been
put forward to explain his congenital anomalies and severe
psychomotor retardation. They all proved wrong when at
the age of 9 years a striking pattern of haemostatic abnormalities
led to the discovery of a new type of congenital
disorders of glycosylation (CDG), namely CDG type IIa,
due to a deficiency in Golgi localised N-acetyl-glucosaminyltransferase
II (ref.). Surprisingly brain imaging showed
no cerebral or cerebellar anomalies. At 18 years J. is a very
dystrophic adolescent with short stature, profoundly
retarded, suffering from a refractory epilepsy, progressive
kyphoscoliosis and muscle wasting in more than one aspect
resembling a progressive myopathy (elevated GOT, normal
GPT). He learned to put a few steps unsupported but now he
is no more able to do so. Communication always remained
very limited. Moreover he has GE-reflux disease with recurrent
GI haemorrhages, gingival hypertrophy, sleep apnea
and an impressive osteoporosis. The coagulopathy almost
impedes any surgical intervention and furthermore several
drugs had to be discontinued because of side effects. His
clinical course will be presented and illustrated and therapeutic
dilemmas will be discussed.
FP-K-026
Familial moyamoya disease in two European children
H. Bazigou a , J. Tolmie b , K. Muir c , C. Fotopoulou d ,A.
Papavasiliou a
a Pendeli Children Hospital, Section of Pediatric Neurology,
Athens, Greece; b Medical Genetics Department, Yorkhill
Hospitals, Glasgow, UK;
c Institute of Neurological
Sciences, Southern General Hospital, Glasgow, UK; d Humboldt
University, Berlin, Germany
MMD is characterized by a cerebral angiographic picture
of stenosis or occlusion of the main cerebral arteries.
Although the etiology is still unknown, we present two
cases of familial MMD in order to stress the importance
of familial factors in the pathogenesis of the disease. The
first case refers to a 14-month old Greek girl with a right
hemiparesis after two brief right focal motor seizures. MRI
of the brain showed diffuse infarcts at the left tempo-parietal
and the right temporo-occipital regions. A digital cerebral
arteriography showed stenosis of the supraclinoid right and
left internal carotid artery and hypertrophy of the lenticulostriate
arteries. Her mother, suffering also from MMD, is
heterozygous for FV Leiden, for mutant FII (20210A) and
MTHFR factors, done by PCR and the patient is positive for
two of these factors. A revascularization procedure had been
performed in the mother at nine years of age and in the
patient recently. The second patient is a 5-year old boy,
coming from Scotland with a three-generation history of
MMD (maternal grandmother and sister and a sibling of
the patient), who during his summer holidays in Greece
developed fever, headache and left-sided motor seizures,
followed by left hemiplegia. MRI and bilateral carotid arteriography
confirmed the diagnosis of MMD (subtotal stenosis
of the initial sections of the right mainly and the left
middle cerebral arteries). Coagulation factors were normal.
Further studies are required to determine etiological factors
in familial MMD and to elucidate the role of hemostatic
abnormalities in its pathogenesis.
FP-K-027
CLN2 – Classic late infantile neuronal ceroid
lipofuscinosis (LINCL). Report on 13 Brazilian cases
S. Rosemberg, D. Fujiwara
Santa Casa of São Paulo Medical School, Department of
Pediatrics, Neuropediatrics Division, São Paulo, Brazil
LINCL is by far the most frequent neuronal ceroid lipofuscinosis
(NCL) encountered in Brazil and, perhaps in
Latin America comprising all but one NCL cases diagnosed
in our Service. We studied 13 unrelated patients (nine
males, four females). Consanguinity was present in eight
cases. Clinical phenotype was very uniform. The onset of
symptoms occurred between 2 years 7 months and 4 years 6
months (mean: 3 years 4 months). In three cases there was a
previous delay of speech and psychomotor development
was somewhat slow. Generalized tonic-clonic or astaticakinetic
seizures were the first symptoms which were
concomitant with or followed in a short period by arrest
and decline of the psychomotor skills. Global ataxia, pyramidal
signs and polymyoclonus were prominent and most
patients could not walk within 18 months after the onset.
Visual decline was difficult to assess due to the mental
regression. Funduscopy disclosed isolated optic atrophy in
three patients and macular degeneration associated with
optic atrophy in six. Three patients died at age 10 years,
five patients aged between 6 and 11 years are alive and
five were not followed up. EEG was abnormal in all
cases. Occipital photosensitive response was found whenever
searched (n ¼ 4). ERG performed in four patients was
extinguished. Cranial CT showed diffuse atrophy in all
patients whose severity increased with the patients’ age.
Vacuolated lymphocytes were never encountered. The
final diagnosis was done through the ultrastructural exam
of conjunctival biopsies performed in all patients, which
showed pure curvilinear storage bodies in fibroblasts,
endothelial or perithelial cells. Brazilian LINCL patients

566
Abstracts
form a very uniform clinicopathological population. Future
genetic studies will show whether there is a corresponding
genetic background.
FP-K-028
Renal lesions in tuberous sclerosis
T. Yamano, K. Tanaka, H. Hattori, T. Seto, O. Matsuoka
Department of Pediatrics, Osaka City University Graduate
School of Medicine, Osaka, Japan
In tuberous sclerosis (TS), the major causes of death
include status epilepticus, renal disease, brain tumors and
lymphangiomyomatosis of the lung. Child neurologists pay
special attention to treatment of intractable epilepsy, but
rarely to for renal involvement. This study was undertaken
to elucidate renal lesions in 14 patients with tuberous sclerosis.
They included six males and eight females between 3
and 37 years of age (mean: 14 years of age) who were
treated in our hospital. They were repeatedly examined by
abdominal echography, CT and/or MRI. Abnormalities
were found in ten patients. Multiple cysts were detected in
one kidney of a patient and in both kidneys of two, one of
whom developed renal failure at 13 years of age and then
received CAPD therapy. Other three female patients had
angiomyolipoma in both kidneys, which increased gradually
in volume year by year. In the remaining four patients,
kidneys revealed rugged surface and heterogeneous echodensity.
Our study indicated that prominent renal lesions
such as multiple cysts and angiomyolipoma occurred in
six of 16 patients (43%) with TS. Furthermore, these lesions
were progressive, occasionally resulting in renal failure.
Periodical evaluations of kidney structure and function are
required in TS patients.
FP-K-029
Infantile dentatorubral pallidoluysian atrophy: distinct
phenotype from juvenile form
T. Fujii a , M. Ito a , K. Ishikawa b , H. Mizusawa b ,T.
Miyajima a , Toshiyuki Kito a , T. Okuno a
a Department of Pediatrics, Shiga Medical Center for Children,
Moriyama, Japan; b Department of Neurology, Tokyo
Medical and Dental University, Tokyo, Japan
Dentatorubral pallidoluysian atrophy (DRPLA) is an
autosomal dominant neurodegenerative disorder associated
with the expansion of an unstable (CAG)n repeat in the
DRPLA gene. There are two clinical forms of DRPLA:
juvenile-onset DRPLA is characterized by progressive
myoclonic epilepsy and dementia; and adult-onset patients
commonly have choreoathetosis, cerebellar ataxia and
dementia. Shimojo et al. reported two infants with
DRPLA. Both had rapid regression and severe involuntary
movements without myoclonus, and an extremely
expanded CAG repeat ($90 repeats). We report an
infant-girl with regression, severe choreoathetosis followed
by dystonia, and the extreme expansion of the CAG repeat
(18/87); similar to two reported patients. Patient: A 5-yearold
girl had psychomotor developmental delay with the
maximum motor ability achieved being sitting and creeping.
The regression started at the age of 3, and she became
unable to sit or creep in 6 months. At age 4, she developed
severe choreoathetosis and oral dyskinesia. CT scan
showed cerebral and cerebellar atrophy. A few months
later, the choreoathetosis diminished, but dystonia became
apparent. Currently, she has severe dystonia and bulbar
symptoms. Discussion: The phenotype of our patient is
similar to that of Shimojo et al.’s two patients. All showed
the infantile-onset regression, involuntary movements
without myoclonus, cerebral and cerebellar atrophy, and
the very long CAG repeats (87 , 93 repeats). Thus, our
patient confirms the existence of another clinical form;
infantile DRPLA.
FP-K-030
Mutation of dystrophin gene of muscular dystrophy in
Javanese children in Yogyakarta Indonesia
Sunartini a , E.S. Herini a , P. Soeryantoro a , Harsono b
a Department of Pediatrics;
b Department of Neurology,
School of Medicine, Gadjah Mada University, Indonesia
Both Duchenne and Becker muscular dystrophies are
caused by dystrophin gene mutations. Due to the large
size of the dystrophin gene, the gene mutation occurs
frequently. This mutation was also found in Javanese Children
in Yogyakarta, Indonesia. Materials and methods: All
patients with DMD/BMD admitted to Dr. Sardjito Hospital
were included in this study. The diagnosis was confirmed by
clinical, laboratory and histopathological examination.
Multiplex PCR for DNA analysis was used to detect the
mutation of dystrophy gene. Results: Sixteen DMD/BMD
patients were included in this study. The mutations were
detected in eight cases (50%). Deletions occurred on the
hot spot exon of 6, 7–21, 12–19, 18–49, 45, 46 and 51. In
the other eight cases the mutations have not yet been
detected. Search for the mutation continues in the other
siblings and family members and will include carriers and
cases with normal clinical appearance.
FP-K-031
The clinical and experimental studies of metachromatic
leukodystrophy
W.-C. Zhang, H.-S. Wu, T.-C. Liu
Department of Neurology, Beijing Children Hospital, Beijing,
China
Objectives: To evaluate the clinical features of metachromatic
leukodystrophy (MLD) and diagnostic value of
measuring ASA activity in peripheral leukocytes. Methods:
The analysis was done in 6 patients with MLD according to

Abstracts 567
the clinical and experimental data. Results: There were five
cases of the late infantile form with onset of symptoms
ranging from 1 to 2.5 years of age and one case of the
juvenile form at 6 years. All had a gait disorder at onset
with a progressive course that resulted in spastic paraplegia
or tetraplegia in both legs or limbs. Speech disturbance
occurred in three cases and mental regression in three.
Cranial CT scan revealed symmetric low density area in
the cerebral hemisphere in three cases. Cranial MRI
showed bilateral symmetric T2-high signal intensity
white matter lesions on bilateral white matter in five
cases. Six cases had low or deficient ASA activity. Conclusion:
The clinical features of MLD are progressive motor
deterioration, speech disturbance and mental regression.
The white matter abnormalities on cranial CT and MRI
are useful for the diagnosis of MLD. The diagnosis is
based on the deficiency of ASA activity in peripheral
leukocytes.
FP-K-032
Leigh syndrome with mitochondrial DNA mutation at
14597 T-C
G.T Riordan a , P.M. Leary b , E.P. Owen a
a Department of Neurology and b Chemical Pathology, Red
Cross Children’s Hospital, University of Cape Town, Cape
Town, South Africa
A 16 month-old female presented with dystonia of
gradual onset over a month. Prior development was
normal. MRI of her brain aged 3 years demonstrated
symmetrical T1-WI hypointense abnormalities in bilateral
basal ganglia. At 10 years clinical deterioration accelerated.
Dystonia prevented ambulation and reflexes were
increased with extensor plantars. Virtually complete external
ophthalmoplegia was present. Irregular respiratory
pattern and severe hypertension suggested autonomic
disturbances. Repeat MRI showed symmetrical extension
of brainstem lesions. Pyruvate dehydrogenase activity was
normal. No mitochondrial (mt) mutations were found at
base pairs 3271, 3243, 8344, 8993 or sites associated
with Leber’s optic atrophy. A point mutation was encountered
at nt T14597C altering amino acid 26, isoleucine, to
threonine. Degree of heteroplasmy in fibroblasts was over
95% with no normal sequence being seen. The mutation
occurred in a highly conserved region of the ND6 gene and
changed a non-polar to a polar amino acid. Normal
sequence was present in her mother. This amino acid
change could potentially cause an unstable subunit of
complex 1. A mutation at ntT14596A changing isoleucine
to methionine was described in a Dutch family with hereditary
spastic dystonia and optic atrophy. (Am J Hum Genet
1996;58:703–711). It is likely that a T14597 C mutation in
mtDNA was responsible for causing Leigh Syndrome in
this patient.
FP-K-033
Evaluation of the floppy infant: contribution of genetic
and neurological disorders
A.N. Prasad a , K. Birdi a , C. Prasad b , M. Cheang c ,B.
Chodirker b , A.E. Chudley b
Sections of
a Pediatric Neuroscience, and b Genetics and
Metabolism; Department of Pediatrics and Child Health,
Biostatistical Consulting Unit; c Department of Community
Medicine, University of Manitoba, Winnipeg, Canada
This study examines in particular the impact of advances
in DNA based diagnostic testing, and neuroimaging on the
laboratory work up in the floppy infant. We present a retrospective
analysis of 89 infants referred for evaluation to the
genetics and neurology services over 11 years (1990–2000).
Cases were ascertained through a systematic search of
genetics/neurology clinic databases, and EMG records.
There were 47 males, 42 females. The majority (83.7%)
presented below the age of 1 mo. The aboriginal population
is over represented in this patient group (37.5 versus 15%),
in comparison to the expected proportion of the aboriginal
population. Operative delivery was necessary in 26.4%
(16.2–36.6, 95% CI), persistently low Apgar scores (,3 at
5 min) were noted in 6% (0.3–11.6, 95% CI). The majority
(56.5%) of infants were delivered at term (45.9–75.1, 95%
CI), and with birth weights between 2500 and 3000 g 61%
(46–75.9, 95% CI). These infants were followed up for a
mean duration of 29 months (24.4–33.5, 95% CI). Genetic
disorders 29% (17.7–40.3, 95% CI), structural brain malformations
35.5% (23.6–47.4, 95% CI) and disorders of the
motor unit 17.7% (8.2–27.3 95%CI) were most frequently
encountered. DNA based diagnostic tests and neuroimaging
studies (CT) were more likely to lead to the final diagnosis
than other tests (P , 0:05). There is significant mortality
(11.6%), and considerable morbidity; 60% showing global
developmental delay, nearly half (56.6%) remaining non
ambulatory, and a small proportion (2.6%) requiring home
ventilation. A diagnostic algorithm for appropriate utilization
of laboratory services based on the findings of the study
is presented.
FP-K-034
Clinical survey of 138 patients with organic aciduria
Y.-L. Yang, Y.-H. Zhang, Y.-W. Jiang, X.-H. Bao, Y. Qi, J.
Qin, X.-R. Wu, S. Yamaguchi, X. Fu, M. Kimura T. Yasiko,
H. Jun, F. Minoru
Department of Pediatrics, The First Hospital of Peking
University, China; Department of Pediatrics, Shimane
Medical University, Japan, and Sapporo Health Institute,
Japan
Objective: To investigate the incidences, clinical features
of organic aciduria in children with mental retardation and
other neurological defects. Subjects and methods: In the past

568
Abstracts
4 years, 1372 patients with mental retardation, seizures,
motor deficit, vomiting, growth disorders, and metabolic
acidosis or lethargy were screened by urine organic acid
analysis (gas chromatography/mass spectrometry). Results:
Seventy-one (5.14%) patients aged from 5 days to 18 years
with typical organic aciduria were confirmed. Among them,
32 were methylmalonic aciduria, 13 were propionic aciduria,
six were multiple carboxylase deficiency, five were
glutaric aciduria, four were hyperoxalic aciduria, three
were oxoprolinemia, two were methylcrotonyl CoA carboxylase
deficiency, one patient with maple syrup urine disease,
one ketothiolase deficiency, one isovaleric aciduria, one
alcaptonuria, one carnitine palmitoyl transferase deficiency
type II, one glyceric aciduria, respectively. In addition, 67
cases (4.88%) of atypical organic aciduria (such as dicarboxylic
aciduria) were detected. Among the above 138
patients, mental retardation, seizures, metabolic acidosis,
vomiting, and SIDS like strike were in 115 (83.3%), 92
(66.7%), 68 (49.3%), 83 (60.1%) and 14 (10.1%) patients,
respectively. A total of 105 (76.1%) patients were treated.
Nineteen (13.8%) died. Clinical improvement was observed
in 94 (68.1%) patients. Twenty-six (18.8%) showed normal
mental development. Conclusion: Most of organic aciduria
involved nervous system intensively. Early diagnosis and
adequate treatment contributed to the improvement of the
mental prognosis of the patients, and screening is critical.
GC/MS was a very important method to the diagnosis for
organic aciduria.
FP-K-035
Autosomal recessive centronuclear myopathy and
cataract: report of a large pedigree
N. Şenbil, Z. Akçören, G. Tuncer, A. Özön, H. Topaloǧlu
Hacettepe Children’s Hospital, Ankara, Turkey
There are three basic inheritance patterns of centronuclear
myopathy (CM). The gene of the X-linked form maps
to Xq28 and codes for a protein called myotubularin. The
autosomal recessive and dominant forms are rare, and their
genetic loci are unknown. Here, we present a large family
in which three out of seven siblings were affected with
autosomal recessive CM. Interestingly, cataracts were
also segregating within the members. All patients showed
hypotonia from infancy, delay in motor milestones and
weakness. There was generalized muscle weakness, facial
involvement, waddling gait, lordotic posture, and absent
deep tendon reflexes. Serum CK was normal. There were
myopathic alterations in the EMG. The evolution was relatively
non-progressive. The eldest affected sib is an 18
year-old boy with bilateral cataracts (removed), ptosis,
ophthalmoplegia, and long and thin face. The second one
was a 15 year-old girl. She had all the findings of the
brother except the cataracts. A 4 year-old-girl was the
third affected sib and her complaints have just begun.
There was also a 22 year-old girl only with cataracts. We
have no detailed information for the nature of the cataracts
as they were removed many years before our evaluation of
the family. There was no known consanguinity, but both
parents were from the same village. Muscle biopsy was
obtained from two of the affected patients. The pathological
studies revealed a prominent variety in fiber size,
centrally placed nuclei in most of the fibers and adiposis
with ATPase type I fiber predominance and with succinic
dehydrogenase myofibrillar disruption and many motheaten
fibers were present. These findings were compatible
with CM. This family may be a candidate for further
genetic analysis to help to search a genetic locus of autosomal
recessive CM.
FP-K-036
Profile of clinical and biochemical characteristics of 41
pediatric patients with cerebral lipidosis
A. Nalini, R. Christopher, D.K. Subbakrishna
Department of Neurology, Neurochemistry and Biostatistics;
National Institute of Mental Health and Neuro
Sciences, (NIMHANS), Bangalore, Karnataka, India
A retrospective study of 41 patients seen between 1993
and 2001 was done. The clinical features and biochemical
parameters were studied. Results: There were 20 cases of
metachromatic leukodystrophy (MLD) (infantile-14, juvenile-6);
Tay-Sachs disease (TSD)-12 (infantile-9, lateG M2 -
3); Sandoff disease (SD)-8; and one of multiple sulfatase
deficiency. 2 0 consanguinity seen in nine of MLD, six of
TSD, three of SD, 3 0 in one from each group. The M:F ratio
was: MLD (13:7); TSD (4:8); SD (6:2). There were 38
Hindus and three Muslims. The mean age at onset was
39.5 ^ 44.2 months in MLD; 10.0 ^ 8.9 months in TSD;
7.7 ^ 5.5 months in SD. Delayed milestones seen in 80% of
MLD; 75% of TSD and 88% of SD. Macrocephaly seen in
one of SD and one of MLD. Seizures seen in 45% of MLD;
50% of TSD; and 75% of SD. Startle myoclonus was seen in
100% of TSD, 75% of SD, 10% of MLD. Visual loss was
present in 42% of TSD, 50% of SD, 10% of MLD. Cherry
red spot was seen in 75% of TSD and 86% of SD. Optic
atrophy in 30% of MLD, 42% of TSD and 25% of SD.
Deafness in one case of TSD and SD. Spasticity with
brisk DTRs in 32 and absent DTRs in eight. Babinski positive
in 33, cerebellar ataxia in three of MLD and two of
TSD, dystonia of limbs in three of MLD. Family history
of similar illness in eight of MLD, one of SD and TSD.
The mean serum arylsulfatase-A level in 20 of MLD was
20.7 ^ 7.1 (10–36) nmol/h. The mean serum b hexosaminidase-A
level in 12 of TSD was 7 ^ 6.9 (3–23) nmol/ml
per h. The mean total serum b hexosaminidase level in eight
cases of SD was 37.3 ^ 32.8 (0–62) nmol/ml per h. The
MSD case had low level of arylsulfatases A and B in
serum and in leucocytes. NCV showed motor sensory
demyelination in eight of MLD. Sural nerve biopsy was
positive for metachromatic material in six of MLD. Conclu-

Abstracts 569
sion: This is the largest series of biochemically confirmed
cases from India.
FP-K-037
MECP2 gene mutation analysis in patients with Rett
syndrome in China
H. Pan a , Y.-P. Wang b , X.-H. Bao a , H.-D. Meng a , Y. Shen b ,
X.-R. Wu a
a Department of Pediatrics, Peking University First Hospital,
Beijing, China; b Chinese National Human Genome
Center, Beijing, China
RTT is a progressive neurodevelopmental disorder that
almost exclusively affects girls. Mutations in the X-linked
methyl-CpG-binding protein 2 (MeCP2) gene have been
found to be a cause of RTT. Methylation of CpG in the
mammalian genome is important for transcriptional silence.
Decreased MeCP2’s activity will lead to derepression of
transcription at multiple central nervous system loci and
overexpression of some genes that may be detrimental
during brain development. The developing brain may be
more dependent on MeCP2 than any other tissues. A lot
of mutations have been reported in different races recently.
In order to study the spectrum of mutations in China, we
employed PCR and direct sequencing to analyze the
MECP2 coding region in 31 classical sporadic RTT Chinese
cases. Twelve different mutations in exon three were identified
in 17 of these 31 patients, including four missense
mutations (R133C, T158M, P225R, R306C), three nonsense
mutations (R168X, R255X, R294X), five frameshift
mutations (806delG, 875insA, 1158–1167/1171–
1186del24, 1164del44, and 1152del44 bp). And there was
1 bp variant at the intron (G21830A). All of missense and
nonsense mutations except R168X were located either in the
methyl-CpG binding domain or in the transcriptional repression
domain. The three large deletion mutations were
located in a hot spot for deletion that has been reported. A
missense variant (P381G) was detected in a patient and her
father. Mutations in MECP2 gene were considered to be a
cause for Rett syndrome, and can be found in about 55%
cases in our study.
FP-K-038
Citrullinuria: report of one case
Q. Chen, K.-M. Xu, L.-W. Wang
Capital Institute of Pediatrics, Beijing, China
Citrullinuria is a genetic disease caused by deficiency of
argininosuccinate synthetase (ASS). ASS is a urea cycle
enzyme that catalyzes the synthesization of argininosuccinate
from citrulline and aspartate. The deficiency of ASS
results in hyperammonemia and increased citrulline in urine
as well as a variety of symptoms. A 5-year-old girl was
admitted to our department because of sleeplessness at
night. She was born after a full-term pregnancy by normal
natural delivery and was breast-fed. Her mother was in good
health and had no illness during her pregnancy. Physical
examination revealed normal vital signs, length, body
weight and head circumference. The patient had a massively
enlarged liver, 4 cm below the costal margins. She had no
splenomegaly. There was no lymphadenopathy or skin rash.
She had mild mental retardation (IQ ¼ 65). Her laboratory
findings were mild hyperammonemia (142 mg/dl, normal
,100 mg/dl). Citrulline in her urine determined by GC-
MS was high (14882.3 nmol/mg, normal ,80 nmol/mg).
Complete blood counts were normal; electrolytes and
blood biochemistry test and blood gas analysis were within
normal limit. She was started on a low protein diet after
diagnosis.
FP-K-039
Analysis of clinical manifestations and follow-up in 130
patients with Wilson’s disease
J.-F. Lu, Q.-X. Shui
Department of Neurology, Children’s Hospital, College of
Medicine, Zhejiang University, Hangzhou, China
Objective: To investigate the clinical and laboratory
manifestations, and to improve diagnosis accuracy and the
quality of patient’s life. Methods: A total of 130 cases with
Wilson’s disease during 1974–2001 were analyzed and 53
of them were followed up for 1.5–16 years. Results: Seventy
patients presented with liver manifestations (about 53.8%),
45 cases were found neurological damage (36.3%). Fiftyone
cases were CT scanned, with 33 to be found abnormal.
A total of 105 of 130 patients (80.8%) were misdiagnosed at
the beginning. Fifty-three patients were followed up, and 12
of them died from this disease. It was found that early diagnosis
and treatment could lead to good outcome. Conclusion:
Patients with Wilson’s disease are usually
misdiagnosed, therefore it was essential to do laboratory
examination and CT scan to the patients with unknown
liver damage and neurological symptoms. Early diagnosis
and long-term supervised treatment are very important for
good outcome.
FP-K-040
Neurological assessments of phenylketonuria (PKU)
patients
Z.-S. Zhou, W.-M. Yu, C.-N. Zheng, P.-F. Xu
Pediatric department, China-Japan Friendship Hospital,
Beijing, China
Objective: To explore the influence of PKU on neurological
system (NS), 563 PKU patients were assessed for
neurology in this study. Subjects and method: A total of
563 PKU patients (aged 2 days–7 years old) were divided
into two groups. One hundred nine early-treated cases (,3

570
Abstracts
months old) including 82 screened neonates, and 454 latetreated
cases (.3 months old). All underwent clinic neurological
assessments. DQ and IQ were used to evaluate
mental retardation. Morphological changes of brain were
evaluated with Staudt’s staging system about brain myelination
and MRI grading method proposed by Thompson et
al. Results: Among the early-treated group, there was no
NS abnormality in 82 screened cases and mild neurological
abnormality in 27 cases. Among the late-treated cases, a
few patients displayed mental retardation, epilepsy, disturbance
of speech, behavior disorders, affective disorder and
motor retardation. Fine motor defect was prominent among
the movement dysfunction. The later treatments started, the
severe damage of NS occurred. Abnormal EEG records
were found in 68% of 360 cases. MRI scans in 73 cases
before treatment revealed abnormal high T 2 -signal intensity
in the deep white matter and delayed myelination.
Follow-up of 210 cases showed that after specific dietary
and anti-epileptic treatments, except mental retardation,
most NS symptoms had different degrees of recovery.
The neuroimage abnormalities were also improved partly.
Conclusion: Late-treated PKU cases had obvious NS
damage, and the mental retardation was more prominent
than motor retardation. The later treatment started, the
severer damage of NS occurred. It is very important to
carry out nationwide early neonatal screening program of
PKU.
FP-K-041
MECP2 mutations in Swedish Rett syndrome clusters
F.-Q. Xiang, Y. Stenbom, M. Anvret
Department of Clinical Neuroscience, Karolinska Institute
CMM-L8-02, Karonlinska Hospital Stockholm, Sweden
Previous genealogic studies on Swedish females with
RTT documented an increased rate of common ancestry,
with a high percentage originating generations back in the
same small area, ‘Rett area’ (homested). These data indicate
that the disease transmission starting with a premutation
that over generations can result in a full mutation
giving rise to RTT, such as trinucleotide repeat expansion
disease. The discovery of the RTT gene, MECP2 made it
interesting to investigate the MECP2 gene in the RTT
clusters to clarify the hypothesis of genetic transmission.
In this study, RTT patients from three so-called RTT clusters
were investigated for MECP2 mutation. Our data indicate
that not all patients belonging to the same cluster had
mutations and those who were positive harbored different
mutations. This suggests that the Swedish RTT girls
belonging to the same clusters may not be genetically
related to each other and theMECP2 is not the only explanation
to RTT.
FP-K-042
Pathogenetic significance of the CLN2 protein in lateinfantile
neuronal ceroid lipofuscinosis
Y.K. Hanafusa b,c , A. Oka d , M. Itoh b , M. Mizuguchi e ,M.
Hayashi f , Y.-I. Goto b , S. Takashima a
a National Institute of Neuroscience, National Center of
Neurology and Psychiatry (NCNP); b Department of Mental
Retardation and Birth Defect Research, National Institute of
Neuroscience, NCNP; c Department of Pediatrics, Faculty
of Medicine, University of Tokyo;
d Division of Child
Neurology, Institute of Neurological Sciences, Faculty of
Medicine, Tottori University; e Department of Pediatrics,
Jichi Medical School; f Department of Child Neuropathology,
Tokyo Metropolitan Institute of Neuroscience,
Kodaira, Tokyo 187-8502, Japan
NCLs are hereditary progressive neurodegenerative
diseases during childhood. The LINCL is caused by a mutation
of the CLN2 gene, which encodes a lysosomal protein
of 46–49 kDa. We previously raised polyclonal antibodies
against the CLN2 gene product, and confirmed that they
recognize the CLN2 protein on Western blots. To clarify
when and where the CLN2 protein is expressed in human
organs, we examined tissue specimens (brain and visceral
organs) and cultured cells (fibroblasts) of both NCL patients
and normal controls, by immunohistochemistry and
Western blotting. Immunostaining of control tissues showed
that the CLN2 immunoreactivity was ubiquitous in extracerebral
organs as well as in the central nervous system.
Intense immunoreactivity was seen in cells with phagocytic
function, such as the Kupffer cells and pericytes. In pericytes,
immunoreactivity was seen from the neonatal period.
In the brainstem, CLN2 expression was already seen in the
perinatal period. In the cerebral cortex and brainstem,
neurons express the CLN2 protein, which increased with
development, and reached the adult level at the age of
around 2–3 years. In tissues and cells of LINCL patients,
CLN2 expression was absent. The developmental profile of
CLN2 expression in cortical neurons may be related to the
clinical onset of LINCL.
FP-K-043
A novel mitochondrial mutation T3386C presenting with
mental retardation, hypoparathyroidism, distal renal
tubular acidosis and lactic acidosis
S.K.H. Tay, K.S. Poh, K.Y. Loke
Department of paediatrics, National university of Singapore,
Singapore
We report a novel mitochondrial mutation in a child with
an unusual presentation of mitochondrial disorder. The child
was 16 years old and was the fourth child of non-consanguineous
parents, with a significant family history of two
maternal aunts with recurrent pathological fractures and

Abstracts 571
acidosis. He presented with mental retardation, recurrent
bone fractures and short stature. He had distal renal tubular
acidosis and hypoparathyroidism and was found to have
mild lactic acidosis once only despite repeated testing. He
was found on direct PCR sequencing of the ND1 gene to
have a novel point mutation of the ND1 gene T3386C. This
was a missense mutation that coded for a change in amino
acid isoleucine to threonine at codon 27. This case illustrates
the diversity of presentation of mitochondrial disorders
and the need to consider mitochondrial disease as a
diagnosis in a patient with multi-organ pathology even in
the absence of persistent lactic acidosis.
FP-K-044
Rett syndrome brain neuromorphology: peculiar
adaptive and protective properties of neurons in
entorhinal cortex and thalamic motor nuclei
L.A. Bereshnaya a , J.K. Mukhina a , P.V. Belichenko b
a Brain Research Institute RAMS, Moscow, Russia; b Department
of Neurology and Neurological Sciences, Stanford
University, Stanford, USA
RS is a neurodevelopmental disorder affecting young girls
with most cases of this disease associated with mutation in
the X-linked MeCP2 gene. RS patients have severe motor
and cognitive dysfunction. Detailed morphological analysis
demonstrated cell loss, increase in neuronal and glial density,
reduced white matter volume and changes in the dendritic
geometry of neurons. It is practically important to study
morphological changes in single neurons related to both
degenerative and compensatory events. The aim of the
present work was to study the peculiarities of adaptation
reactions in neurons of thalamic nuclei (ventral anterior
and ventral lateralis) and in entorhinal cortex, the areas
which were most affected in RS. Six cases of RS brains (8–
12 years old) were studied by classical Nissl and Kluver-
Barrera methods. Destructive changes included swelling of
neuronal soma and dendrites, diffuse or peripheral chromatolysis
of neurons, neuronal hyperchromia, vacuolization
and presence of ‘ghost’ cells. Also, compensatory adaptation
in neurons could be seen on the RS brains: juxtaposition of
two neurons with joint cytoplasm and/or with common
surface contact, formation of neuronal clusters from similar
cells. These adaptive changes could be directed to maintain
survival functions for these modified neurons.
FP-K-045
Leber’s congenital amaurosis: neurological and systemic
findings
E. Fazzi a , S.M. Bova a , S.G. Signorini a , J. Lanners b ,G.
Lanzi a
a Department of Child Neurology and Psychiatry - IRCCS C.
Mondino, Pavia, Italy; b R. Holmann Foundation, Cannero
Riviera (VB), Italy
Leber’s congenital amaurosis (LCA) is the earliest and
most severe form of inherited retinal dystrophies. It is an
autosomal recessive disease, which accounts for 10–18% of
congenital blindness. Its incidence is 2–3 per 100 000
births. Currently accepted diagnostic criteria refer to ocular
findings, but neurodevelopmental delay, mental retardation,
and systemic anomalies have been frequently reported. We
studied a sample of 35 children affected by LCA (mean age
at first observation: 19 months, range: 6–50 months) in order
to clarify the clinical aspects of this disorder, and to contribute
to a new classification, correlating genotype with
phenotype. All subjects underwent neurological and
neurophthalmological examination, neurophysiological
examinations (ERG, VEPs), EEG, BAEP, brain MRI,
screening for metabolic disease (mitochondrial and peroxisomal
disorders) and systemic involvement (abdomenpelvis
US, hand X-ray). A developmental assessment
using specific Scales for visually impaired children, and
videotape (for stereotypic behaviours-SB) were also
performed. Neurological examination was normal except
for mild muscular hypotonia, mental retardation was documented
in 20%, SB were very frequent (18–93% depending
on the kind of SB), EEG showed floating alpha (21%) or
slow activity (21%), pathological findings on brain MRI
were detected in the 53% (ventricular enlargement, mild
cerebral atrophy, white matter abnormalities, median line
cysts). Systemic abnormalities were detected in a small
but significant percentage of subjects. Our data confirm
that LCA is a heterogeneous entity which can present itself
as an isolate ocular pattern, or associated to neurological
and systemic abnormalities, and support the need for a
multidisciplinary approach and for genotypic-phenotypic
studies.
FP-K-046
Rett syndrome cases at the Philippine Children’s
Medical Center (1989–2001)
M.H. Ortiz, L.V. Lee, E.L. Avendaño
Child Neuroscience Division Philippine Children’s Medical
Center Quezon Avenue, Quezon City Philippines
Rett syndrome is an X-linked neurodevelopmental disorder
affecting mainly females characterized by an apparently
normal psychomotor development for the first 6
months of life. After this age, developmental progress
slows. Eighteen cases of Filipino girls with Rett syndrome
diagnosed over a 12-year period will be presented. Their
clinical manifestations as well as temporal profile will be
described in detail. Age at onset ranged from 6 to 24
months and age at initial consult was 1.1–5.10 years. Thirteen/18
had an initial diagnosis of Rett syndrome, three
were initially diagnosed to have autism and two were diagnosed
to have psychomotor retardation of unknown etiology.
All had a history of an apparently normal prenatal and
perinatal period with 15/18 presenting with regression of

572
Abstracts
previously learned skills. Of the 11 patients with head
circumference measurements noted at birth, ten (90%)
had normal head circumference while one (10%) had a
head circumference below the 2nd percentile. Hand
apraxia, repetitive stereotypic hand movements, impaired
receptive and expressive language with psychomotor retardation
as well as truncal and gait ataxia was seen in all
patients. Nine/18 children had breathing dysfunction.
Seventeen presented with an abnormal EEG and 13 were
diagnosed to have seizure disorder.
FP-K-047
Mutation analysis of methyl-CpG binding protein gene
family in autistic patients
H. Li, T. Yamagata, M. Mori, M.Y. Momoi
Department of Pediatrics, Jichi Medical School, Minamikawachi,
Tochigi, Japan
Autism is a complex neurodevelopmental disorder with
multigenic etiology. Methyl-CpG binding protein 2 gene
(MeCP2) mutation was reported to be the cause of Rett
syndrome, and was also involved in X-linked mental retardation
and autistic disorder. Methyl-CpG binding protein
family consists of MeCP2, MBD1, MBD2, MBD3 and
MBD4, sharing the common methyl-CpG binding domain
(MBD). All MBDs except for MBD4 form complexes with
histone deacetylases and are involved in recruiting histone
deacetylases to methyl-CpG enriched regions in the genome
to repress transcription. Gene silencing is suspected to be
involved in etiology of autism. Thus, we screened these
genes as candidate genes for disease-causative mutations
in autism patients using denaturing high-performance liquid
chromatography (DHPLC) followed by sequencing. We
detected several polymorphisms and found some interesting
base changes. In MBD1, one novel missense mutation,
R269C, in the exon 9 was detected in one patient. It was
also detected in his father, who also has some autism-like
symptom and his normal sister, but not in 100 normal
controls. This mutation may relate to the genesis of autism
although normal sister had same mutation, relating to sex
bias or some other gene’s combination. In MBD2, two
repeat variants (GGC and GGGGCC) in exon1 were
detected in five patients. Although these were also detected
in normal control, it looks interesting to screen more
patients because more expansion out of the range of PCR
may exist and relate to the disease. Autism is thought to be
polygenic disease and methyl-CpG binding proteins family
genes’ high-polymorphic nucleotide changes may have
some effect on the etiology of autism. Further analysis on
more samples is required to clarify their contribution to
autism.
FP-K-048
Down syndrome: a link between development and aging
I.T. Lott, E. Head, L. Nelson, K. Osann, E. Doran
University of California, Irvine Departments of Pediatrics
and Neurology; Institute on Brain Aging and Dementia UCI
Medical Center, Orange, CA, USA
The brains from individuals with Down syndrome (DS)
show the changes of Alzheimer’s disease (AD) by age 40
years. We have shown that b amyloid (Ab) is deposited in
DS beginning in childhood (Exp Neurol 2000;163:111) and
is associated with inflammatory and oxidative changes in
the course of brain maturation (Neurbiol Dis 2001;8:792).
The topography of cell loss secondary to AD in DS involves
cholinergic neurons and recent reviews have suggested that
a pathological cascade may involve both Ab and cholinergic
dysfunction in the disorder. Based upon these observations,
we undertook a pilot investigation of an
acetycholinesterase inhibitor (donepezil) in 15 patients
with trisomy 21 for a 5-month period. Treated (n ¼ 9) and
control subjects (n ¼ 6) were comparable with respect to
initial level of dementia, length of follow-up, and age.
Utilizing the Down Syndrome Dementia Scale (Gedye,
1995), the subjects treated with donepezil showed an
improvement in test scores (mean ¼ 24.3, SEM ¼ 4.0)
after 5 months compared to the control group
(mean ¼ 30.7, SEM ¼ 3.0) with the results statistically
significant at P ¼ 0:03. No serious side effects of the drug
were encountered. The results of this pilot study suggest that
further placebo-controlled trials of acetycholinesterase inhibitors
are indicated for treatment of dementia in DS
(supported in part by AG 05142).
FP-K-049
Describing the phenotype in Rett syndrome
H. Leonard a , L. Colvin a , S. Fyfe b , J. Christodoulou c,d ,L.
Raffaele c,d , S. Leonard a , T. Schiavello a , C. Ellaway c,d ,M.
Davis e , N. De Klerk a
a TVW Telethon Institute for Child Health Research, Western
Australia; b Curtin University of Technology, Perth, Western
Australia; c Children’s Hospital at Westmead, Sydney New
South Wales; d Department of Paediatrics and Child Health,
University of Sydney, Sydney, New South Wales; e Royal
Perth Hospital, Perth, Western Australia
The Australian Rett syndrome epidemiological research
program is internationally unique with ongoing case ascertainment
on a population basis since 1993. By July 2000,
199 verified cases had been reported to the database and
comprehensive phenotype data were collected on 152 of
these. By end of 2001 there were 227 verified cases, fourteen
of whom had died. Molecular testing has now been
undertaken on 77% (174/227) cases and X inactivation
status is complete on one third of the cohort. Of the 152

Abstracts 573
cases surveyed in the 2000 follow up 84 (55.2%) were categorised
as classical, 40 (26.3%) as early onset atypical and
28 (18.5%) as mild atypical. Using the scoring system
suggested by Kerr et al. (2001) we were able to show
considerable variation in clinical severity with a mean of
22.9, and a range from 9.3 to 32.0. Similar variation was
seen using the scale adapted by Percy from Amir et al.
(2000) and the scale designed by Monros et al. (2001) and
modified by Pineda. Age-related changes were seen with the
Kerr (20.5–24.2) and Percy scores (34.4–39.4) but not with
the Pineda score. By including a questionnaire version of the
WeeFIM (the Functional Independence Measure for Children)
(Msall et al., 1994) we were also able to calculate a
WeeFIM score. The mean WeeFIM score was 29 (range 18–
75). Mutations have been identified in 73% (68/93) of classical
and 65% (53/81) of atypical cases. The mean phenotype
scores for the three scales and the mean WeeFIM score
were calculated for each of nine common mutations. The
R133C mutation had a significantly lower Pineda score
(11.8) and a higher WeeFIM score (36) than the others
indicating better functioning. This was followed by the
R294 X with scores of 12.9 and 34.9. The poorest functioning
was for the R270 X and P152R mutations with Pineda
scores of 19.2 and 19.8 respectively. Overall the mean
WeeFIM score was higher in those cases with skewed
(31.9) than in those with random (25.6) X inactivation
(P ¼ 0:03). In the 27 atypical cases in which X inactivation
studies were complete, 10 of the 13 mild atypical cases had
skewing compared with four of 14 with early onset atypical
(P ¼ 0:01). In Australia ongoing case ascertainment
combined with active follow up of existing cases provides
a unique longitudinal profile of a genetically characterised
Rett syndrome cohort over a period of 10 years.
FP-K-050
Neurobehavioral findings in early and late treated
children with phenylketonuria
K. Yalaz, L. Vanlı, I. Ozalp, T. Coşkun, A. Tokatlı, M.
Özgüç
Hacettepe University Departments of Pediatric Neurology,
Metabolism, and Medical Biology, Ankara, Turkey
We evaluated 47 children with PKU treated before they
were 45 days old and 99 children treated after this age.
Neurological and neurobehavioral findings including head
circumference, motor performance, behavioral pattern,
intellectual capacity as well as phenylalanine level and
molecular analysis for phenotype-genotype correlation
were examined. Microcephaly, depressed or absent deep
tendon reflexes, tremor of the hands, mental retardation,
hyperactivity were present in both groups but were less
severe in early-treated children. Autistic signs were present
only in late-treated patients. Children whose clinical
response to diet restriction was unsatisfactory or less than
expected had additional medical problems: maternal PKU in
addition to the child’s encephalopathic episode, or other
metabolic disorder. The degree of impairment could differ
between siblings with the same genotype; in addition, earlytreated
siblings could have lower function than later-treated
ones. Further studies are needed to confirm the effect of
other genes modulating the phenotype in PKU.
FP-K-051
Preserved speech variants of classic Rett syndrome:
beneficial effects of long term l-carnitine therapy
M. Topcu, C. Akyerli, R.S. Kocoglu, A. Sayi, G. Haliloglu,
D. Yalnizoglu, T. Ozcelik
Hacettepe University Faculty of Medicine Department of
Pediatric Neurology, Bilkent University Department of
Molecular Biology and Genetics, Ankara, Turkey
RTT is a neurodevelopmental disorder of early childhood
that is primarily seen in girls. The gene responsible for this
disorder has been identified as MECP2. Although the type of
mutations in MECP2 have been found to be correlated with
the phenotypic manifestations of RTT to some degree, the
pattern of X inactivation appears to be the major determinant
in different studies including our own series. The disease is
incurable as yet, and treatment strategies are essentially
symptomatic and supportive including l-carnitine for
improvement in patient well-being and hand function. We
performed a detailed phenotype-genotype correlation in our
MECP2 mutation positive RTT patients (n ¼ 20) including
three girls who can be classified as preserved speech variants
of the disorder. Two of these girls, an 11-year-old who carries
the T158M mutation and a 4-year-old who carries the P152R
mutation, associated with the classic form of the disease. The
well established androgen receptor assay revealed that X-
inactivation patterns are random. Both of these girls have
been on 500 mg/day l-carnitine treatment since 18 months
of age. The third preserved speech variant carries a 44 bp
deletion at the 3 0 -end of the MECP2 gene, and mild phenotype
associated with these mutations has been reported in the
literature. These results may suggest that beneficial effects of
l-carnitine may go beyond an improvement in patient wellbeing
and hand function when administered at the early
stages of the disorder.
FP-K-052
Rett syndrome in Hungary: clinical and mutation
analysis of the Hungarian Rett syndrome girls
K. Hollódy a ,J.Kárteszi b , J. Bene b , M. Czakó b , B. Melegh b ,
G. Kosztolányi b
a Department of Pediatrics, b Department of Medical Genetics
and Child Development, University of Pécs, Pécs,
Hungary
Rett syndrome is a neurodevelopmental disorder. In a
high proportion of sporadic patients it is caused by muta-

574
Abstracts
tions in the X-linked MECP2 gene. Aim: To characterize the
spectrum of the clinical forms and the mutations in Hungarian
girls with Rett syndrome and to relate the genetic results
to the clinical features of the disease. Methods: So far we
have examined 22 patients registered in the Hungarian Rett
Syndrome Association. Two girls were twin with classical
features. Twenty patients had no relationships at all. Fifteen
suffer in the classical, seven girls in the atypical form of the
syndrome. One patient has preserved speech, another one
has tuberous sclerosis. A very thorough history was taken
from the parents of the girls and a detailed clinical examination
was performed by a pediatric neurologist, a clinical
geneticist, an orthopedist, a radiologist, a gastroenterologist
and a psychologist. Mutation analysis of MECP2 coding
region was done by direct sequencing. Results: Mutations
in the MeCP2 gene were found in 12/22 patients (54%).
Among them, all but four have the clinical signs of the
classical form, four have the atypical form. Eight already
described mutations were detected in ten patients (R294X in
three patients, R106W in two patients, in one each patient
R133C, R168X, R270X, P152R, T158M). Among the three
patients with the R294X mutation two have the classical and
one the preserved speech variant form of Rett syndrome. In
our oldest, atypical Rett syndrome patient (22 years of age)
a novel single base insertion in exon 3 (276insG) was
detected. A large deletion in exon 4 was found in a patient
with classical form. Similarly to the published Swedish data
no mutations of MECP2 gene were identified in the twin
Rett syndrome patients. According to the prevalence of the
disease there must be ca. 40–45 girls with Rett syndrome
aged 0–18 years in Hungary. Thirty different mutations
were found in 97 patients with RS. In 81% of the patients
we detected nonsense and missense mutations in the coding
parts of MECP2. Twelve different mutations and one polymorphism
were found. MECP2 may be involved in a
broader phenotype than classical RT including preserved
speech variants. Mutations were identified in 72% of
patients with classical RS.
FP-K-053
Pyruvate dehydrogenase deficiency: Clinical and
molecular analysis
A.M. Garcia, D. Wang, P. Kranz-Eble, D.C. De Vivo
Colleen Giblin Laboratories for Pediatric Neurology
Research, Columbia University, New York, USA
Defects in the pyruvate dehydrogenase complex are an
important cause of primary lactic acidosis, a frequent manifestation
of metabolic disease in young children. Clinical
manifestations are heterogeneous ranging from episodic
ataxia and weakness to chronic encephalopathy and congenital
lactic acidosis. The great majority of cases result from
mutations in the E1a subunit gene, located on the X chromosome
(Xp22.1). We present 19 patients (12 males) with pyruvate
dehydrogenase deficiency caused by mutations in the
E1a gene. Eleven patients (two females) showed early onset
with severe lactic acidosis, six of them associated with abnormal
neuroimages. Six patients (one male) developed a
chronic encephalopathy with diverse CNS malformations
in the five females. Two boys had a benign syndrome characterized
by recurrent limb weakness, muscular cramps and
exercise intolerance. Eight patients had genomic rearrangements;
all located in exons 10 and 11. Six patients had a
tandem repeat insertion (S370ins, Q382fs, S388ins,
G365ins, K387fs, N326ins) and two had a microdeletion
(S312fs, R310del). Four missense mutations were located
in the upstream region of the gene involving exons 4–6,
respectively. A number of previously recognized gender
specific hot spots for mutations were detected. Those associated
with females (S312fs, R302C, Q382fs) suggest that
these mutations are severe and probably lethal in male
fetuses, whereas those associated with males (K387fs,
R263Q, N164S, R127W, R310del) are presumably female
asymptomatic.
FP-K-054
Gene transfer to CNS by intrauterine administration of
adenovirus.
J.-S. Shen a , X.-L. Meng a , T. Ohashi a,b , Y. Eto a,b
a Department of Gene Therapy, Institute of DNA Medicine;
b Department of Pediatrics, The Jikei University School of
Medicine, Tokyo, Japan
Early and severe CNS involvement occurs in some lysosomal
storage disorders such as Tay-Sachs disease, type II
Gaucher disease and infantile form of Krabbe disease. In
these diseases, lysosomal storage and considerable neuropathology
was observed even prior to birth. Postnatal therapies,
including bone marrow transplantation, enzyme
replacement therapy and gene therapy are often insufficient
in the treatment of these diseases. Therefore prenatal gene
therapy to the CNS may be necessary. In order to investigate
gene transfer to the CNS prenatally, we administered
recombinant adenovirus encoding E. coli -galactosidase (-
Gal) to the lateral ventricles of the brain of mouse embryos
at mid-gestational period, and studied expression pattern of
the transgene at various time points. Results showed that
most of -Gal positive cells are distributed in the cerebral
cortex and some in the brainstem and cerebellar cortex.
Immunohistochemical observations using cell type specific
antibodies indicated that the most of them are neurons and
some are astrocytes. Histochemical and biochemical analysis
showed the expression of -Gal started as early as 2 days
after administration and persisted at least 2 months after
birth without significant decrement of expression level.
The results indicate that intrauterine administration of
adenoviral vector may be useful as a gene therapy strategy
for some lysosomal storage diseases with early CNS involvements.

Abstracts 575
FP-K-055
Frequency of major mutations in Ukrainian patients
with metachromatic leucodystrophy
N.V. Olkhovich, N.A. Pichkur, A.N. Nedoboy
Ukrainian Specialized Children’s Hospital ‘OKhMATDET’,
Kyiv, Ukraine
Nine patients with MLD from eight families were examined
using clinical, biochemical and molecular-genetic
methods. Three patients were diagnosed with late-infantile
form and the illness debut at the age of 12–24 months. Four
patients were diagnosed with juvenile form, the illness
began at the age of 3–7 years. Two patients were the siblings
and had adult MLD form. Arylsulfatase A (ASA) activity
was determined by the standard method of Baum A. et al.
All the examined patients had decreased ASA activity.
Mutations causing MLD were detected in the ASA gene
by polymerase chain reaction amplification of fragments
of the ASA gene and digestion by the appropriate endonuclease.
All patients were screened for ASA-pd mutation.
One homozygote for the splice mutation in intron 2
(459 1 1G ! A) and one compound heterozygosity with
splice mutation 459 1 1G ! A and missence mutation P
426L were detected. Compound heterozygosity with
P426L mutation and unknown mutation were detected in
two patients. The overall frequency of the 459 1 1G ! A
change was three of all 18 MLD alleles and of the P426
change was three of all 18 MLD alleles. Thus, these two
common alleles together accounted for 33% of all MLD
alleles.
FP-K-056
Disease progression in X-linked adrenoleukodystrophy
based on magnetic resonance imaging
D.J. Loes, A. Fatemi, E.R. Melhem, N. Gupte, L. Bezman,
H.W. Moser, G.V. Raymond
Suburban Radiology Consultant Ltd., Minneapolis, MN;
Department of Radiology, University of Pennsylvania,
Philadelphia, PA and the Kennedy Krieger Institute and
School of Public Health, Johns Hopkins Medical Institutions,
Baltimore, MD, USA
Introduction: X-linked adrenoleukodystrophy (X-ALD)
has many variants with outcomes that range from no deficits
to death. The purpose of this study was to evaluate the
degree to which MRI findings can predict MRI disease
progression in male X-ALD patients. Methods: Follow-up
MR Imaging studies were performed in 140 X-ALD patients
with cerebral involvement (median age: 12.3, range 1.7–
73.8). Data after bone marrow transplant was excluded.
Gadolinium enhanced axial T1W imaging was performed
in 40 of these patients. Depending on the anatomical location
of the primary involved area three patterns were defined
(pattern 1: parieto-occipital; pattern 2: frontal lobe; and
pattern 3: frontal-pontine/cortical-spinal projection fibers).
Regression analyses were performed to determine the
effects of multiple MRI factors on disease progression.
Results: Average follow-up time was 3.51 years (59 days–
11.1 years). The mean progression of the MRI score per year
was 2.2 ^ 0.55 for pattern 1 and 2.3 ^ 0.75 for pattern 2,
but was much lower, 0.4 ^ 0.16, in patients with pattern 3
(P ¼ 0:001). In patients with pattern 1, age (P ¼ 0:006) and
MRI severity score (P , 0:0001) at initial exam and
presence or absence of perilesional contrast enhancement
(P , 0:0001) accounted for 96% of the variability of MRI
progression after 1 year, while in patients with pattern 2 age
(P ¼ 0:05) and the initial MRI severity score (P , 0:0001)
accounted for 79% of the variability. Conclusions: Our
results demonstrate that differences in anatomical patterns
and perilesional contrast enhancement play a significant role
in prediction of disease progression and can serve as additional
disease markers in the evaluation and management of
X-ALD patients.
FP-K-057
Brain magnetic resonance imaging abnormalities in
females heterozygous for X-linked
adrenoleukodystrophy and association with X
inactivation in fibroblasts
A. Fatemi, P.A. Watkins, A.B. Moser, P.B. Barker, G.V.
Raymond, H.W. Moser, S. Naidu
The Kennedy Krieger Institute, Johns Hopkins Medical
Institutions, Baltimore, MD, USA
Objective: Although the degree of cerebral involvement
has been investigated extensively in affected males with X-
ALD, there is limited data for heterozygote females. The
purpose of this study was to determine the degree of brain
involvement in this group and to investigate the possible
association between MRI abnormalities and the degree of
X inactivation in fibroblasts. Methods: Conventional MRI
studies were performed in 75 X-ALD heterozygotes (mean
age: 42.2, median: 41.3, range: 13–75). Indirect immunofluorescence
analysis of cultured skin fibroblasts using an
antibody raised against the C-terminal 18 amino acids of the
human peroxisomal ALDP revealed a punctate, peroxisomal
immunostaining pattern and average percentages of
immunopositive cells were calculated. Results: Prominent
bilateral white matter T2 signal hyperintensities were
observed in two girls (13, and 14 years old) and a 42
years old women heterozygous for X-ALD. While in heterozygotes
with normal MRI a mixed population of normal and
abnormal cells was demonstrated, typically ranging from 20
to 80% normal ALDP positive cells, the three patients with
MRI abnormalities had 0, 1 and 7% (in the 14, 13 and 43
year old, respectively) immunopositive cells suggesting
skewed X inactivation. Conclusion: MRI changes similar
to cerebral childhood X-ALD in males were detected in
heterozygote females who showed an absence or a very

576
Abstracts
low ALDP expression in fibroblasts. These results suggest
that assessment of X inactivation in fibroblasts correlates
with the degree of brain involvement.
FP-K-058
Contiguous deletion of the X-linked
adrenoleukodystrophy gene and DXS1357E cause a
neonatal phenotype similar to peroxisomal biogenesis
disorders
S.J. Steinberg a,b , K. Johnson a , G.R. Cutting a,c ,P.A.
Watkins a,b , A.B. Moser a,b , H.W. Moser a,b
a The Kennedy Krieger Institute and b Department of Neurology,
c Institute of Genetic Medicine, Johns Hopkins University
School of Medicine, Baltimore, MD, USA
Plasma VLCFA analysis is a reliable screening tool for
the identification of patients with a perturbation of peroxisomal
fatty acid metabolism, including the disorders X-
ALD, peroxisomal biogenesis disorders (PBD) and other
single enzyme deficiencies (SED) such as D-bifunctional
protein deficiency. The spectrum of X-ALD clinical phenotypes
is readily distinguishable from PBD and SED.
Whereas PBD and SED patients have congenital malformations
and are ill from birth, patients with mutations in the X-
ALD gene, ABCD1, thrive postnatally. We recently identified
three male neonates presenting with severe hypotonia,
cholestatic liver disease and a failure to thrive. All three
died at less than 1 year of age and had increased plasma
VLCFA. Other peroxisomal pathways were normal and
ruled out a PBD. Immunocytochemistry indicated that
their cells lacked ALD protein, but otherwise had normal
peroxisomal structure. Mutation analysis showed that all
three patients had large deletions of ABCD1 that extended
beyond the promoter region and into the neighboring gene
DXS1357. We recommend naming this new disorder
‘Contiguous ABCD1 DXS1357E Deletion Syndrome,’ or
CADDS. Failure to identify X-linked CADDS may lead to
misdiagnosis as autosomal recessive PBD or SED and cause
serious errors in genetic counseling.
FP-K-059
Leukoencefalopathy with vanishing white matter: report
of five Brazilian cases
M.S.T. Souza a ,F.Kok a , C.C. Leite b , M.T.C. Lacerda b ,
M.O.R. Costa b , M.G. Otaduy b , A. Diament a , U.C. Reed a
a Departments of Neurology,
b Radiology of the School of
Medicine of São Paulo University, São Paulo, Brazil
In 1993 Hanefeld et al. reported an autosomal recessive
brain white matter abnormality clinically characterized by
early and slowly progressive developmental regression with
episodes of acute deterioration, cerebellar and pyramidal
signs but no remarkable mental deterioration. Brain MRI
showed a widespread leukodystrophy with subcortical cyst
formation. In the last 3 years, we evaluated five patients
from four families who developed such clinical and imaging
changes. All had widespread symmetrical cerebral white
matter involvement, characterized by MRI signal intensity
similar to CSF on all sequences, particularly on T2-
weighted intense signal. No gadolinium enhancement was
detected. In two patients, it was noted a partial preservation
of subcortical U-fibers, and in four individuals, the central
pontine tegmental tract and cerebellar white matter were
also involved. All patients had cavum septi pellucidi and
marked vermis cerebellar abnormality. Spectroscopic analysis
of frontal and parietal white matter revealed decrease but
not complete disappearance of N-acetyl aspartate signal and
height, and marked? slight? increase of choline and myoinositol
peaks. Lactate and glucose spectroscopic peaks
were normal in all patients. Very long chain fatty acids
level, arylsulfatase A and beta galactocerebrosidase activity
were in the normal range. Electroneuromyography was
normal in all patients. We conclude that leukoencefalopathy
with vanishing white matter can be a relatively common
condition in Brazilian children and that this diagnosis
should be considered in the presence of striking MRI
brain white matter changes associated with a chronic clinical
course with acute episodes of motor deterioration and
relatively preserved mental function.
FP-K-060
The participation of microglia in experimentally
induced phenylketonuria
G.G. Skibo a , J.A. McKanna b , O.V. Girnyk a
a Department of Cytology, Bogomoletz Institute of Physiology,
Kiev, Ukraine; b Department of Cell Biology, Vanderbilt
University, Nashville, TN, USA
PKU, the neurogenetic disease, is an inherited deficiency
of the enzyme phenylalanine hydroxylase which lead to
increase serum and brain phenylalanine and, as a consequence,
to mental retardation. The primary pathologic findings
during PKU are the loss of number of neurons,
hypomyelination and astrocytosis of central nervous system.
But participation of microglia in this pathological condition
remains unknown. The aim of the present investigation was
to follow the state of microglia in the rat brain with experimentally
produced PKU. The model of PKU was the next:
the rat pups were injected with l-phenylalanine and dl-?-
methylphenylalanine during 3–14th postnatal days. The
tissue of cerebellum and hippocampus was studied at
the14th postnatal day. Lipocortin 1 immunoreactivity
(LC1-ir) was used as a marker for microglia. The area and
the shape factor of microglial cells and density of microglia
in PKU brain were compared with control by computer
system BioQuant OS/2. The area of microglial somata
was larger and processes of microglial cells were shorter
and thicker in cerebellum and hippocampus of treated rats.
The density of LC1-ir microglia was higher in all layer of

Abstracts 577
hippocampus and cerebellum of the PKU brain as compared
with control pups, but the greatest increase in the number
was noted within white matter of cerebellum and in stratum
orients, stratum radiatum and stratum moleculare of hippocampus.
The revealed reaction may suggest the mysterious
functional role of micriglia in the current of PKU: or microglia
may play definite role in repairing of abnormal brain or
its reaction is the response to pathological condition.
FP-K-061
Increase of disaturated phosphatidylcholine in brains of
Pex5 knockout mice, a mouse model of Zellweger
syndrome
M. Nagura a , M. Baes b , M. Saito a , I. Kimura a , H. Hirose a ,M.
Kubota a , Y. Sakakihara a
a Department of Pediatrics, Faculty of Medicine, the University
of Tokyo, Tokyo, Japan; b Laboratory of Clinical Chemistry,
Katholieke Universiteit Leuven, Belgium
Zellweger syndrome is a familial lethal disease presenting
neuronal migration arrest in cerebral cortex and
dysmorphism in liver and kidney. Its cause was proved to
be the failure of peroxisome biogenesis, but the relation
between disturbance of neural differentiation and peroxisomal
dysfunction still remains unknown. Previously we
reported that fibroblasts of patients with mutation of Pex2
gene, one of the responsible genes for peroxysomal
biosynthesis, contain significantly higher level of glycolipids.
In this report, we aim to demonstrate changes in lipid
compositions such as the increase of glycolipids in brains of
Pex5 knockout mice. The result showed that the amount of
disaturated phosphatidylcholine (disat-PC) was five-times
higher in the brains of knockout mice than those in the
control. The amount of glycosphingolipid was scarce both
in the brains of knockout and control mice. The amounts of
other types of lipids such as cholesterol, sphingomyelin and
total glycerophospholipids did not differ between knockout
and control mice. Disat-PC is involved in pulmonary surfactant
and contributes to the surface tension of alveolus. The
role of disat-PC in the CNS has not been reported, but
increase of disat-PC may be related to peroxisome function
and derangement of neuronal development.
FP-K-062
MECP2 mutation analysis in Rett syndrome
M. Li a , V. Wong b
a Department of pediatrics, First hospital, Peking University,
b Department of pediatrics, The University of Hong
Kong, China
MECP2 gene, encoding methyl-CpG binding protein 2
(MECP2), is the responsible gene for Rett syndrome. As
RTT is a clinical syndrome without any diagnostic biologic
markers, mutation analysis of MECP2 gene is the only diagnostic
golden standard. It has been obvious that the phenotypes
of Rett syndrome have shown a wide range of
variability. The aims of this project is to identify the
MECP2 mutation in RTT patients using direct sequencing
so as to explore the spectrum of phenotypes resulting from
MECP2 mutations. We identified three mutations of
MECP2 gene among the six RTT patients. The mutations
of P322A and R106W were found in two patients classified
as fruste type. A patient with R306C mutation had the classic
type of Rett syndrome. The patient with P322A mutation
had no deceleration of head growth. Our report is the first
that confirms the normal head growth can occur in RTT with
MECP2 mutation. We think that the head growth deceleration
should not be a necessary criterion for RTT, especially
in the milder form of RTT.
FP-K-063
Progressive cerebello-cerebral atrophy - a new
syndrome with microcephaly, mental retardation and
spastic quadriplegia
B. Ben-Zeev a , C. Hoffman b , D. Lev c , N. Watemberg c ,N.
Brand a , T. Lerman-Sagie c
a Pediatric Neurology Unit,
b Neuroradiology Unit, Sheba
Medical Center, Ramat-Gan, Metabolic-Neurogenetic
Clinic; c Pediatric Neurology Unit, Wolfson Medical Center
Holon, Sackler School of Medicine, Tel-Aviv University,
Israel
Progressive cerebellar atrophy in infancy can be seen in
several genetic and metabolic diseases. Occasionally, it is
accompanied by cerebral atrophy. Mental retardation is
commonly seen. Hypotonia is more common than spasticity
in these cases. We describe seven (five females, two males)
children from six families with similar clinical and radiological
features. Clinically, all suffer from profound mental
retardation, and progressive spastic quadriplegia with joint
contractures. Other common characteristics include: generalized
seizures (5) and irritability (5). The children are nondysmorphic.
Head circumference is normal at birth with
progressive microcephaly evident from the 1st year of life.
Radiologically, early magnetic resonance imaging can be
normal but repeat studies show progressive cerebellar atrophy
followed by cerebral atrophy involving both white and
gray matter. All families are non-consanguineous Sepharadi
Jews from Moroccan or Iraqi origin. An extensive metabolic
evaluation was normal, including: blood amino acids,
lactate, very long chain fatty acids, carnitine, lysosomal
enzymes, isoelectric focusing of transferrin, urine organic
acids and purines, CSF lactate, amino acids and biogenic
amines, and muscle skin and nerve biopsies. The clinical
and radiological presentation of this group of patients bears
the closest resemblance to pontocerebellar atrophy type 2.
However, this disorder can be ruled out because in our
group of patients the cerebellar changes are progressive
and are not recognized at birth, there is no pontine involve-

578
Abstracts
ment and clinically the patients develop severe spasticity
but no chorea. We suggest that this is a new syndrome
with profound mental retardation, spasticity and microcephaly.
FP-K-064
Abnormal movements in Leigh syndrome with
cytochrome c oxidase deficiency due to SURF 1
mutations
B. Echenne a , A. Roubertie a , F. Rivier a , V. Humbertclaude a ,
A. Dubot b , M.T. Zabot c , C. Godinot b
a Neuropédiatric Department C.H.U. Montpellier. France;
b U.M.R. 5534 C.N.R.S. Lyon. France; c Debrouse Hospital
Lyon, France
Two unrelated children with an up to now normal
psychomotor development presented abnormal movements
when they were respectively 14 and 16 months old. The
first patient had tremor on hands and arms, occurring in
clusters, lasting a few minutes, without triggering factor,
many times a day. The second patient also had hands and
arms tremor on awakening, occurring in clusters on brief
duration, associated with myoclonic jerks of high amplitude,
affecting arms and legs, triggered by noises,
emotions, or voluntary movements. These abnormal movements
lasted 6–8 months, and were during this period the
main clinical abnormal sign, associated with deafness and a
slight ataxia. Then, the neurological status of both children
worsened, with progressive mental deficiency, pyramidal
signs and motor regression. Death occurred at 7 years 10
months and 3 years of age, respectively. In both cases, a
marked complex IV deficiency was found on muscle
samples, with a SURF 1 gene mutation. These observations
are interesting for two reasons: (1) the early onset and long
lasting abnormal movements, which were during several
months the most striking clinical abnormalities; and (2)
biological aspects, concerning the physiopathology of
mitochondrial diseases.
FP-K-065
Van der Knaap syndrome
K.S. Rana
Army Command Hospital (Central Command), Lucknow –
2, India
Progressive neurological diseases of white matter origin
may be due to failure of myelination or progressive demyelination.
Clinical features of leucodystrophy are spastisity,
gait abnormalities, special sensory deficits. Leucodystrophies
with macrocephaly progress rapidly with early
death. Van der Knaap et al. reported such leucodystrophy
with slow progression. We present eight such children.
Material and method: Case records of patients diagnosed
as leucodystrophy over 10 years time. Patients compatible
with Van der Knaap descriptions were included in the study.
The comprehensive workup included history, neurological
examination, metabolic workup included; arterial blood gas,
aminoacidogram, Cerebrospinal fluid and serum lactate
pyruvate, assays for mucopolysaccharidosis/glycogen
storage diseases, arylsulfatase A. Electroencephalogram,
visual and auditory evoked responses, nerve conduction
studies and Magnetic Resonance imaging in all patients.
Results: Of the eight, four were females and four males,
aged between 2 and 20 years. Onset was within 1st year
of life. Head circumference ranged from 54 to 60 cm.
Motor symptoms progressed up to 5–6 years of age in all.
All children had seizures and were well under control. Six
children were attending normal school. All had increased
tone in all limbs, brisk reflexes. Neuroimaging revealed
diffused involvement of white matter with cysts mainly in
temporal region. Other investigations were non-contributory.
Discussions: Combination of megalencephaly with
leucodystrophy is seen in Canavan’s and Alexander’s
disease. Both have rapidly progressive course. Slow course,
macrocephaly, striking neuroimaging abnormalities is quite
unique in Van der Knaap syndrome into which our eight
patients qualify.
FP-K-066
Effectiveness of creatine monohydrate in mitochondrial
encephalomyopathies
K. Komura a , E. Hobbiebrunken b , E. Wilichowski b ,F.
Hanefeld b
a Department of Pediatrics, Tokyo Women’s Medical
University, Tokyo, Japan;
b Abteilung Kinderheilkunde,
Schwerpunkt Neuropädiatrie Georg-August-Universität,
Göttingen, Germany
The mitochondrial encephalomyopathies are chronic
progressive disorders affecting predominantly the neuromuscular
system. Symptoms are induced by insufficient
energy supply resulting from a deficiency of oxidative phosphorylation.
We studied one male and four females with
genetically proven mitochondrial encephalomyopathies.
Their ages ranged from 7 to 19 years (two Kearns–Sayre
syndrome (KSS); one neuropathy, ataxia, and retinitis
pigmentosa, NARP; two MELAS), using retrospective
study method. We studied the effect of creatine supplementation
(0.08 g–0.35 mg/kg body weight/day; 9 months–4
years and 10 months) and measured skeletal muscle power
analysis (bicycle ergometer). After creatine supplementation
all patients showed an increase in their maximum
performance (W) (14– 1 30%; mean: 112.1%).These
results indicate an improved aerobic oxidative function of
mitochondria following creatine administration in the
patient with mitochondrial encephalomyopathies. Continuous
physical exercise was improved to a greater extent than
instantaneous activity.

Abstracts 579
FP-K-067
Early onset spastic paraplegia in purine nucleoside
phosphorylase deficiency
B.M. Tabarki, M. Yacoub, H. Selmi, A. Trabelsi, M. Dogui,
A.S. Essoussi
Pediatric department, Farhat Hached Hospital, Sousse,
Tunisia
Deficiency of purine nucleoside phosphorylase causes
recurrent infections, usually beginning in the 1st year of
life, and neurologic abnormalities. Neurologic findings
range from spasticity to developmental delay, to mental
retardation. We report two siblings with progressive spastic
paraplegia noted from the first few months of life. MRI of
the brain and spine were normal. EMG showed peripheral
neuropathy. Three years later, they developed T cell immunodeficency.
Enzyme study showed complete deficiency of
purine nucleoside phosphorylase. Spastic paraplegia at early
onset can be a prominent clinical feature of purine nucleoside
phosphorylase deficiency. Proposed mechanisms for
neurologic dysfunction in this disease are discussed.
FP-K-068
Mitochondrial encephalomyopathies: cases presentation
L.A. Selim
Street 86 Maadi, Cairo Egypt, Egypt
Mitochondrial disorders, once thought to be relatively
rare, are now thought to be the most prevalent metabolic
disease. They form a heterogeneous group of disease with
multi system presentation that affect mitochondrial ATP
production. The most severely affected organs in mitochondrial
disorders are those Depending on high rate of
aerobic metabolism, such as the brain, the skeletal muscles
and cardiac muscles, the sensory organs, and the kidneys.
Defects in mitochondrial metabolism result in a wide variety
of human disorders, which can present at any time from
infancy to late adulthood and involve any tissue either
alone or in combination. Neurologic presentations of mitochondrial
disorders are protean, they may be highly
suggestive of a specific syndrome such as: MELAS,
NARP, MERRF, Lebers hereditary optic neuropathy, and
more commonly may be non specific as intractable
seizures, global developmental delay and severe hypotonia.
The clinical presentation of five cases diagnosed as mitochondrial
myopathy and encephalomyopathy will be
discussed.
FP-K-069
Electromyographic, clinical and genetic features of
Prader-Willi syndrome in infants and children
L.G. Khachatrian, O.I. Masva, V.M. Studenikin
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific Center of China Health (Russian Academy
of Medical Sciences), Moscow, Russia
Background: Prader-Willi syndrome is usually presented
by such features as muscular hypomentia, hypogonadism
and obesity. Electroneuromyographic (ENMG) investigation
is an essential method in differential diagnostics
between this pathological condition and various diseases
accompanied with hypotonia. Aim: Complex diagnostics
of pediatric patients with Prader-Willi syndrome and
evaluation of ENMG in establishment of this diagnosis.
Method: Case histories of 14 infants and children (aged 6
months–5 years) with Prader-Willi syndrome were
analyzed, ENMG, CT, and MRT can of brain and genetic
analysis were incorporated. Results: Muscular hypotonia
was presented in all cases, motor milestones were delayed
(walking achieved after 26 months of age). In 79% of
patients combination of micropenia, cryptorchidism and
hypoplastic labia. Gross obesity developed after children
learned to walk. Mental retardation was evident (IQ
between 40 and 80). CY and MRT scans were performed,
revealing ventriculomegaly (64% of patients), frontal and
temporal lobes atrophy (14% of cases). Chromosome 15
anomalies have been registered in 71% of pediatric patients:
chromosome 15/15 transformation (57%), deletion in chromosome
15 (14%). In 29% of cases the karyotype was
normal. ENMG disorders were discovered in all patients,
and included increased M-reply amplitude, H-reflex, F-
wave (30%) and raised motor-neural conduction (40%).
Conclusion: In spite of clinical symptoms characterizing
Prader-Willi syndrome, ENMG plays an important role in
verification of this diagnosis, especially when dealing with
chromosome-negative versions of Prader-Willi syndrome.
FP-K-070
Tuberous sclerosis in children: a clinical and
neuropathological study
J. Qin a , J.-M. Wang b , M. Itoh c , X.-H. Bao a , Y.-H. Zhang a ,S.
Takashima c
a Department of Pediatrics, Peking University First Hospital,
Beijing, China; b Department of Pediatrics, Shangqiu
First People’s Hospital, Shangqiu, Henan, China; c Department
of Mental Retardation and Birth Defect Research,
National Institute of Neuroscience, NCNP, Kodaira,
Tokyo, Japan
Tuberous sclerosis complex (TSC) is a rare hereditary
multiorgan disease. The present study is to summarize the
clinical and neuropathological features of TSC. The clinical
data from 25 children, aged 6 months–13 years, and the
postmortem brain samples from four cases with TSC were
investigated retrospectively. The main clinical findings in
25 TSC children included seizures (23), mental retardation
(16), skin lesions (25, with hypomelanotic macules in 23
cases, angiofibromas in 15, shagreen patch in five, and fore-

580
Abstracts
head plaque in two), cardiac rhabodamyoma (three) or
rhabodamyosarcoma (one), and multiple renal cysts (four).
In two cases positive familial history of TSC was demonstrated.
The mother of one case had severe seizures and
mental retardation with typical dermatological TSC
features, whereas the father of another case manifested
only as typical dermatological TSC features and intracranial
calcifications with no neurological symptoms. Cranial CT/
MRI scan showed calcifications (22) in subependymal or
other area, and cortical or subcortical tubers (nine). Neuropathological
findings in all the four cases were cortical or
subcortical tubers, subependymal giant cell astrocytomas,
and heterotopic nodules in cerebrum or in cerebellum.
Histologically, these hamartomas contain abnormal giant
cells that show evidence of abnormal differentiation of
immature neural cells. (*This work was partly supported
by a key clinical project, 2001-03, from the Ministry of
Public Health of China).
FP-K-071
Myopathy with Allgrove syndrome
S.H. Ibrahim a , J. Vajsar b , V. Jay b
a Aga Khan University, Karachi, Pakistan; b The Hospital for
Sick Children, Toronto, Canada
‘3A’ Syndrome or Allgrove syndrome was first described
by Allgrove et al. in 1978. This syndrome typically consists
of adrenal insufficiency, achalasia and alachrima. Since its
first description more than 100 cases have been described.
We report a 9 years old boy with the typical manifestations
of achalasia, adrenal insufficiency, alachrima, and neurological
manifestations of generalized muscle weakness, developmental
and cognitive delay, who underwent muscle
biopsy, which showed evidence of myopathy. Although
various neurological manifestations including neuropathy,
ataxia, impaired intelligence have been described, as yet no
report has been made of myopathy in children with Allgrove
syndrome. This is the first report of association of Allgrove
syndrome with myopathy.
FP-K-072
Megalencepahlic leukodystrohy in children in a distinct
aslan ethnic group – the agarwlas in India
U.P. Bhat
Department Of Neurosciences, Apollo Hospital Chennal,
India
We are presenting six cases of megalencephalic leukodystrophy
who presented with a very large head in early
infancy from a distinct ethnic community of Agrawals –
head circumference was above the 95th centile in all with
almost normal range of functioning. History of seizures
was present in two cases and minimal neurological deficit
in the form of pyramidal and cerebellar signs was documented
in one case. Neuromaging studies revealed
evidence of severe bilateral white matter changes in the
cerebrum, with characteristic cystic changes in the
temporal lobes. Screening for known metabolic and degenerative
disorders was negative. The striking feature in these
was the extensive white matter changes in the brain as
contrasted with almost near normal intellectual functioning
and minimal neurological deficit.
FP-K-073
Novel TSC2 mutations in Japanese patients with
tuberous sclerosis complex
J.-H. Feng, T. Yamamoto, E. Nanba, H. Ninomiya
Gene Research Center, Tottori University, Yonago, Japan
TSC is an autosomal dominant disorder characterized by
hamartomas in many organs. Here we report mutation
analysis of TSC2 in ten unrelated patients, using singlestrand
conformational polymorphism analysis of all coding
exons. We detected six novel mutations in addition to three
mutations that had been previously reported, including two
frameshifts, one in-frame deletion, two nonsense point
mutations, three missense mutations and one splice site
mutation. We also found five new polymorphisms. Mutations
found in this study were distributed on various exons
and there was no clustering of the mutations. In accordance
with the previous findings, there was no obvious relationship
between the location of the mutation and the clinical
symptoms.
FP-K-074
Brain MRI findings in PKU patients
E.S. Suh a , Y.H. Kim a , D.W. Lee a , H.S. Hong b
a Department of Pediatrics, b Radiology, College of Medicine,
Soon Chun Hyang University, Seoul, Korea
Purpose: Abnormalities of MRI of the brain occur in
some patients with phenylketonuria (PKU). The purpose
of this study was to evaluate relation between MR findings,
age and serum phenylalanine level. Methods: We investigated
16 patients with biochemically documented PKU
who also underwent MRI. Typical classic form was in 13
patients, atypical in other two patients and malignant
hyperphenylalaninemia in the other two patients. We evaluated
signal intensity, the distribution of abnormal signal
intensity, and the extent of the brain atrophy in MRI, and
possible clinical correlation between age and serum phenylalanine
level and abnormal signal intensity. Results: MRI
scans revealed areas of abnormally increased signal intensity
on T2-weighted images in ten (62%) patients, preferably
involving the parieto-occipital lobes. In one advanced
case, the high signal intensity of both the parietal and
frontal lobes was seen on T2-weighted images, and brain
atrophy and gyriform enhancement on contrast enhanced

Abstracts 581
T1-weighted images. In six (38%) patients, the findings
were normal. No abnormalities were found in the basal
ganglia, brain stem and cerebellum. Nine patients were
under age of 5 years old. There were no remarkable difference
in the average serum phenylalanine levels for the
various groups having different degree of MRI abnormalities
(normal, mild, moderate, and severe – 26.4, 27.6, 28.2
and 20.8 mg/dl, respectively). Conclusion: Although MR
findings were not specific, PKU patients showed symmetrical
high signal intensity, predominantly in the peritrigonal
region. In the advanced case, on T2-weighted images,
high signal intensity extended to the periventricular and
subcortical white matter. There was no correlation between
age, serum phenylalanine level and severity of high signal
intensity.
FP-K-075
Influence of MECP2 gene mutation type and X
chromosome inactivation on the phenotype of Rett
syndrome
J. Chae, H. Hwang, K.-J. Kim, Y.-S. Hwang
Department of Pediatrics, Seoul National University Children’s
Hospital, 28 Yongondong, Jongnogu, Seoul 110-744,
Korea
Rett syndrome is a progressive neurodevelopmental
disorder occurring predominantly in females. Recently,
Amir et al. identified mutations in the MECP2 gene on
Xq28, which encodes methyl-CpG binding protein 2, as
responsible for some cases of Rett syndrome. In this
study, we analyzed the entire coding sequence of the
MECP2 gene and their X chromosome inactivation pattern
in 42 sporadic cases of Rett syndrome. Of the 42 patients, 30
(71.4%) had pathogenic mutations, which included 14
different mutations. Altogether, there were nine missense
mutations (D97Y, L100V, R133C, S134P, P152R,
T158M, A279V, R306C, P322L), four nonsense mutations
(R168X, R255X, R270X, R294X), and one frameshift
mutation (a 41-bp deletion at 1157–1197). Three of these
were novel mutations (D97Y, L100V, S134P). Most of the
nucleotide substitutions involved C to T transitions at CpG
hotspots. There was a tendency for patients having nonsense
mutations in TRD region to show earlier onset of regression
and severer language retardation than patients having mutations
in MBD region. However, the clinical severities were
variable among patients with same type of mutation,
depending on the pattern of X chromosome inactivation.
This study suggests that the X chromosome inactivation
pattern can modify the phenotype of Rett syndrome that is
initially determined by type and site of MECP2 gene mutation.
FP-K-076
Preliminary study on linkage mapping of a Chinese
benign familial infantile convulsions pedigree
B. Xiao, X.-S. Yang, J.-C. Ning, Z.-G. Wu, G. Xiong
Department of Neurology, Xiangya Hospital, Central South
University, Changsha, 410078, China
Objective: Genetic linkage analysis were performed to
map the disease gene locus of a Chinese benign familial
infantile convulsions pedigree on chromosome 19q12–
13.1 or 2q24. Methods: Seven polymorphic microsatellite
DNA markers (five markers on 19q, and two on 2q) have
been typed, and parametric linkage analysis has been
performed to analyze the segregation of the gene locus
within our BFIC family. Results: There was not a differential
haplotype that co-segregated with BFIC in this family.
When the recombinant rate u was zero, the linkage between
the investigated family and the mapped genes of BFIC was
excluded because the two-point LOD scores were less than
22. When the LOD scores were in the range of 22 and 0,
there was no evidence of linkage as well. So the analysis
showed no evidence of linkage between chromosome
19q12–13.1 or 2q24 and the BFIC phenotype. Conclusions:
There was no evidence that the BFIC gene of the investigated
family was mapped on chromosome 19q12–13.1 or
2q24. We suggest that BFIC shows genetic heterogeneity
and maybe another gene locus is responsible for the Chinese
BFIC family.
FP-L
Metabolism
FP-L-001
A possible new disorder of catecholamine metabolism; a
patient with recurrent serotonin syndrome like-episode
T. Ohtsuka, A. Kobayashi, M. Ito, H. Kakinuma
Department of Pediatrics, Kanazawa Medical University,
Uchinada, Japan
Serotonin syndrome is characterized by various combinations
of myoclonus, rigidity, hyperreflexia, confusion
and diaphoresis, caused by the use of serotomimetic agents.
Rhabdomyolysis and death are rare effects. We describe a
7-year-old girl with a 5-year history of recurring serotonin
syndrome-like episodes. Her elder brother died of renal
failure caused by rhabdomyolysis, which was due to overwhelming
involuntary movement persisting several days at
age 5 years and were similar to those in the patient
presented here. No relatives in the family showed any
movement abnormalities. This suggests an autosomal
recessive hereditary disease. She was born after an
uneventful pregnancy and delivery with an Apgar score
of 9. At age 2 years and half she presented with trunk
hypotonia and involuntary dystonic movements. After a

582
Abstracts
Table 4
Changes in ALT/AST during antiepileptic treatment a
Pretreatment
During treatment 2.6 ^ 1 year
AST ALT AST ALT
VPA (N:124) 25.06 ^ 3.12 16.36 ^ 7.06* 30.01 ^ 2.22 4.2 ^ 2.6* / **
CB2 (N:101) 26.08 ^ 6.5 16.28 ^ 8.12 28.01 ^ 7.1 18.32 ^ 6.88**
VGB (N:5) 28.07 ^ 9.3 17.16 ^ 7.45 29.11 ^ 6.4 3.0 ^ 1.31
VPA 1 CB2 (N:31) 31.04 ^ 2.0 18.13 ^ 5.56 33.61 ^ 3.1 16.26 ^ 3.26
VPA 1 LTG (N:24) 29.9 ^ 1.43 12.86 ^ 7.43 32.18 ^ 3.4 17.01 ^ 4.0
VPA 1 VGB (N:4) 33.18 ^ 6.1 18.20 ^ 6.2 34.25 ^ 7.1 3.8 ^ 1.76
Total 289
a
*P , 0.05; and **P , 0.05 (the difference between VPA and CBZ).
viral illness, her movement worsened rapidly and eventually
she became unable to control herself. She also
showed agitation, restlessness, and diaphoresis. This attack
persisted over a month and repeated itself every 2 or 3
months. Normal laboratory findings included plasma
amino acids, organic acids, serum lactic, uric acids, copper
and ceruloplasmin. Five-hydroxyindole acetic acid, homovanillic
acid, neopterin, teterahydroxybiopterin, and 5-
methyltetrahydroforate in the cerebrospinal fluid were
normal. Brain MRI showed mildly enlarged ventricles.
She has slowly developed motor skills, sitting at age 2
years, crawling at 4 years, and standing with support at 5
years. At present her language comprehension is at the 3–4
year-old level. She was treated with antiepleptic drugs and
muscle relaxants, but frequency of the attacks did not show
any change. We propose that this patient has a defect in the
CNS catecholamine metabolism.
FP-L-002
Alanine aminotransferase levels during chronic use of
anticonvulsant drugs
D. Içaǧasioǧlu, Ö. Ünal, G. Deda
Department of Pediatric Neurology, Ankara University
Medical School, Ankara, Turkey
We aimed to determine the effect of antieplectic drugs on
the serum alanine amino transferase levels in 289 epileptic
children. This study, before and after 2.6 ^ 1.06 years,
compared ALT and gamma-glutamly transferase activities
in sera of children who were under either VPA (42.9%),
CBZ (34.9%) or vigabatrin (VGB) (1.7%) monotherapy,
or VPA 1 CBZ (10.7%), VPA 1 LTG (8.3%), and VPA 1
VGB (%1,41) combined therapy (Table 4). Previous studies
have reported reductions in plasma ALT activity due to
VGB and it is not known whether this can mask early elevations
in transaminases in patients with hepatocellular
damage. Our data indicate that in 28% of patients serum
ALT levels reduced during VPA medication and careful
monitoring of hepatic function is mandatory in patients
under VPA therapy.
FP-L-003
The effect of valproic acid on bone mineral metabolism
and bone density
D. Içaǧasioǧlu, G. Deda, Ö. Ünal, M. Berberoǧlu
Departments of Pediatric Neurology and Endocrinology,
Ankara University Medical School, Ankara, Turkey
Many reports of altered calcium, vitamin D and bone
metabolism associated with anticonvulsant therapy have
been published. The purpose of this study was to determine
whether bone mineral density is decreased by VPA treatment
in epileptic children. We studied 18 children with new
onset of epilepsy. Their ages ranged between 9 and 12 years.
Children who had mental and motor retardation, had used
medication known to alter bone metabolism, or had had
neurologic deficit and abnormal cerebral imaging findings
were excluded from the study. Before and after 11 ^ 2.1
months of VPA therapy we measured biochemical parameters
of bone mineral metabolism and dual energy X-ray
absorptiometry was used to assess lumbar spine (L2–4)
bone mineral density (BMD). Comparison of the mean
biochemical parameters and BMD values of the before
and after VPA treatment are shown in Table 5. Our data
indicate that VPA therapy in epileptic children does not
Table 5
Laboratory parameters of patients a Before therapy
After therapy
Male/female 11/7 11/7
Height 30.8 ^ 0.7 31.9 ^ 2.3
Serum drug level (mg/ml) – 60.1 ^ 1.3
Ca (mg/dl) 9.32 ^ 0.1 8.27 ^ 0.5
P (mg/dl) 5.8 ^ 0.7 5.0 ^ 0.2
Alkalin phosphatase (IU/l) 189 ^ 85.40* 237 ^ 97.86*
PTH (pg/ml) 21.48 ^ 19.18 27.31 ^ 16.02
Calcitonin 11.16 ^ 8.91 10.2 ^ 9.18
Osteocalcin 12.66 ^ 7.44 14.12 ^ 6.98
BMD 0.558 ^ 0.084 0.506 ^ 0.046
a *P , 0.05.

Abstracts 583
seem to have an adverse effect on bone mineral metabolism
and BMD.
FP-L-004
Neonatal hypoglycemic brain injury – a specific clinical
and imaging syndrome in 51 children
V. Udani, M. Bawdekar, S. Karia, S. Mandasar
P.D. Hinduja National Hospital and Medical Research
Centre, Mumbai, India
Brain injury attributed to neonatal hypoglycemia
remains a significant problem in developing countries
and is responsible for multiple disabilities. We describe
51 children seen at a mean age of 39 months with a spectrum
of disabilities and characteristic imaging findings.
Forty-five/51 were males. Twenty-two were ,2.5 kg
(LBW) and 22 were FT-AGA though only eight were #
kg and above. A total of 79% had a neonatal encephalopathy
on D2-3; 84% had microcrania; 59% were visually
impaired; 57% had oromotor apraxia; and 75% had
delayed language. Cerebral palsy was uncommon and
was seen in only 10%. Apraxia of hand use was seen in
57%. Epilepsy was seen in 88% and was partial (55%) or
spasms (45%) or both. Spasms were refractory in 55% and
partial seizures in 35%. Outcome in 35 children .5 years
age revealed normal school performance in three, LD in
seven, mild MR in six, severe MR in 11 and frank autism
in eight. Cerebral palsy was noted in only five. Statistically
significant association was noted between presence of
epileptic spasms and autism, apraxia of hand use and
oromotor apraxia. Generalised seizures were more
common following spasms. Imaging revealed mostly bilateral
occipital lesions in 84%, parietal lesions in 35% while
only 7% had temporal lesions. No frontal lesions were
noted. Conclusions: Neonatal hypoglycemic brain injury
damages posterior regions with resultant visual deficits,
apraxia of hand use and oromotor skills, cognitive impairment
and autism, microcephaly, and often refractory
epilepsy. Tone alterations are relatively less common.
This preventable perinatal insult needs top priority in
developing countries.
FP-L-005
Acute intermittent porphyria: case presentation
L.A. Selim
street 86 Maadi, Cairo, Egypt
Acute intermittent porphyria is an autosomal dominant
disorder of heme biosynthesis. The basic biochemical
defect is reduced activity of the enzyme porphobilinogen
deaminase caused by a genetic defect the deaminase gene
located on the 11chromosome. Due to this defect only 50%
of the normal enzyme quantity is produced. The disease
becomes manifested only in the case of increased demands
on a given metabolic pathway resulting in porphobilinogen
accumulation and storage in the organs. Clinical evolution
is characterized by acute attacks of abdominal pain, neurologic
and psychiatric symptomatology, induced by drug
intake, infection, alcohol intake or unknown factors.
Laboratory diagnosis is based on the detection of deltaaminolevulinic
acid, porphobilinogen, uroporphyrin and
coproporphyrin in the urine. The therapy is based on infusions
of 20% glucose solution and hydromineral imbalance
correction. When neurologic symptoms occur it is necessary
to administer hem-arginate intravenously. We report a
case of a female child suffering from the disease with
discussion of the treatment.
FP-M
Neuroimaging
FP-M-001
MRI study on brain in patients with tetrahydrobiopterin
(BH 4 )deficiency
Z.-X. Zhang, W.-M. Yu, Z.-S. Zhou, X.-Z. Zhang
Department of Pediatrics, China-Japan Friendship Hospital,
Beijing, China
Objective: To observe the brain abnormalities of the
white matter in BH 4 deficiency children in order to investigate
the effect on the brain myelination. Methods: Eight
patients with BH 4 deficiency were observed. In six cases,
the level of blood Phe was between 480 and 1500 mmol/l
on newborn screening. A low-Phe-diet was given early
within 1 month after birth. Although the blood Phe had
been controlled in an ideal range (120–240 mmol/l),
there were the progressive myodystonia, the abynamia,
and difficulty in raising their head in these patients.
Among them, four patients had convulsion and the other
two also had the oligophrenia accompanied by high blood
Phe (.1200 mmol/l). All eight patients were diagnosed as
BH 4 deficiency by using the analysis of the urine pterin,
BH 4 loading test and the analysis of dihydropteridine
reductase in RBC. The eight cases were subjected to
0.5T superconductive MRI examination before the treatment
with BH 4 and pro-neurotransmitter. Results: Delayed
myelination was detected in all cases, mainly in the frontal
lobe (eight cases, 100%), in the occipidfol lobe (five cases,
62.5%), and in the temporal lobe (four cases 50%). There
existed abnormal hyperintense foci in white matter shown
by T 2 WI in all cases. Conclusions: (1) Children with BH 4
deficiency have a high occurrence of brain abnormalities in
the white matter. (2) These abnormalities may be related
not only to HPA, but also to the decreased synthesis of
neurotransmitters, such as 5-HT and DA, which could
affect the development of the white matter.

584
Abstracts
FP-M-002
3-D MRI volume of frontal lobe in autistic children
Y. Toda, K. Mori, K. Tao, K. Inoue, M. Miyazaki, T. Saijo,
T. Hashimoto, Y. Kuroda
Department of Pediatrics, School of Medicine, University of
Tokushima, Tokushima, Japan
Purpose: We measured volume of the whole brain and
frontal lobe using 3-D MR image and coherence with laterality
and DQ examined volume of frontal lobe. Object/
method: Object is totaled 35 examples of 19 autistic children
(3–5 years old, DQ 50–100) and 16 healthy contrasts (3–5
years old) visiting our hospital. We got informed consent
from family with both group about MRI examination and
measured horizontal image with 3-D SPGR method. We did
reconstitution using image analysis software ‘Scion Image’
and got the volume of the whole brain and frontal lobe and
surveyed volume of right and left each about frontal lobe.
We used Enjyoujishiki developmental test about DQ.
Result: We did not recognize significant difference between
autism group and healthy control group about the volume of
the whole brain and frontal lobe. We compared even rate for
volume of the whole brain (%) about frontal lobe, but did
not still recognize significant difference. The right side was
big in significance compared with the left with autism group
in comparison of right and left frontal lobe volume. We did
not recognize correlation during brain and frontal lobe
volume about DQ. Conclusion: In volume of frontal lobe,
the right side is big in significance compared with the left
with autism group and coherence with frontal lobe dysfunction
was suggested.
FP-M-003
Clinical and magnetic resonance image analysis of 36
cases of periventricular leukomalacia in children
X.-D. Yuan
First People’s Hospital of Shangqiu City, Shangqiu, Henan,
China
Objective: To investigate the clinical and MRI characteristics
of PVL in children. Method: To analysis the clinical
data of PVL in 36 children. Result: (1) Gestational age:
between 30 , 37 weeks in 22 cases, 38 , 42 weeks in 14
cases. (2) Clinical manifestation: (a) paralysis in 36 cases;
(b) cortical blindness in two cases; (c) mental retardation in
nine cases; and (d) epilepsy in ten cases. (3) MRI manifestation:
(a) foci form: patchy areas in 16 cases, punctate areas
in 13 cases, streaks in seven cases; (b) section: symmetric
signal abnormalities were observed in the periventricular
and the subcortical white matter; (c) the signal of the
focus: hyperintense/intense on T1 weighted and hyperintense
on T2 weighted; and (d) other: bilateral ventricular
bodies dilatation in eight cases. Trigon dilatation in five
cases. Conclusion: PVL is a main cause of cerebral palsy,
especially of spastic paralysis, MRI is a main way to diagnose
PVL in early infants.
FP-M-004
Automated histogram-based brain segmentation and
volume measurement in T1-weighted 3-D MR head
images
J.Z. Liu, Z.Y. Shan, G.H. Yue
Department of Biomedical Engineering, Cleveland Clinic
Foundation, Cleveland, OH, USA
Information on human brain volume changes may help
better understand structural and functional development and
adaptations of the brain. Current semi-/automated MRbased
brain segmentation and volume measurement methods
are complex and not sufficiently accurate. We have
developed a simpler, more accurate automated algorithm
for whole-brain segmentation and volume measurement in
T 1 -weighted 3-D MR images. This histogram-based brain
segmentation (HBRS) algorithm is based on histogram
analysis and simple morphological operations. It includes
three major steps: (1) foreground/background thresholding;
(2) disconnection of brain from skull and other head tissues;
and (3) removal of residual fragments (cerebrospinal fluid,
sinus, dura and marrow). Brain volume was measured by
counting the number of brain voxels. Accuracy was determined
by comparing brain volume rendered by applying the
algorithm to computer-simulated MR data with the volume
on which the simulation was based; average error was
1.48%. Reproducibility of brain volume measurements
was assessed by comparing data from two sessions (two
trials in each session) with human volunteers. Intra-session
variability of brain volumes for sessions 1 and 2 was
0.55 ^ 0.56 and 0.74 ^ 0.56%, respectively; mean difference
between the two sessions was 0.60 ^ 0.46%. These
results show that the HBRS algorithm is a simple, fast,
and accurate method for human brain volume assessment
with high reproducibility. This algorithm may find various
applications in research and clinical investigations regarding
cortical development and child neurology.
FP-M-005
Diffusion weighted MRI for early diagnosis of acute
neurological disease in the neonate
W. Brussel, I.M. van Beynum, M.C. Molenschot, V.A. Bok
Rijnstate Hospital, TA Arnhem, Netherlands
Diffusion weighted-MRI (DW-MRI) uses water movement
and transport of water in biological tissues as a source
of contrast. Therefore DW-MRI can be used to obtain information
about molecular displacement over distances
comparable to cell’s dimensions and, consequently, about
geometry and spatial organization of tissue compartments.
Functional insight concerning exchanges between these
compartments in various disease states can be gathered. In
the presence of magnetic field gradients, protons carried by
free moving water molecules, undergo a phase shift of their

Abstracts 585
transverse magnetization. As diffusion is characterized by
the random Brownian displacements of molecules, these
phase shifts are widely dispersed. Interfering with each
other finally results in attenuation of the MRI signal. The
attenuation depends on the amplitude of molecular displacement
and intensity of the magnetic field gradient. In
conventional MRI diffusion effects are extremely small
and invisible. By using strong magnetic field, gradient diffusion
becomes visible and measurable. Especially in intracellular
cytotoxic edema diffusion is strongly limited and
the involved areas will show with high signal on the images.
DW-MRI can detect pathological changes within minutes of
the onset of injury, which is well before conventional MRI
shows changes. Although recent publications reveal the
importance of DW-MRI for early diagnosis of hypoxic
ischemic injury and focal infarction in the neonatal brain,
the information on clinical diagnostic significance in other
neonatal neurological disease is sparse. From examples of
several acute neurological diseases in the neonate, such as
bacterial and viral meningoencephalitis, hypoglycemia and
cerebrovascular accident, the diagnostic value of DW-MRI
in an early stage of these diseases is shown.
FP-M-006
Predictive value of neonatal MRI with respect to late
MRI findings and clinical outcome-a study in infants
with periventricular densities on neonatal ultrasound
L.T.L. Sie, M.S. van der Knaap, A.A.M Hart, J. van Hof, L.
de Groot, W. Lems, H.N. Lafeber, J. Valk
Vrije Universiteit Medical Center, Amsterdam, The Netherlands
Objectives: To correlate hypoxic-ischemic white matter
damage on neonatal MRI with late MRI and neurologic
outcome at 1.5 years. Methods: MR images of 46 infants
(mean gestational age of 31 weeks) with periventricular
densities on ultrasound were obtained at a mean age of 20
days after birth and at 1.5 years. To establish agreement
between the neonatal and follow-up MRI (general, motor
and visual scores), the weighted Cohen’s kappa was used.
Predictive values of neonatal MRI with respect to the neurologic
indices at 1.5 years were calculated. Results: There
was a moderately good to good agreement between the
general, motor and visual neonatal and follow-up MRI
scores: weighted kappa ¼ 0.59 (95% CI: 0.44–0.74), 0.82
(95% CI: 0.72–0.93), and 0.70 (95% CI: 0.56–0.84), respectively.
Neonatal MRI scores provided a good prediction of
the three neurologic outcome measures (developmental
delay, cerebral palsy and cerebral visual impairment): sensitivity,
specificity and predictive values were high, with little
difference between the three MRI scores. The 32 patients
with (nearly) normal neonatal MRI scores were neurologically
(nearly) normal at 1.5 years on all three outcome
measures, whereas eight patients with seriously abnormal
neonatal MRI scores were neurologically abnormal at 1.5
years on all three outcomes measures. Conclusions: Neonatal
MRI is able to predict the precise localization and size of
perinatal leukomalacia on follow-up MRI and provides a
good prediction of neurologic outcome at 1.5 years.
FP-M-007
Ultrasonographic assessment of the fetal frontal lobe
Y.-L. Zhang, Y. Wu, Z.-R. Zhang
Department of Pediatrics, Daqing Medical College,
Daqing, China
Objective: To study the characters of normal growth of
the fetal frontal lobe, and to obtain a nomogram of frontal
lobe measurements in fetuses with different gestational
weeks. Methods: A prospective ultrasound evaluation was
conducted in 338 normal pregnant women with gestational
ages ranging from 15 , 40 weeks. Several biometric
measurements were obtained throughout pregnancy, including
the frontal lobe distance (FLD), the thalamic frontal lobe
distance (TFLD), the biparietal diameter (BPD). A nomogram
of frontal lobe measurements from different gestational
age (GA) and BPD were generated and included the
x ^ s. The linear analysis and the parabolical curve fitting
analysis were made between FLD, TFLD and the GA, the
BPD. Results: The analysis of these data revealed a high
degree of linear correlations between FLD, TFLD and the
GA, the BPD (R. between 0.9562 , 0.9708 P , 0:0001).
The second order polynomes for these two measurements
versus GA and BPD were produced with the parabolical
curve fitting analysis (P , 0:0001), and the growth curves
were plotted by computer. FLD was almost linearly
increased with the gestational age. TFLD was increased
curvely with the gestational age. Conclusion: The results
of this study demonstrated the pattern of normal growth of
the frontal lobe. They offer a method by which early prenatal
diagnosis of developmental anomalies of the fetal brain
(especially microcephaly) can be made.
FP-M-008
Evaluation of 3D-TOF MRA in children ischemic
strokes
X.-L. Yu, Y.-Q. Zhang, L.-M. Ye
Department of Neurology, Tianjin Children Hospital, Tianjin,
China
Objective: To evaluate the applying of 3D-TOF Magnetic
resonance angiography (MRA) in children ischemic strokes.
Methods: Between December 1999 and June 2001, 15
patients (eight males, seven females) were studied. Their
ages ranged from 11 month to 12 years old (mean 3
years). With diagnosis of MRI, ECLIPSE 1.5-T of Marconi
Company, all patients do the examination within 1 week.
All ischemic foci were found on one side (nine left, six
right). Frontal lobe one case, parietal lobe one case, multi-

586
Abstracts
focus eight cases, basal ganglia lacuna infarction five cases.
Result: MRA (2) four cases, single/accompany implicated
cerebral media artery (MCA) eight cases, single implicated
cerebral anterior artery one case and basilar artery two
cases. The initial position of artery and the first branch
stricture/infarction four cases, periphery artery tenuous/
branch decreasing five cases, basilar artery tortuous two
cases. Through therapy, the beginning time of recovery of
muscle strength are 4 , 7 days to MRA (2) and artery
stricture/infarction patients, 7 , 18 days to periphery artery
tenuous/branch decreasing patients, 3 , 10 days to basilar
artery tortuous patients. Conclusion: Most of the ischemic
strokes patients have the corresponding artery changes in
MRA, the majority changed position is MCA system, the
variability was related with prognosis. The patients with
polyartery implicated need followed up for a long time, in
order to exclude moyamoya, polyarteritis, etc.
FP-M-009
Potential epileptogenecity of calcified neurocysticercosis
V. Kalra a , K.S. Rana a , S. Gulati a , B. Ekka a , A.K. Gupta b ,
R.M. Pandey c
a Departments of Pediatrics, b Radiodiagnosis, c Biostatistics,
All India Institute of Medical Sciences, New Delhi, India
Background: Epileptogenecity of calcified neurocysticercosis
being unknown, the AED duration remains a dilemma.
Objectives: To study calcification in neurocysticercosis as a
risk factor for epilepsy. Materials and methods: Retrospective
analysis of 296 patients with CT imaging compatible
with neurocysticercosis- single/multiple parenchymal or
calcified lesions after exclusion of tuberculosis. Follow up
period was 3–6 years for seizure recurrences: breakthroughseizures
recurring on adequate AED and relapse-recurrence
of $2 seizures 3 months after AED discontinuation.
Results: A total of 296 children, mean age 7.9 ^ 2.8 years
of either sex. The initial CT showed calcification in 18.5%.
AED were administered for 1.9 ^ 1.4 years. Albendazole
was given to 33/296 and follow up scans in albendazole
treated group revealed calcification in 52%. On follow up
CT scans (170), 6/31 with normal scans had breakthrough
seizures and similar figure for persisting lesions was 8/50
and calcified 33/89 (P-0:04). Similar figures for relapse
were 1/31 with normal CT scan, 3/50 persisting lesions
and 12/89 calcified (P-0:39). The follow up duration after
AED discontinuation was 2.2 ^ 1.8 years. A total of 130
children had calcification in the initial and/or follow-up CT
scans. Breakthrough seizures were seen in 50/130 (38.4%)
children with calcified lesions versus 32/166 (19.2%) in the
rest (P , 0:001). Similar figures for relapse were 16/130
(12.3%) and 10/166 (6.0%), respectively (P ¼ 0:09).
Conclusions: Calcification in neurocysticercosis, though
an inactive healed lesion, is associated with increased risk
of seizures as compared to persisting lesions or normal scan
on long term follow-up.
FP-M-010
Face perception of Asperger syndrome child
I. Kimura, M. Kubota, H. Hirose, M. Yumoto, Y.
Sakakihara
Department of Pediatrics, Department of Laboratory Medicine,
The University of Tokyo, Tokyo Japan
Asperger syndrome (AS) is one group of the pervasive
developmental disorders and accepted as autistic spectrum
entity. Although they have almost normal or superior intelligence,
no significant speech delay, AS adults and children
have problem in social communication skills and are
supposed to have specific deficit in understanding others’
mind. Considerable number of studies has shown that AS
or high-function autism adults are inferior to IQ-matched
controls in understanding emotions from others’ facial
expression, or even if they could detect rightly, some
studies revealed that they might use different pathway in
the brain. As it has not been clearly understood the way AS
child percepts face, we examined an 8-year-old AS girl by
magnetoencephalography (MEG) with the task of selecting
‘laughing faces’ among a series of projected face photographs.
The task was also done with inverted faces. It is
known that basal occipitotemporal cortex is mainly participated
in face processing, especially at early stage, within
latency of about 200 , 300 ms, and all of the controls
except one showed such pattern of MEG activity waveforms
and dipole locations. But the AS girl showed distinct
pattern from this, with larger activation of bilateral parietal
than occipitotemporal cortex. This distinct pattern was not
observed when the faces were inverted. It was suggested
that AS child might use special dorsal way of parietal
cortex in face processing.
FP-M-011
Diagnostic value of magnetic resonance imaging in West
syndrome
F. Fang
Beijing Children Hospital Beijing, China
Objective: To study the value of MRI in West syndrome
(WS) diagnosis. Methods: MRI results of thirty-one patients
with WS were reviewed retrospectively. Results: Twentyfive
patients were diagnosed symptomatic WS, three
patients were cryptogenic and three patients were primary.
Thirty-one patients were classified into four groups on the
basis of their MRI findings: Periventricular leukomalacia,
myelination delay, the other groups and normal. Conclusion:
MRI was helpful in finding etiology and assessing
prognosis in WS.

Abstracts 587
FP-M-012
Using MRI analysis the relationship between the
disorder in basal ganglia in children and the reason and
symptom of it
T.-L. Han
Beijing Children Hospital Beijing, China
Objective: To study the relationship between the causes,
symptoms and the disorders of basal ganglia by MRI in
children. Method: Forty-one patients were studied by MRI,
using CSF, EEG, lactic acid, etc., as the combined diagnostic
method. Results: Forty-one cases all had disorders
on basal ganglia. The number of cerebrovascular disease
was 12 (29.3%), and it was the leading cause, it included
eight cases of idiopathic cerebral vasculitis, and four cases
of moyamoya disease. The number of the immunologic
disorders on nervous system (NS) was eight (19.5%), it
included four cases of acute disseminated encephalomyelitis
(ADEM), two cases of Acute hemorrhagenic leukencephalitis,
a case of multiple selerosis (MS) and a case of
systemic lupus erythematosis (SLE). The number of metabolic
diseases and heredodegenerative diseases of the NS
was 10 (24.4%). The number of other causes or causes of
unknown was 11. Conclusion: (1) Acquired disease was the
leading cause of the disorders of basal ganglia in childhood.
(2) Clinical manifestation of disorders of basal ganglia
was widespread, and was not limited to dystonia and
involuntary movements, and also was not limited to the
nervous system.
FP-M-013
CT analysis of mental retardation children caused by
perinatal cerebral lesion
Z.-H. Song
Woman and Infant Healthcare Hospital, Yangquan City,
Shanxi, China Purpose: Research of the pathological
changes of mental retardation children resulting from perinatal
cerebral lesion and exploration of the early remedial
ways. Way: Selecting 144 patient pupils equal to or under 7
years old without ablepsia and deafness, who have the
history of perinatal cerebral lesion without inherited metabolic
diseases and mental test IQ/DQ is under 69 with head
CT scan. Result: CT abnormal rate is 71.52% and with
significant difference between severe and moderate mental
retardation children. CT showed mainly the atrophy and
hypogenesis, mostly in the frontal lobe. Conclusion: (1)
Perinatal brain injury may be one of the factors to cause
future mental retardation (MR). (2) Head CT scan of the
children with MR showed mainly atrophy and hypogenesis,
mostly in the frontal lobe.
FP-M-014
Abnormal cerebral glucose metabolism in patients with
alternating hemiplegia of childhood
M. Sasaki a , A. Fukushima a , H. Sakuma a , N. Shiroma a , K.-I.
Yamada a , T. Ohnishi b , H. Matsuda b , N. Sakuragawa c
a Department of Child Neurology,
b Radiology, National
Center Hospital for Mental, Nervous and Muscular Disorders,
National Center of Neurology and Psychiatry (NCNP),
c Department of Inherited Metabolic Disease, National Institute
of Neuroscience, NCNP, Kodaira, Japan
Alternating hemiplegia of childhood (AHC) is a rare and
intractable disorder, the cause of which remains unknown.
To determine the extent and degree of neuronal damage, the
brain glucose metabolism in three children and two adult
patients with AHC was studied by PET using 2-deoxy-[18F]
FDG. FDG-PET was performed between hemiplegia attacks
in all patients. All the patients met the criteria of Aicardi.
Hemiplegic attacks occur 2–8 times a month in all patients.
The child patients consisted of 1–3-year-old boys. They all
showed psychomotor retardation and hypotonia. The adult
patients consisted of 22 and 30-year-old women, whose
brain MRI revealed mild cerebellar atrophy. Interictal
FDG-PET revealed abnormal cerebral glucose metabolism
in all patients. Low glucose metabolism in the frontal lobes
with some laterality was demonstrated in all patients, and
low glucose metabolism in the ipsilateral putamen was seen
in three patients. The brainstem and cerebellum were
normal as to glucose metabolism except for an adult patient.
Areas of low glucose metabolism indicate local or regional
neuronal damage, and could be related to neurological
symptoms, for example, hypotonia, mental retardation,
involuntary movement, etc. The unknown cause of AHC
might induce focal abnormal glucose metabolism progressively
or permanently in the brain.
FP-M-015
Neuroimaging methods for diagnosis of behavior
deviations in the early life
A. Jaxybayeva
Institute of Medical Doctor’s improvement, Child Neurology
Department, Almaty, Kazakhstan
The purpose of our study was to investigate MRI-changes
of the brains structure. We analyzed 50 MRI-scans of children
with attention deficit hyperactivity disorder, aged 4–5
years. On the MRI-scans at all of that children were detected
a structure abnormalities as insignificant atrophy of the frontal
lobe, insignificant dilatation of the third ventricle of the
brain, decreased volume of the left caudate nucleus and
increase of the right caudate nucleus. Therefore a neurobehavioral
deviation as ADHD has been associated with thin
brain structure abnormalities as insignificant atrophy of the
frontal lobe, insignificant dilatation of the third ventricle of

588
Abstracts
the brain, asymmetry of basal ganglia (decrease volume of
the left nucleus caudate and increase of the right nucleus
caudate).
FP-M-016
Neuroradiological changes in infants with cysta cisterna
ambiens
K.S. Ormantaev, D.R. Kachurina, L.O. Saulebekova
Scientific Center of Pediarics and Children’s Surgery,
Almaty, Kazakhstan
The purpose is study the character of changes in infants
with some kinds of arachnoid cysts. During the period from
1999 till 2001 years 1000 infants, aged from 0 to 3 months
were examined at ultrasound diagnostic system ‘Aloka-
2000’ (the transducer with frequency 7.5 MHz) was used.
In seven children (0.7%) a small anechoic zone situated on
the midline behind and below the dorsal end of the choroid
plexus of the third ventricle, and above the vermis of the
cerebellum was found. Its diameter was 7–10 mm. These
changes were confirmed by computer tomography. Doppler
investigations of cerebral arteries have not found any
changes in cerebral blood flow. A total of 42.8% infants
had convulsions, and 57.2% infants had not got severe
neurological disturbances, their development was according
their age. Cysta cisterna ambiens is very rare pathology. It
has not specific neurological symptoms. It was found occasionally
only during neurosonography and computer tomography.
FP-M-017
The features of image examination in children with acute
hemiplegia
Y.-F. Lu, L.-Q. Chen, Y.-C. Zhang, Q. Lu
Shanghai children’s hospital, Shangha, China
Objective: To observe the image features of acute central
hemiplegia in children. Methods: Thirty-one cases with
acute hemiplegia were hospitalized in neurological department
of Shanghai children’s hospital from 1992 to 2001.
We analyze the image features of cranial CT, MRI or
MRA. Results: Thirty-one children were aged from 4
months to 11 years (median 3.6 years), with the course
from 6 h to 20 days. All cases were abruptly, 16 cases
with preceding viral upper respiratory infection, two
cases with similar history, two cases with convulsion,
and two cases with fever and coma. Nineteen cases were
abnormal in 23 cases by CT, and nine cases were abnormal
in 11 cases by MRI. Three cases were abnormal by MRI in
CT negative, and two cases were diagnosed with moyamoya
disease by MRA but MRI negative. Twelve cases
were shown with lateral basal ganglia embolus (38.71%);
three cases were represent with bilateral basal ganglia
embolus (9.68%); two cases were revealed with by nucleus
lentiformis embolus; three cases were found with lateral
temporo-parital lobes embolus. Two with lateral parietooccipital
lobes embolus, two with lateral cerebral peduncles
embolus, two with lateral internal capsula embolus,
two with subdural hemorrhage, and two with moyamoya
diseases. Conclusion: CT and MRI are the important diagnostic
methods in acute hemiplegia, and MRI is superior to
CT. It is necessary to do MRA when CT or MRI finding is
negative.
FP-M-018
Attention deficit hyperactivity disorder (ADHD): frontal
aMRI and motor inhibitory deficits
M.B. Denckla
Kennedy Krieger Institute, Baltimore, MD, USA
On volumetric MRI, children with ADHD showed
decreased volume in multiple frontal lobe regions.
Comparing 12 boys with ADHD with 12 age-matched
male controls using semi-automated Talairach-based
parcellation methods (Kaplan, 1997; Kates, 1999), total
cerebral volume was significantly smaller (8.3%) with
ADHD. Frontal lobe volumes both gray and white matter
accounted for most of this decrease; total non-frontal and
other lobar volumes were not significantly smaller. The
ratio of frontal lobe to non-frontal tissue volume in the
ADHD group was smaller than that of the control group
but only as a trend. Frontal lobes were subparcellated into
motor, premotor (includes supplementary motor area
(SMA), ventral premotor cortex (PMv), frontal eye field
(FEF), and supplementary eye field (SEF)), prefrontal
(includes dorsolateral-prefrontal (DLPF) and OF cortices,
amongst other regions), and anterior cingulate, and deep
white matter; reductions were observed in both prefrontal
and premotor functional modules (as well as deep white
matter). Forty-two children with ADHD were impaired on
tests of skeletomotor response inhibition compared with 30
age and gender-matched controls; most significant was the
conflicting motor response test. Physical and neurological
examination of soft signs (PANESS) in a slightly smaller
subsample of children (37 ADHD, 27 controls) revealed
significantly more overflow (including mirror movements)
in children with ADHD. Conflicting motor response test
scores significantly predicted overflow movements for all
children, both ADHD and controls implicating shared inhibitory
function. Oculomotor response inhibition was implicated
in ADHD; unmedicated children with ADHD made
significantly excessive directional errors on an antisaccade
task and anticipatory errors (premature eye movements) on
a memory-guided saccade task.

Abstracts 589
FP-M-019
The clinical significance of evaluation of glucose
metabolism of brain with FDG-PET brain image during
the phase and interphase of refractory epilepsy in
children
Q.-X. Zhai, X.-Y. Lin, X.-H. Zhou, Z.-X. Qiao, Y.-X.
Zhang, H.-X. Qiao
Department of Pediatrics, Guangdong Provincial People’s
Hospital, Guangzhou, China
Objective: To assess the diagnostic value of 18F-FDG
imaging for interictal and ictal epileptic foci. Methods:
Twenty-three patients with refractory epilepsy were examined
in the interictal and ictal periods by 18F-FDG PET, the
results of examination have been compared with MRI and
EEG. The patients are ten cases in male, 13 cases in female.
The youngest is a 1 year old, the oldest is 14 years old.
Result: There are some abnormal metabolism focuses on
PET imaging in 21 (91.3%) patients with epilepsy. Lower
metabolism focus is in 19 cases, higher metabolism focus is
in two cases; and two cases is in normal. One case of two
with higher metabolism focus had seizures during the examination.
Before examination in 3 h there is an epileptic
attack in other one case. The epileptic focus had been
moved by operation in another two cases. It has been
found that the location of focus is the same with the abnormal
metabolism focus from PET. Younger children with
epilepsy, their abnormal metabolism focus is much more
than elder children. MRI is abnormal in 69%, EEG is abnormal
in 86%. Conclusions: The diagnostic value of 18F-FDG
PET imaging foci was higher than that of MRI and EEG in
sensitivity. The specificity and accuracy of 18F-FDG PET
imaging for the diagnosis of epilepsy during epilepsy ictal
period will be the best.
FP-M-020
Diagnostic value of MRA on acute hemiplegia syndrome
in childhood
W.-C. Zhang, H.-H. Wu, T.-C. Liou
Department of Neurology, Beijing Children Hospital, Beijing,
China
Objective: To study the diagnostic value of MRA on
acute hemiplegia syndrome. Methods: Thirty-four AHS
children were studied by 3D TOF MRA, using MRI as the
combined diagnostic method. Results: Twenty-four patients
had cerebrovascular abnormalities including nine stenoses
and 15 severe narrowing or occlusions, which were respectively
located in ICA of 11 patients, MCA of 24 patients,
and ACA of six patients. MRA showed vascular compensation
of 20 patients. Conclusion: The results indicate that
MRA showed good ability of deterting intracranial vascular
disorders in AHS study as a non-invasive, quick and efficient
tool with no need of radiographic agent. MRA will
probably become an important and commonly used diagnostic
technique for CVD in children.
FP-M-021
Functional MRI study in school children with attention
deficit hyperactivity disorder
Z. Jin a , L. Zhang a , Y.-F. Zang b , Y.-W. Zeng a , Y. Wang a , Y.-
F. Wang b
a fMRI Center, Hospital 306, Beijing, China; b Mental Institute,
Peking University, Beijing, China
Background: ADHD is a major developmental disorder
affecting 3–7% of school children, which is associated with
a low educational outcome and increased risk for antisocial
disorders and/or drug abuse in adulthood. The pathogenic
mechanisms of ADHD remain unknown. Method: Brain
activation maps had been observed pre and post one dose
of Ritalin (10 mg) in nine schoolboys with ADHD (aged 10–
14 years) and nine matched healthy controls using blood
oxygenation level dependent (BOLD) functional MRI technique.
Event-related Simon tasks contained neutral and
interference tasks were served as the stimuli. Results:
Comparing with healthy controls, ADHD children showed
significantly reduced activity in the prefrontal lobe, anterior
cingulate cortex, basal ganglia, cerebellum, and inferior
parietal lobulus during interference (difficult) task. During
neutral (easy) task, reduced activities in ADHD children
were mainly found in the prefrontal lobe, whereas more
activation was found in basal ganglia, cerebellum, and inferior
parietal lobules in patients compared with those in
healthy controls. After one dose of Retalin, reduced brain
activation were recovered in ADHD group. Conclusion: In
ADHD children, there seems to be some dysfunctional brain
areas, including prefrontal lobe, basal ganglia, cerebellum,
and inferior parietal lobulus. Retalin may help to reverse the
dysfunction partially.
FP-M-022
Early detection of hypoxic injury of the fetal human
brain by using MRI and 1 H MRS
V.A. Rogozhyn a , Z.Z. Rozhkova a , L.G. Kirillova b , E.M.
Lukjanova b , A.P. Perfilov b
a Radiological Center, Academy of Medical Sciences of
Ukraine, Kyiv, Ukraine; b Institute for Pediatrics, Obstetrics
and Gynecology, Academy of Medical Sciences of Ukraine,
Kyiv, Ukraine
Our goal is evaluating the brain anomalies in human
fetus by MRI, for measurement of fetal brain structures,
and 1 H MRS, for determination of cerebral metabolite
concentrations. Two groups of 17 women with (G1) and
15 women without (G2) complicated pregnancies are
studied by 1.5 T Magnetom Vision System (SIEMENS).
MR-images of fetal brain in utero (from 25 to 39 weeks of

590
Abstracts
gestational age) were obtained with HASTE sequence (TR/
TE ¼ 80/8, 16, 24, 32 ms; FA ¼ 30; FOV ¼ 360, slice
thickness ¼ 4 mm, matrix ¼ 256 £ 256) in sagittal, axial,
and coronal planes. Fetal brain structures including lateral
ventricles, corpus collosum, and cerebellar vermis are
measured.
1 H spectra are recorded with the STEAM
sequence (TR/TE ¼ 1365/135, 20; 1500/270 ms) combined
with PRESS sequence (TR/TE ¼ 1500/10 ms) for more
effective suppression of water signal, VOI ¼ 15 £ 15 £ 15
mm, NS ¼ 128. Integral intensity of signals of NAA, Cr,
Cho, Lac, and Glu-Gln complex are determined. The metabolite
ratios NAA/Cr, Cho/Cr, NAA/Cho and NAA/
(Cho 1 Cr) are calculated from spectra. From MR-images
of fetal brain in G2 it is found that the ratio ventricle/brain
decreases, and the length of corpus collosum, and also the
length, height, and area of cerebellar vermis increase with
gestational age. The growth and development of the fetal
brain in G1 are different from ones in G2. The normal brain
demonstrate rapid changes during the fetal period, and in
the case of hypoxic ischemic injury the growth retardation
of fetal brain is observed. The ratios NAA/(Cho 1 Cr),
NAA/Cr and NAA/Cho for G1 and G2 are not significantly
distinguishable. The ratio Cho/Cr decreases significantly in
G1 compared to G2. Lac signal present in nine cases in G1
and in two cases in G2. In seven cases in G1 the decrease
of the intensities of Glu-Gln signals in comparison with G2
is observed. This occurs together with reducing the ratio
Cho/Cr and appearing Lac. MRI and 1 H MRS are very
sensitive and effective methods for early detection of
fetal hypoxic ischemic injury. Both the methods can be
applied in obstetric diagnostics for assessment and understanding
of intrauterine growth retardation.
FP-M-023
Using digital subtracted angiography to detect children
intracranial vascular malformation
Z.-P. Wang, J.-M. Yu, W.-X. Zhong
Shanghai Children’s Medical Center, Shanghai Xin Hua
Hospital, Shanghai Second Medical University, China
Objective: To report various types of intracranial vascular
malformations in children; and to determine the clinical
value and safety of angiography. Methods: Review of the
data of 42 patients who had been suspected to have vascular
diseases by CT scan or MRI examination. Digital subtracted
angiographies (DSA) were conducted through the femoral
artery by inserting the catheter into the brachiocephalic
artery. Results: Thirty patients were found to have anomalous
vessels in their brains. Among them, 15 had arteriovenous
malformations, three had intracranial aneurysms, six
had arteriovenous fistulas, five had moyamoya syndrome,
and one had Yasuda syndrome. There were no abnormal
findings in the remaining 12 patients. MRI showed two
patients with cryptic intracranial vascular anomalies.
Conclusion: DSA played an important role in diagnosis
and treatment of vascular diseases, indicating a promising
future for its use in child neurology diseases.
FP-M-024
Bilateral hypodensity of the basal ganglia. Clinicoevolutionary
correlation in children
A. Martínez-Bermejo, J. Arcas, V. López-Martín, A.
Tendero, C. Roche
Service of Neuropediatrics, Hospital La Paz, Madrid, Spain
Introduction: The presence in neuroimaging of areas of
symmetrical bilateral hypodensity in the basal ganglia
(SBHBG) is a striking and unusual finding. Objective: To
determine the aetiology, clinical significance and evolution
of a group of paediatric patients with SBHBG. Patients and
methods: We made a study of 21 patients with neuroimaging
studies (CT or MR) showing SBHBG. The affected area was
related to the aetiology, clinical features and evolution.
Results: The ages varied between 4 months and 16 years.
In seven cases Leigh’s disease was diagnosed, five had had
acute hypoxia, four glutaric aciduria type I, and one case
each of methylmalonic aciduria, Ia glycogenosis, CO intoxication,
acute striatal necrosis and bacterial meningitis. The
putamen was affected in six cases, globus pallidus in four
cases and the lenticular nucleus was damaged in the rest.
Three cases also had lesions in the caudate nucleus. MR was
better than CT for localization of the precise area involved.
Clinically, 13 cases had extrapyramidal signs. We found no
relation between the size, localization of the lesion and the
prognosis, which was more dependent on the aetiology, only
one patient (CO intoxication) recovered and eight died
(Leigh’s disease and one case of hypoxia). Conclusions:
The presence of SBHBG in a patient makes extensive
study necessary to find the aetiology. It is a non-specific
finding, usually of metabolic origin and with little correlation
with the clinical condition. Its presence implies a poor
prognosis and raises suspicion of the presence of certain
neurological disorders.
FP-M-025
Cranial ultrasonograpic characteristics and clinical
correlates in young infants with Sturge-Weber
syndrome (SWS)
Y.-C. Chang a , C.-C. Huang b , L.-T. Huang a
a Department of Pediatrics, Chang Gung Children’s Hospital,
Kaohsiung, Chinese Taipei; b National Chung Kung
University Hospital, Tainan, Chinese Taipei
SWS is a rare congenital disorder that is marked by angiomatosis
involving face, eyes, and leptomeninges. Early identification
of the extent of pial angioma, cortical atrophy and
calcification is critical in determining the prognosis. Ultrasonographic
(US) studies are scarce in infants with SWS. In
this study, cerebral US findings of SWS were characterized in

Abstracts 591
four infants, including two infants presented with hemiparesis
or seizures, and two newborns without neurological
signs. Increased echogenicity of the pia-arachnoid was seen
in all four patients, with extensive temporo- parieto- occipital
involvement in three, and temporo-parietal in one. A thin
ribbon of hyperechogenicity subjacent to the affected cerebral
cortex was found in all four patients. Subcortical white
matter hyperechogenicity was shown in three and two evolving
to cortical calcification during follow-up. Contrastenhanced
MR imagines performed in three patients revealed
enlarged choroid plexus in three, white matter abnormalities
in two and cerebral atrophy in one. One newborn developed
intractable epilepsy at age 6 months, along with rapidly
progressive cortical and subcortical hyperechogenicity and
cortical atrophy demonstrated by US. The other newborn
with stable subcortical white matter hyperechogenicity
remained seizure-free, and had mild hemiparesis and normal
cognitive development at age 1 year. Our study demonstrated
that US is useful in early detecting the pial and parechymal
involvement of SWS in newborn infants who have facial
nevus flammeus, and is also of prognostic values. Moreover,
it can detect more extensive cerebral abnormalities associated
with SWS than contrast-enhanced MR images in
young infants.
FP-M-026
SPET imaging of striatal dopamine transporters and D 2
receptors in patients with autosomal recessive l-DOPA
responsive infantile Parkinsonism due to tyrosine
hydroxylase deficiency
J.F. de Rijk-van Andel a , J. Booij b , P.J. van Noorden c , R.A.
Wevers d , E.A. van Royen b
a Department of Neurology, Amphia Hospital Breda, The
Netherlands; b Graduate School of Neurosciences Amsterdam,
Department of Nuclear Medicine, University of
Amsterdam, Academic Medical Centre, The Netherlands;
c Department of Nuclear Medicine, Amphia Hospital
Breda, The Netherlands; d Laboratories of Pediatrics and
Neurology, University of Nijmegen, The Netherlands
The enzyme tyrosine hydroxylase (TH) catalyzes the
conversion of l-tyrosine to l-dihydroxyphenylalanine (l-
DOPA), the rate-limiting step in the biosynthesis of dopamine.
TH deficiency (THD) has recently been found in the
recessive form of dopa-responsive dystonia (DRD), which is
characterized by extrapyramidal symptoms in infancy and a
good clinical response on low-dose l-DOPA. In the present
study, striatal dopamine transporters and D 2 receptors were
studied by means of single photon emission tomography in
two drug-naive children with THD. Striatal dopamine transporters
were also studied in a third child with THD, already
treated with a low dose of l-DOPA on the moment of
imaging. The results of the study suggest normal integrity
of striatal dopaminergic nerve terminals and striatal D 2
receptors in recessive DRD with THD. These findings
give hope for a sustained response to low-dose l-DOPA,
without the development of unfavourable side-effects, in
patients with THD.
FP-M-027
Congenital mirror movement: investigation of
functional magnetic resonance imaging and transcranial
magnetic stimulation
Y. Maegaki a , T. Koeda b , A. Seki a , I. Suzaki a , S. Sugihara c ,
T. Ogawa c , T. Amisaki d , C. Fukuda e
a Division of Child Neurology, Institute of Neurological
Sciences, Faculty of Medicine; b Department of Humanity
Education, Faculty of Education and Regional Sciences;
c Department of Radiology, Faculty of Medicine; d Department
of Biological Regulation, School of Health Science,
Faculty of Medicine; e Department of Pathological Science
and Technology, School of Health Science, Faculty of Medicine,
Tottori University, Japan
Two patients with congenital mirror movement, one a
child and the other an adult, were studied using fMRI and
TMS. Bilateral primary sensorimotor cortices were activated
during unilateral hand gripping on fMRI. Bilateral
motor evoked potentials were induced from the hand and
forearm muscles after TMS of each hemisphere. Bilateral
motor responses were also induced from the arm muscles in
the adult patient. The contralateral motor responses to TMS
were smaller than the ipsilateral ones in the hand muscles,
while the former were larger than the latter in the arm
muscles. The contralateral motor responses reduced in
amplitude or disappeared in the hand muscles and increased
in the forearm muscles with increasing age while his mirror
movements decreased gradually. Our results suggest that an
unusual cortical motor organization develops in patients
with congenital mirror movement. Bilateral activation of
the primary sensorimotor cortices during intended unilateral
hand movement and ipsilateral motor evoked potentials are
responsible, at least in part, for the pathophysiology of
congenital mirror movement. Reduction of the contralateral
hand motor responses and increase of the contralateral forearm
motor responses may be related to the decrease in
mirror movements during development.
FP-M-028
Correlation of apparent diffusion coefficient of water
changes with expression of a water channel protein,
aquaporin 4, in developing rat brain following hypoxiaischemia
S. Meng a,b , M. Qiao a , P. Latta a , M.R. Del Bigio, B.
Tomanek a , U.I. Tuor a
a Institute for Biodiagnostics (West), National Research
Council; b Department of Pediatrics, The Second Clinical
College, China Medical University, NW, Calgary, Alta,
Canada

592
Abstracts
Introduction: Decreases in the ADC of water measured
using magnetic resonance imaging techniques have been
well documented to occur in cerebral ischemia. Cellular
edema is considered an underlying cause of these changes.
Recent studies have demonstrated a key role of water channel
protein, aquaporin 4 (AQP4) in the development of brain
edema, however, few studies have examined whether
ischemic ADC changes in developing brain are related to
changes in the expression of AQP4. Methods: One or 4
weeks old rats were subjected to an episode of cerebral
hypoxia-ischemia (HI) (unilateral carotid artery occlusion
plus exposure to 8% oxygen 1 h). ADC images were
acquired before, during, and 1 or 24 h after HI. AQP4
expression was assessed immnuohistochemically in
subgroups of animals at each time point. The correspondence
between hypoxic-ischemic changes in AQP4 expression
and ADC changes were assessed. Results: In the brain
of sham control rats, there were no left-right differences in
ADC values or AQP4 staining although ADC values were
greater in 1-week than 4-week old animals. In both age
groups, ADC values in the ipsilateral hemisphere decreased
during HI, partially recovered at 1 h after HI and decreased
again at 24 h after HI. The areas of ADC decreases were
distributed throughout much of the ipsilateral hemisphere
during HI, with some recovery at 1 h after HI, and further
recovery at 24 h after HI. AQP4 expression was decreased
during, and at 1 and 24 h after HI in both age groups, with
the changes corresponding well to the distribution and
values of altered ADC at the different times. Conclusion:
The diffusibility of water in brain as detected with magnetic
resonance Imaging is dependent on the stage of postnatal
development. The correspondence between AQP4 and ADC
changes during or following hypoxia-ischemia suggests an
important role for this water channel protein in the cell
swelling, however, other tissue changes should also be
considered to account for the hypoxic-ischemic changes in
the ADC of water.
FP-M-029
A quantitative MRI study of the corpus callosum in
autistic children
K. Tao, K. Mori, T. Hashimoto, K. Inoue, Y. Toda, M.
Miyazaki, T. Saijyo, Y. Kuroda
Department of Pediatrics, School of Medicine, University of
Tokushima, Tokushima, Japan
The corpus callosum (CC) is the main interhemispheric
commisure of the brain and connects homologous cortical
areas. We measured the total midsagittal area and seven
subregions of the CC, which denote cortical regions according
to Witelson, on MRI scans from 30 autistic and 17
normal young children (age, 3–5 years). Area of the anterior
subregions (Region 1: the rostrum 1 Region 2: the genu)
was smaller in the autistic group (autistic group;
118.5 ^ 14.6 mm 2 versus control group; 134.6 ^ 21.3
mm 2 , P ¼ 0:048). There were no significant differences
between groups in the total midsagittal area and the other
areas. This observation suggests decreased functional
connections between cerebral cortex and delayed maturation
in the frontal lobe of autistic children.
FP-M-030
Magnetic resonance imaging (MRI) changes in
childhood Wilson’s disease after treatment
J.-M. Zhang, M.-X. Wang, K.-R. Bao
Xinhua Hospital, Shanghai Second Medical University,
Shanghai, China
Objectives: To observe the changes of MRI during the
course of treatment in Wilson’s disease and evaluate its
clinical significance. Methods: Fifty-seven patients with
Wilson’s disease received routine SE sequence MRI of
brain and liver. Nineteen patients with abnormal MRI (ten
pretreated or newly-treated and nine follow-up patients) and
each of them had 2 , 4 times of serial MRI examinations at
interval of 1 , 1.5 years. Clinical data with MRI were
analysed. Results: In nineteen patients with abnormal MRI
findings, nine patients had lesions in both brain and liver
(46.37%), seven patients in brain (36.84%), three patients in
liver (15.79%). Most of the patients with abnormal MRI
showed a pattern of bilateral, symmetrical T2W high signal
or/and T1W low signal. Abnormal MRI of the liver showed
multiple rounded T1W high signal lesion and T2W low
signal lesion. The major lesions disappeared or decreased
after rational treatments. The rates of improvement in
patients with adequate treatment were markedly higher
than patients with inadequate treatment (x 2 ¼ 19.36,
P , 0:01). Conclusions: Hepatic and brain lesion as
shown in MRI may disappear after adequate treatment.
There were only mild or no change in MRI abnormalities
in patients with inadequate treatments comparable with poor
clinical improvement. MRI was helpful in understanding
therapeutic effect and prognosis of Wilson’s disease.
FP-M-031
Brain SPECT and MR imaging in postinfectious acute
cerebellar ataxia: A case report
T. Isagai, Y. Yamashita, S. Yatsuga, R. Fukui, A. Kusaga,
C. Fujimoto, T. Matsuishi
Department of Pediatrics and Child Health, Kurume
University School of Medicine, Fukuoka, Japan
Purpose: There have been few reports on SPECT findings
in patients with acute cerebellar ataxia in children. We have
previously reported on five patients with postinfectious
acute cerebellar ataxia with decreased cerebellar blood
flow using 123 I-IMP SPECT (J Neurol Neurosurg Psychiatry
1999;67:109–112). No abnormal MRI findings were found
in these five patients and they all recovered in 2 weeks. We

Abstracts 593
here report a patient with acute cerebellitis who showed
prolonged symptoms and abnormal MRI/SPECT findings.
Case report: A 7-year-old girl was admitted to Kurume
University Hospital with headache, ataxia and slurred
speech, 2 days after upper respiratory infection. Neurological
examination was unremarkable except cerebellar signs.
Laboratory data showed elevated WBC of 13600 and CRP.
Cerebrospinal fluid studies demonstrated 91 lymphocytes/
mm 3 , total protein content of 135 mg/dl. The myelin basic
protein was normal. Epstein-Barr and varicella-zoster viral
titers in serum were negative. Mild consciousness disturbance
and dysphagia were observed for a few weeks, but
she gradually recovered in 2 months. Her initial MRI
demonstrated diffuse cerebellar edema in T1-weighted
image and increased cerebellar intensity in T2-weighted
image, however SPECT image was normal. One month
later her SPECT showed decreased cerebellar and pontiine
blood flow with normal MRI images. Conclusion: The
combined use of MRI and SPECT was useful in evaluating
pathophysiology of patients with acute cerebellar ataxia and
cerebellitis
FP-M-032
Quantitative ultrasonic tissue characterization of the
neonatal brain
C. Fujimoto a , Y. Yamashita a , H. Kanda a,b , E. Harada a,b ,Y.
Maeno a,b , T. Hori b , T. Matsuishi a
a Department of Pediatrics and Child Health; b Maternal and
Perinatal Medical Center, Kurume University School of
Medicine, Fukuoka, Japan
Purpose: Increased echogenecity of periventricular white
matter in the acute phase and later development of cysts is
the classic sonographic picture of cystic PVL, however
evaluation of periventricular echodensities (PVE) is rather
subjective and normal periventricular ehogenic halo may
mimic PVL in some patients. To clarify the significance
of ultrasonic integrated backscatter (IBS) as a quantitative
method of evaluating PVE, we investigated sequential
change of IBS levels in neonatal brain by birthweight.
Patients and method: Cranial sonography were routinely
performed by neonatologists using SONOS 5500 (Philips)
with a 12.5 MHz probe in 43 newborn neonates admitted to
Maternal and Perinatal Medical Center, Kurume University
Hospital. The neonates were divided into four groups: group
A, nine extremely low birthweight infants; group B, 14 very
low birthweight infants; group C, 15 infants with birthweight
1500–2499; and group D, 15 infants with birthweight
more than 2500 g. The first scan was performed
within 24 h after birth, then weekly follow-up scans until
1 month were performed. The IBS levels were measured at
five region of interests (ROIs) bilaterally; periventricular
white matter (WM), choroid plexus (CP), TH, lateral ventricle
(LV), and occipital skull bone (SB). Results: The IBS
levels varied by individuals, however there were no differences
in IBS values of five ROIs between right and left
parasagittal view in each patient. The IBS levels were
higher in the order of SB . CP . WM . TH . LV in all
groups. The IBS levels were sequentially decreased in perivenrtricular
WM in all groups. Conclusion: A real-time IBS
imaging system may be useful in characterizing brain tissue
in the neonate.
FP-M-033
Juvenile neuronal ceroid lipofuscinosis: progressive
brain atrophy on serial MRI
R. Fauze, H.B. Bonet, M.C. Boente, M. Winik
Neurology, Dermatology, Pathology, Children Hospital,
Tucumán, Argentina
A 13-year-old girl, daughter of unrelated healthy parents.
Pregnancy and delivery were normal. Psychomotor development
was normal until 7 years when her teacher noticed a
progressive decrease in her attention level in addition to
communication dysfunction and social withdrawal. A
psychiatric condition was suggested. At the age of 8 she
developed myoclonic jerks. Neurological examination
revealed an inattentive girl with poor fine motor skills. CT
scan showed mild atrophy. EEG showed pseudoperiodic
burst of high amplitude slow waves with posterior spikes
to low frequency photic stimulation. Valproic acid was
prescribed. Two months later, however, myoclonic epilepsies
became more frequent and clonazepam was added to
the regimen. At that time, she was completely mute. By the
age of 11, fell due to ataxia. MRI showed moderate atrophy.
ERG and VEP were decreased. Vacuolated lymphocytes
were present. Skin ultrastructural examination showed
predominance of fingerprint profiles. Ophthalmological
examination not revealed macular degeneration. By the
age of 13 she had myoclonias and a motor disturbance
with ataxia. Myoclonic epilepsies, mental deterioration,
ataxia and progressive brain atrophy suggests NCL,
however a definitive diagnosis is based on skin or conjunctival
biopsy that reveal storage of specific lipopigments.
NCL are a group of progressive, autosomal-recessive and
finally fatal neurodegenerative diseases in children marked
by lysosomal accumulation of disease-specific lipopigments
in cerebral and extra-cerebral tissues and neuronal loss in
brain and retina.
FP-M-034
Magic angle spinning 1 H MR spectroscopy of autopsy
samples from patients with neuronal ceroid
lipofuscinoses reveals decreased neuronal metabolites
T.H. Autti a , A.-M. Häkkinen b , B. Sitter c , J. Tyynelä d ,T.
Bathen c , U. Sonnewald c , I.S. Gribbestad c
Departments of
a Radiology and b Oncology, Helsinki
University Central Hospital, Finland, Helsinki, Finland,
c MR Center, SINTEF Unimed and Department of Clinical

594
Abstracts
Fig. 1. HR MAS spin-echo spectra of autopsy samples from a control (top)
and an INCL patient (bottom).
Neurosciences NTNU, Trondheim, Norway; d Department of
Biochemistry, Helsinki University, Finland
NCLs are among the most common progressive childhood
encephalopathies. We have applied MAS 1 H MRS to
brain autopsy samples of infantile and juvenile NCL
patients, along with samples of controls without neurological
deficits. Material and methods: The autopsy samples
of frontal cortices from patients with INCL (n ¼ 10), JNCL
(n ¼ 6) and controls (n ¼ 9) were kept at 2808C until MR
analysis. HR proton MAS spectra were recorded using a
BRUKER AVANCE DRX600 spectrometer equipped with
a 1 H/ 13 C MAS probe with gradient. Principal component
analysis (PCA) of mean normalised spectra was performed
on the spectral region 4.6–0.6 ppm. The lactate doublet
was excluded from PCA due to high intensity compared
to other metabolites. Score and loading plots were examined
in detail. Results: Fig. 1.
FP-M-035
Acute disseminated encephalomyelitis and multiple
sclerosis: is there any association?
W.-T. Lee, T.-W. Yu, J.-S. Liang, Y.-Z. Shen
Department of Pediatrics, National Taiwan University
Hospital, Taipei, Chinese Taipei
Both MS and ADEM are rare inflammatory demyelinating
diseases of the CNS in children. Compared with
western countries, the incidence of MS is much lower in
Taiwan. In the past 7 years, only 12 patients below 18
years of age were diagnosed to have ADEM in our hospital.
Of these, two (17%) turned out to have MS 6 and 2
years later, respectively. The first was an 18-year-old girl.
She was diagnosed to have ADEM at 12 years old, with
initial manifestations of acute onset of psychiatric symptoms
followed by focal neurological signs. The MRI
showed multiple lesions over gray and white matters and
spinal cord, compatible with ADEM. She developed
sudden onset of bilateral optic neuritis followed by right
hand weakness 6 years later, when MS was diagnosed. She
experienced another relapse one month later. Another
patient was an 8-year-old boy, who was diagnosed to
have ADEM at 6 years old, when he had preceding infection
followed by limb weakness. However, one episode of
optic neuritis, followed by another episode of optic neuritis
and limb weakness occurred 2 years later. MRI showed
multiple lesions compatible with MS. Judging from the
low incidence of MS and ADEM in Taiwan, we conclude
that there may be some correlations between the pathogenesis
of MS and ADEM. Longer follow-up of the children
with ADEM may be helpful to clarify the relationship of
MS and ADEM.
FP-M-036
Lactate quantification by means of proton magnetic
resonance spectroscopy in patients with congenital lactic
acidosis
N. Iwasaki a , R. Tanaka a , T. Isobe b , A. Matsumura b ,I.
Anno c , T. Ohto a , A. Matsui a
a Department of Pediatrics, b Neurosurgery and c Radiology,
Institute of Clinical Medicine, University of Tsukuba, Ibaraki,
Japan
Proton MRS provides a useful approach for monitoring
children with disorders of lactate metabolism. However,
most studies have evaluated lactate only by its existence
or by relative ratio. In the present study, we have a developed
method for lactate quantification and applied it to three
clinical cases with congenital lactic acidosis. Two of them
had pyruvate dehydrogenase complex deficiency and one
had Leigh syndrome. All patients were examined on a clinical
1.5 T super-conducting MR whole body system; two of
them twice. Data were acquired by using point resolved
spectroscopy (PRESS) sequence. We used a coupling
constant of J ¼ 7.35 Hz (2/J ¼ 272 ms). T2 relaxation
correction was performed for water and lactate in each
case. For lactate, in-phase signals were used to calculate
the T2 relaxation time. The volume of interest was placed
in the ventricular space. The tissue water signal was used as
an internal standard using a water concentration of 100%
(55 mmol/l). The calculated lactate concentration from
proton MRS was compared to the CSF lactate level. The
calculated lactate concentration and CSF level were
3.21 ^ 1.73 and 3.53 ^ 1.52 mmol/kg, respectively. The
correlation coefficient was 0.91. To our knowledge, this is
the first study to estimate the reliability of lactate quantification
using proton MRS in vivo and has allowed us to
examine the precise lactate metabolism of the brain noninvasively.

Abstracts 595
FP-M-037
Use of transcranial ultrasound in the diagnosis of
intracranial bleed in critically-ill children 2 months–2
years old. A pediatric intensive case unit experience
J.A. Comuelo, J.A. Robles, M. Sancho, H. Cifra, L.
Tolentino
Child Neuroscience Division Philippine Children’s Medical
Center Quezon Avenue, Quezon City, Philippines
A retrospective cross-sectional validation study was
conducted to determine the accuracy of transcranial ultrasound
(CUTZ) in the immediate detection of intracranial
(IC) bleed in critically-ill children at a pediatric ICU of a
tertiary hospital over a 30-month period. Inclusion criteria:
2 months–2 years, patent anterior fontanelle, suspected
intracranial bleed, with initial CUTZ and confirmatory CT
scan. Exclusion criteria: clinical deterioration in-between
procedures, .24 H-interval between procedures. Age, sex,
and initial impression were recorded, and CUTZ findings
were compared with CT scan results as to presence of IC
bleed, location, and associated findings. From 239 cases
enrolled, 41% had IC bleed. Male comprised 61%, with
70% less than 1 year old. Significant correlation was
noted with 19–24 months old group-82% had IC bleed
(0.131). Non-traumatic causes (61%), with 70% less than
1 year old. Significant correlation was noted with 19–24
months old group-82% had IC bleed (0.131). Non-traumatic
causes (61%) include seizure disorders, CNS infections,
sepsis, and APCD. Only sepsis showed a significant correlation
(0.175)-73% had IC bleed. IC bleeds were detected at
the ff. areas: intraparenchymal (47.5%) intraventicular
(28%), subdural (13%), subarachnoid (9%), and cerebellar
(3%). Associated findings were cerebral edema (20%),
meningitis (5%), and hydrocephalus (1%), CUTZ had a
sensitivity of 94% and specificity of 99%; 1PV-98.9%;
PV ¼ 95.8%; accuracy ¼ 95%. Using correlation and
regression analysis, CUTZ had a very high correlation
(0.895; sig value ¼ 0.126) with CT scan with no significant
difference. Conclusion: Transcranial ultrasound can be used
as an accurate diagnosis tool for the immediate detection of
intracranial bleed in critically-ill children 2 months–2 years
old.
FP-M-038
Occipital MRI abnormalities following neonatal
hypoglycemia
D. Yalnizoglu, M. Topcu, G. Turanli, A. Cila
Hacettepe University Department of Pediatric Neurology
and Department of Radiology, Ankara, Turkey
Imaging features of infants who suffered brain damage as
a result of neonatal hypoglycemia were shown to have similar
patterns, and to affect the parietal and occipital lobes
most severely. A variety of disorders may underlie similar
neuroimaging features in childhood. We report ten patients
with predominately occipital lesions on MRI, and attempt to
relate the neuroimaging findings with underlying neonatal
and early infantile insults. The medical records of ten
patients with predominately occipital lesions on MRI were
reviewed retrospectively. CT was available in three patients.
Imaging studies were obtained between 40 days and 11
years of age. The ages at final follow up ranged between
5.5 months and 11 years; age at admission ranged between 2
days and 11 years. Four/10 patients had well documented
hypoglycemia. Seven patients had sepsis and/or hyperbilirubinemia,
associated with hypoglycemia in two. Seven
patients had perinatal hypoxia. All patients have suffered
mild to moderate global developmental delays. Nine
patients had refractory epilepsy, and two had cortical blindness.
MRI showed severe parietooccipital/occipital volume
loss with some asymmetries in all patients. CT showed
occipital calcification compatible with laminar cortical
necrosis in one patient. Our patients who suffered merely
from neonatal hypoglycemia showed occipital predominance
of MRI abnormalities similar to the previous reports
on neonatal hypoglycemia. Early insults such as hypoxiaischemia,
neonatal sepsis may result with similar lesions.
Prompt recognition and treatment of symptomatic neonatal
hypoglycemia is essential to minimize future neurological
sequelae such as cortical blindness, mental retardation and
refractory seizure disorder.
FP-M-039
PET in Landau-Kleffner syndrome
I. György, I. Velkey, B. Gulyás, L. Trón
Pediatric Department of University of Debrecen, Country
Hospital of Miskolc, Karolinska Institute, Stockholm, PET
Center of University of Debrecen Pediatric Department of
University Debrecen, Debrecen, Hungary
LKS is a rare disorder with a variable course characterized
by gradual or rapid onset of aphasia in a previously
normal child. Children with LKS syndrome have abnormal
EEG: focal spikes or spikes and waves in the temporal or
central area of the brain and electrical status epilepticus in
slow sleep. Epileptic seizures are not frequently in this
syndrome and they are easy to treat. We observed three
children with LKS. Two of them suffered from difficulty
of comprehension of the speech and the other one from
expressive aphasia. All three children had only few epileptic
seizures, but their EEG showed massive paroxysmal signs:
focal spikes awake and electrical status epilepticus in slow
sleep. MRI was negative in all three cases. The cause of
disorder remained unknown. All three children were righthanded.
FDG PET was made in our patients. We found
decrease in glucose utilization in all patients in the left
auditory speech cortex. PET of the boy with difficulty of
expressive speech showed decrease in the glucose utilisation
in the left Broca area, too. All patients were treated with

596
Abstracts
antiepileptic drugs. Prednisolone was done plus to the two
girls with disturbance of receptive speech and intravenous
gammaglobulin to the boy with expressive aphasia. One girl
treated with prednisolone cured. In the other, aphasia
remained and a severe regression of mental function was
observed. The boy with expressive aphasia cured, too. The
PET investigation after healing showed marked improvement
but not normalisation in glucose utilization of the
speech-areas of the brain.
FP-M-040
The spectrum of MRI findings in subacute sclerosing
panencephalitis
Ö.F. Aydin, N. Şenbil, Y.K.Y. Gürer
Departments of Pediatric Neurology, Dr. Sami Ulus Children’s
Hospital, Ankara, Turkey
Purpose: Evaluation of patients diagnosed as SSPE with
MRI and investigation of the relation between MRI findings
and the stage of the disease. Material and methods: Fortytwo
patients with SSPE were evaluated by MRI on T1, T2,
FLAIR and PD series, and the findings were compared with
the stages of the disease. Results: Pathological findings were
found in 33 patients and nine patients had normal findings.
Inflammatory demyelinating lesions were seen mostly with
hypointense appearance on T1 series and with hyperintense
appearance on T2, FLAIR and PD series. Lesions were
asymmetrically located, patchy and multifocal. Pial and
parenchymal contrast enhancement, or local mass effect of
parenchymal lesions was not found. Lesions tended to be
more common in the subcortical white matter (87.8%).
Cerebral atrophy was found in four patients, but this was
not found to be related with the age of the disease. There
was deep gray matter involvement in six patients and brainstem
involvement in three patients, and cortical involvement
was found in two patients. One of the two patients
with cerebellar involvement, showed evident cerebellar
folia and the other one showed hyperintense lesions on
middle cerebellar pedincules. Conclusions: Brainstem,
deep gray matter and cortical involvements observed on
MRI were found at further stages of the disease. Cerebellar
atrophy and cerebellar involvement were not related with
the clinical stages of the disease.
FP-M-041
Clinical and MRI findings in children with transverse
myelitis
Y.-H. Huang
Shantou Central Hospital, Shantou, China
Objective: To explore the clinical and MRI features in
children with transverse myelitis. Methods: Provide spinal
cord MRI examination with the machine of SMT-100XX
from Japan Daojing to four patients, who have been onset of
illness within 3 months. Results: (1) The T2WI in all four
patients shows high intensity, with focal border showing
unclear, little patch, dot shape or reinforcement on the
border. In these four patients, two cases did not have any
change on the T1WI, two did not have augmentation on
spinal cord and I have little augmentation on it. (2) On
neck lesion patients, MRI shows obviously on clinical
dysarthria, not obviously on malfunction of respiratory
muscle and sphincter, slight sensory disturbance and quick
recovery. Conclusion: In children with transverse myelitis,
MRI shows the T2WI is the most sensitive one and the focus
shows little local reinforcement. It seems that children
patients have different features comparing to adults. For
children patients, they have slight sensory disturbance and
most of them have dysarthria, not obviously on malfunction
of respiratory muscle and sphincter. Not all patients show
augmentation on spinal cord.
FP-N
Autism/Learning/Behavior
FP-N-001
Usefulness and limitation of ‘draw-a person’ test to
determine intellectual level
C. Zix
Hospitalor rue Ambroise Pare Saint-Avold Lorraine,
France
The draw-a-man (or draw-a-person) test has been
proposed since 1926 by Goodenough for the rapid evaluation
of intellectual efficiency. We made a prospective study,
comparing the draw-a-man test to conventional IQ studies,
in order to assess the usefulness of this method. Methods:
We studied 81 children (22 girls, 59 boys) who had been
refused to a specialised paediatrician for learning disorders.
All children performed the draw-a-person test, which was
evaluated according to the Goodenough/Harris rating
system. IQ was evaluated with WISC or WPPSI, depending
on age. Six children were tested twice. A total of 87 cases of
combined draw-a-person/IQ tests were analysed. For each
child, we established a ‘main’ diagnosis, after through
analysis of the learning difficulties, including a neurological,
psychological and orthophonic examination. Patients
were assigned to the following categories: MR, instrumental
disorders (dyslexia, dysphasia, dyspraxia: ID), hyperactivity
with attention disorder (ADD), neurotic problems, epilepsy,
and others (psychosis, prematurity). Correlation between
the draw-a-person test and IQ was calculated with correlation
coefficient and regression line. Results: Twenty-two
girls and 59 boys have been examined. The average age
was 9.47 ^ 2.57 years. Diagnosis distribution was as
follows: 21 MR, 19 ID, nine ADD, ten neuroses, 15
epilepsy, seven others. There was a low correlation
(r ¼ 0:42) between the draw-a-person test and global IQ.
Results were most divergent between children with instru-

Abstracts 597
mental disorders and ADD children. When ID and ADD
children were excluded, the correlation coefficient between
draw-a-person tests and IQ tests improved to 0.67. Discussion:
Literature shows an overall good correlation between
draw-a-person tests and global IQ tests. Our study showed
that draw-a-person test must be interpreted with caution,
taking into account instrumental as well as attention disorders,
which may lower the results. The draw-a-person test is
still valid for a rapid initial diagnosis.
FP-N-002
Analysis of topography of spike wave in children with
autism
A. Yasuhara, Y. Yoshida, A. Hori
Department of Pediatrics, Kansai Medical University Kohri
Hospital, Neyagawa, Osaka, Japan
We analyzed the EEG spike in order to clarify the critical
age and expression site of the seizure wave in children with
autism. Fifty autistic children with spike detected and
followed up for over 2 years in our Hospital were included.
Informed consent was obtained from a family member.
Recorded spike was analyzed by the topography and the
dipole analysis (GE Marquette: SynaPoint). Of 50 subjects,
39 showed a spike in the frontal region, 29 in the central, 13
in the parietal, and four in the occipital. The average age of
onset was 3.6 ^ 1.3 in the frontal, 3.5 ^ 0.9 in the central,
4.7 ^ 1.4 in the parietal, and 3.0 ^ 0.17 in the occipital
region. In older patients, the spike appears in the parietal
region further than the front-central region (P , 0:05).
Analysis of patients (n ¼ 8) with the spike in the frontal
region showed a map with the highest potential existing in
Fz. Dipole analysis revealed a focus in the position which
agreed with the deep direction of the frontal region, especially
the cingulate gyrus, the Cz and Pz division showed the
highest potential respectively in the EEG potential map with
the spike in the central and the parietal area. The dipole
accumulated on the spike in the frontal region, especially
in the cingulate gyrus. Dysfunction of the cingulate gyrus
appears to be related to the pathophysiology of autism.
FP-N-003
Clinical study on intelligence and behavioral
characteristics in epileptic children with sub-clinical
seizures
Y.-C. Chen, S.-S. Ren, H.-T. Huang
Department of Pediatrics, Daqing Oilfield General Hospital,
Daqing City, Heilongjiang Province, China
To study the intellectual level and behavior in children of
sub-clinical attack of epilepsy. Methods: Forty-eight cases
of epilepsy with sub-clinical outbreak were studied by
WISC, Eysenck personality questionnaire (EPQ) and
CBCL. Results: There were no significant differences in
VIQ, PIQ and FIQ in the two groups but the code and
number of WISC in the epilepsy group were obviously
lower than those in the control group (P , 0:05). The
total marks on CBCL were significantly higher in the
epilepsy group than the control group, including depression,
restlessness, attack and association. Conclusion: Behavioral
characteristic of children with epilepsy should also be paid
attention though no clinic attacks are observed. Overall
comprehensive treatment should be given to control
outbreak, clear up psychology hindrance and increase quality
of life in children with epilepsy.
FP-N-004
The effect of secretin multidose treatment on the
behaviour of autistic children
W. Brussel, S. van Daalen, M.E. de Richemont
Rijnstate Hospital, Arnhem, Netherlands
In 1998 Horvath et al. reported three children with autistic
spectrum disorders who underwent upper gastrointestinal
endoscopy and intravenous administration of secretin to
stimulate pancreaticobiliary secretion. Not only amelioration
of the diarrhoea and increased pancreaticobiliary secretion
were observed, but also a dramatic improvement in the
behaviour, manifested by improved eye contact, alertness
and expansion of expressive language. After this publication
several anecdotal reports of behavioural improvement were
done by parents on internet. Until now, four randomized,
placebo controlled studies are published, which showed no
effect on the behavioural status after one or two doses secretin.
Secretin is a polypeptide hormone secreted by the
pancreas; secretin has a function in the gastrointestinal
tract, but animal experiments have shown effects on the
central nervous system and behaviour in rat. We want to
know whether secretin multidose treatment should give any
effect on the behaviour of autistic children. Pilot prospective
study was done on four autistic children (age 4–6 years/
DSM-IV criteria); during half a year monthly intravenous
administration of secretin (2 IU/kg bodyweight) was given.
Behavioural status was reviewed by weekly parent rating
scales and Childhood Autism Rating Scale (CARS) tests
before the first dose and 4 weeks after the last (sixth) dose
of secretin. CARS test was done by a blinded child psychologist.
Results showed significant improvement in two of the
four children. Conclusion: One or two doses of secretin have
no effect on the behavioural status in autistic children, but
multidose treatment may be effective.
FP-N-005
Study of behavioral problems of children in Fujian
Coastal area
R.-N. Ren, X.-M. Chen, M.-Y. Ling, L.-Y. Ye, H.-Q. Cao
Pediatric Centre of PLA, Department of Pediatrics, Fuzhou
Dongfong Hospital, Fuzhou, China

598
Abstracts
Objective: To explore behavioral problems of children in
coastal area of Fujian. Methods: Achenbach Child Behavior
Checklist was applied to investigate behavioral problems of
children in Fujian coastal area. Children who live in the city
were compared with those in the country. Results: The
prevalence of childhood behavioral problem is 13.5% in
the city group and 12.4% in the non-city group. There is
no significant difference between the groups. Factors affecting
childhood behavioral problems in the city included
physical disease, relationship between parents and children,
correct nursery method, administration attitude, schooling,
achievement; whereas the country included low birth
weight, physical disease, relationship between parents and
children, correct nursery method, administration attitude.
Conclusion: Physical attribute and family relationship
were the main factors affecting behavioural problems.
Medical care and familial education should be introduced
to treat behavioral problems.
FP-N-006
Reactus in patients with diabetes mellitus and Turner
syndrome
N. Nakamoto, S. Kaneko, F. Inouchi, Y. Fujii, Y. Fujita, H.
Kodama, Y. Yanagawa, M. Miyao
Department of Pediatrics, Teikyo University School of
Medicine, Tokyo, Japan
Aim: Cognitive function is disturbed in adults with
diabetes mellitus (DM) and Turner syndrome (TS).
WISC-R (or WAIS) and event related potential (ERP)
can be used for evaluating cognitive function. Recently,
‘Reactus’ was developed as a test for evaluating cognitive
function. We examined cognitive function in young
patients with DM and TS using ERP, WISC-R and Reactus.
Patient and methods: Eight patients with DM aged 13–
24 years old, and six with Turner syndrome aged 7–19
years were tested. Four patients had IDDM, and four
patients had non-insulin-dependent diabetes (NIDDM),
including two patients with mutation in mitochondria
gene and two patients with maturity onset diabetes of
youth-Dorland (MODY). A patient with IDDM suffered
from hypoglycemic attack, and cognitive function was
examined before and after the attack. Results and discussion:
Visual motor performance was disturbed two patients
with NIDDM and mutation in mitochondria gene. In the
DM patient with hypoglycemia, visual motor performance
was disturbed after the attack. This disturbance continued
for 1 month. In the two patients with TS, visual reaction,
acoustic reaction and visual motor performance were
markedly disturbed. These results suggest that cognitive
function is disturbed in some DM and TS patients.
FP-N-007
The investigation of related causes of abnormal behavior
and emotion in school age children
L.-Q. Chen, Y.-F. Lu, Y.-C. Zhang, Q. Lu
Shanghai Children’s Hospital, Shanghai, China
Objective: To investigate the causation of abnormal behavior
and emotion in school age children. Methods: Thirtyfive
cases (male 17 and female 18) were analysed on school
age children with problem of behavior and emotion in
Shanghai Children’s Hospital. Results: The first age range
of abnormal behavior and emotion was 7–13 years (mean
age was 9.1). The possible causes were related with family
condition, educational model, social living environment and
heredity. Conclusions: Mental problem were susceptible to
outside environment. The morbidity may be increasing in
school children. The behavior and emotional disorders
could lead to organic dysfunction. In order to reduce incidence
of psychiatric problem in childhood, the health educational
model should be put to practice.
FP-N-008
A controlled study of behavioral problems in children
with tension headache
B. Wei, L.-Y. Zhu, M.-L. Liu, Y.-M. Teng
Pediatric Department, PLA 202 Hospital, Shenyang, China
To investigate the characters of behavioral problems in
children with tension headache, 38 patients and 38 healthy
children were assessed using the Achenbach Child Behavior
Checklist. Behavior problems were showed in 17 patients
(44.74%) and in six healthy children (15.79%). There were
significant differences between two groups (x 2 ¼ 6.12,
P , 0:05). Behavior problems in boys were more than in
girls. The major behavior problems in boys with tension
headache were depression, interpersonal difficult, obsession,
social recession, aggressive behavior and violating
discipline, while girls had higher scores on depression,
social recession, physical complains and schizoid-obsession.
We suggest that the children with tension headache
had more behavior problems than healthy children, especially
being in depression and social intercourse. Behavior
problems of the patients should be considered in the treatment,
to get rid of the source of tension headache.
FP-N-009
Selected neuropsychological data in children with a
cavum of the septum pellucidum
A. Kubik, B. Prajsner, M. Kacinski, A. Gergont, S. Kroczka
Department of Pediatric Neurology, Collegium Medicum,
Jagiellonian University, Krakow, Poland
Purpose: This paper presents the effect of a cavum of the

Abstracts 599
septum pellucidum (CSP) on cognitive functions and
emotional-social functioning in children at various stages
of development. Material: We examined 27 children with
CSP (13 boys, 14 girls), aged 4 months–14 years, divided in
three groups (,2, 2–6, .6 years), and 27 as the control
group. Results: IQ of 17 of children with CSP (63%) was
normal, including four patients with IQ above the average.
Four children had IQ below the normal range and six had
overt mental retardation. The intellectual development of
17/27 children was dysharmonic. In the younger group
(five children) poorer results were obtained mostly in active
speech, manual skills and visual perception. In all nine children
from Group 2 visuo-motor coordination development
was delayed. Eight children from this group showed delayed
development of active speech, in three of them passive
speech development was also retarded. Of 13 children in
Group 3, ten achieved higher IQ scores in the verbal than
in nonverbal scale. All children from this group had linguistic
function and logical thinking in the normal range.
Decreased scores were most often observed in the rate of
visuo-motor education, immediate memory and hand motor
functioning. Four children with mental retardation (Groups
2–3) had attention deficits, psychomotor hyperactivity and
lower level of emotional and social maturity. Four children
from Group 3 presented neurotic disturbances. Conclusions:
Development of intellectual functions in more than half
children with CSP was disharmonic.
FP-N-010
Clinical localization in children with attention deficit
hyperactivity disorder (ADHD)
R.J. Konkol, P.F. Carey
Kaiser Permanente NW, Portland, OR, USA; Oregon
Health Science University, Portland, OR, USA
The neurological examination in children diagnosed with
ADHD is usually to rule-out treatable conditions including
epilepsy and tumors. However, functional imaging studies
suggest right frontal abnormalities in ADHD. Our goal was
to determine if there is a relationship between a motor
examination and cognitive impairment in 74 unselected
ADHD children, diagnosed by DSM IV criteria and
completing a neuropsychological exam. The neurological
exam sought lateralized upper motor neuron signs. The
ADHD group consisted of 51 boys and 23 girls (mean
age: 11.7 ^ 3.7 SD). A control group contained 61 consecutive
migraine patients with 19 boys and 42 girls (mean
age: 12.9 years ^ 3.9 SD). Age differences were non-significant
(P , 0:05). Lateralized upper motor pattern of weakness
was found in 41/74 (55%). Only three children had
right-sided weakness with a language-based learning
disability, while 54% (37/74) had left sided weakness. Of
those with left weakness, 46% (17/37) also had verbal learning
difficulty. No weakness plus verbal learning disability
occurred in 36% (13/35). In contrast, only 3% (2/61) of
migraine subjects had left weakness and ADHD. Half of
the children with ADHD had a weakness pattern implicating
the right hemisphere. The few with left hemisphere weakness
had a language problem. Verbal learning problems
were common in those with right hemisphere motor localization
(54%), compared to those with no localization (36%).
These results point to dysfunction in several regional brain
networks, with a significant component in the right hemisphere,
as supported by the neurological examination and
emerging imaging studies.
FP-N-011
Early diagnosis of autism at child development centers in
Israel
Y. Senecky a , G.W. Diamond a , D. Inbar a , A. Apter b ,R.
Weitz c
a Center for Child Development, b Child Psychiatry Department,
c Child Neurology Department, Schneider Children’s
Medical Center, Sackler Tel Aviv University School of
Medicine, Petah-Tiqva, Israel
Background: Children with a variety of cognitive, physical
language related handicaps are being diagnosed at a
younger age. Earlier diagnosis of neurologically based
debilitating conditions, such as Autistic spectrum disorders,
including PDD, provide more effective intervention strategies
aimed at rehabilitation during the critical developmental
period of a child’s language and social skills. Objective:
To determine the median age of diagnosis of a cohort of
subjects with autistic spectrum disorder, and to compare
the age at diagnosis by three groups of physicians involved
in the diagnostic process: (1) pediatricians and neurologists
at child development centers; (2) neurologists working
solo; and (3) psychiatrists. Design: The charts from the
National Social Security office between 1982 and 1997
on 1159 children with a diagnosis of autistic spectrum
disorders were subjected to a retrospective survey. Of the
total, 1127 were Jews, and 32 Arabs. A total of 652 charts
yielded information pertaining to age at diagnosis and
identified the professional making the diagnosis. Data
was subjected to chi square analysis for significance.
Results: The regional distribution of diagnosed cases of
autistic spectrum disorders varied widely, with statistics
from the central metropolitan area reflecting incidence
figures common in other countries, i.e. 2:1000. Peripheral
areas and the Arab sector were significantly underrepresented.
The median age at diagnosis was 35 months
(^12 months), reflecting a relatively young age as
compared to the literature. Developmentally based physicians
diagnosed autism at 33.4 months, Independent
neurologists at 37.9 months, and psychiatrists at 42.5
months. Conclusions: Early diagnosis of neurological
disabilities by professionals at child developmental centers
will enable more efficient management and better intervention
services.

600
Abstracts
FP-N-012
Cognitive processing in paediatric epilepsy: a study of
long latency auditory evoked potentials
M. Zgorzalewicz, B. Zgorzalewicz
Chair and Department of Developmental Neurology
University of Medical Sciences, Poznań, Poland
ERP reflect cognitive process such as memory, attention
or speech processing. The most useful ERP for clinical
evaluation of mental processing is P300 complex. The
purpose of this study is to estimate treatment of epilepsy
in children and adolescents. We assessed ERP in newly
diagnosed patients aged 8 to 18 years before antiepileptic
therapy and in those with CBZ or VPA administered as
monotherapy. They were diagnosed as having generalized
tonic-clonic seizures and partial seizures (simple or
complex), both with and without secondary generalization.
Neuropediatric status and neuroimaging were unremarkable.
All had normal mentality measured by battery of
psychological tests. According to the IFCN recommendation
standards, the ERP was elicited with auditory discrimination
paradigm by presenting a series of binaural 1000
Hz frequent (standard) versus 2000 Hz rare (target) tones.
Statistical analysis showed differences in P300 latencies in
Fz and Cz derivations did not change in amplitudes before
and after CBZ therapy. Based on ERP results, impairment
of cognitive processes in these epileptic children was
detected. ERP recordings after VPA treatment did not
indicate any influence on cognitive function.
FP-N-013
Non-linear dynamic analyses of inter-words time
intervals in children’s speech as a measure of
developmental stage
S. Avissar, D. Todder, G. Schreiber
Faculty of Health Sciences, Ben Gurion University of the
Negev, Beer Sheva, Israel
Speech analyses are usually focused on words as signifiers
ignoring inter-words time intervals (ITIs), which are
related to the ‘form’ of speech, rather to its ‘content’.
Speech of healthy children aged 5–15 was recorded, and it
was analyzed by non linear dynamical-mathematical methods
of correlation dimension, symbolic dynamics and bispectral
analysis. The results of all three methods have
shown age dependency. A significant correlation was
detected between the combined ranks of findings based on
these methods and children’s age (Spearman’s r ¼ 0:850;
P , 0:0001). The results of both symbolic dynamics
entropy calculations, correlation dimension analysis (i.e.
presence or absence of saturation) and the bi-spectral analysis
indicated that there was a transition from one type of
thought processes (primary process, psychotic-like thinking)
to normative adult-type thought processes (secondary
process) at approximately the age of 78. The transition age
obtained by ITIs is thus in agreement with the age generally
accepted for transition, between Piaget’s pre-operational to
concrete operational periods, in the field of developmental
psychology. The analytical techniques of the present study
may therefore become highly suitable for use in the assessment
of child development and the detection of learning and
developmental problems in children at the early years of
school.
FP-N-014
Cognitive sequelae of closed brain injury in children:
efficacy of nootropics
N.N. Zavadenko a , A.I. Kemalov b , A.S. Petrukhin a
a Child Neurology Department, Russian State Medical
University, Moscow, Russia; b MEDEX Medical Center,
Almaty, Kazakhstan
Many children suffered closed brain injury (CBI) have
persistent neurobehavioural sequelae due to the combination
of cortical damage and diffuse involvement of axial
and subcortical structures. A total of 110 children (aged 7–
12 years) with cognitive problems suffered moderate or
severe CBI (contusion) in the period from 6 months–4
years prior to this study. They were divided into four
groups to enter open-label controlled study of efficacy of
Nootropics. The length of treatment was 20 days. Examination
procedure before and after the treatment included
structured questionnaire, scored physical and neurological
examination of subtle signs, testing of working memory
(ten words retention) and sustained attention, EEG. In addition
to symptomatic therapy 1st group (n ¼ 30) was administered
Cerebrolysin (EBEWE, Austria), containing
biologically active neuropeptides (2 ml i.m. daily), 2nd
(n ¼ 20) – Instenon (1 ml i.m. daily, containing etamivan
25 mg, hexobendine 5 mg, ethophylline 50 mg) and 3rd
(n ¼ 30) – Semax, synthetic analogous of ACTH fragment,
devoid of hormonal activity (12.5 mg/kg nasally daily).
Control group (n ¼ 30) received symptomatic therapy
(acetazolamide, vitamines). A total of 80% of patients in
the 1st group (Cerebrolysin), 70% in the 2nd (Instenon)
and 67% in the 3rd (Semax) responded to the treatment
in contrast to 10% of controls. Relief of complaints for
headaches, tiredness, emotional instability, restlessness,
poor concentration and memory, sleep disturbances was
evident after the treatment with Noortopics. Significant
improvement of attention and memory measures was
revealed in the 1st, 2nd and 3rd groups: decrease of executive
time and omissions in attention testing, improvement
of acoustic-verbal memory. These changes corresponded to
positive dynamics on the EEG in responders. Nootropics
are effective for the treatment of cognitive sequelae in CBI
children.

Abstracts 601
FP-N-015
Association of single nucleotide polymorphisms in
serotonin and dopamine receptor genes in autism
Y.-H. Ma a , E. Nanba b , K. Takeshita b
a Institute of Child and Adolescent Health, Peking University,
China; b Division of Child Neurology, Institute of
Neurological Science, Faculty of Medicine, Tottori University,
Yonago, Japan
Family and adoption studies indicate that genetic factors
play a role in the development of many psychiatric disorders
including autistic disorder, a severe neuro-developmental
disorder that affects approximately 2–10/10 000 individuals.
Although many studies have been attempted to elucidate
the genetic background of autism, the genetic factors
associated with its pathogenesis are still not well understood.
In order to evaluate the role of SNPs in serotonin
and dopamine receptor genes in autism, we performed association
studies using 57 autism and 100 normal controls for
SNPs with PCR-SSCP. The results showed that among 20
SNPs in serotonin gene tested, 18 revealed no positive association
with autism, but two SNPs in HTR1A showed significant
association (P , 0:05); among ten SNPs in dopamine
gene tested, seven revealed no positive association with
autism but three SNPs in DRD2 showed significant association
(P , 0:00001). The results indicated that difference in
allele and genotype frequency of HTR1A and DRD2 may
play an important role in the pathogenesis of autism.
FP-N-016
Phencyclidine treatment in newborn rats: behavioral
and neurochemical effects
X.-M. Gao, A. White, C.A. Tamminga
Maryland Psychiatric Research Center, University of Maryland
school of Medicine, Baltimore, MD, USA
Perinatal accidents contribute to the risk for schizophrenia.
Moreover, the illness may involve some abnormality of
the glutamatergic system, especially of NMDA-sensitive
receptor, possibly of neurodevelopmental origins. Whereas,
low doses of phencyclidine (PCP) can mimic the symptoms
of schizophrenia in normal humans and exacerbate symptoms
in those with the illness, high doses can cause cell
death, possibly due to failure of downstream GABAergic
inhibition. We hypothesized that perinatal treatment with a
lesioning agent, especially one directed toward the NMDAsensitive
glutamate system, might result in long lasting
changes in cerebral systems and abnormalities in behavior
in an experimental animal and produce an informative
animal preparation for schizophrenia. We treated newborn
rat pups with high doses of PCP (10 mg/kg) and evaluated
behavioral and neurochemical measures of CNS response in
the grown rat. The rat pups were treated with a single dose
of the PCP or saline on days, PN7, PN9 and PN11 (three
treatments). All subjects were raised to PN42 without
further treatment, sacrificed at that time, and their brains
harvested for study. We used in situ hybridization to quantify
gene expression for zif268, arc, GAD67, and glutamate
receptor subunits (NR1, NR2A and NR2B) in this tissue.
Half of the animals were assessed on PN42 their locomotor
response to PCP (3 mg/kg) and for PCP disruption of
prepulse inhibition.
FP-N-017
Sleep-disordered-breathing and child behavior
J. Kohyama, W. Furushima, J.S. Ohinata, T. Hasegawa
Department of Pediatrics, Faculty of Medicine, Tokyo
Medical and Dental University, Tokyo, Japan
Aim: To assess behavioral problems in children with sleep
disordered breathing (SDB). Methods: Twenty-four children
with suspected SDB (M/F 19/5, age: 4–9 years
(mean, 5.5)) were studied. Their care-takers filled the Japanese
version of the child behavior check-list/4–18 (CBCL
(by Achenbach TM)). One-night polysomnography was
done for each child. The correlation coefficients (r) between
the three items on SDB (1. incidence of obstructive apnea of
more than 10 s per hour of total sleep time; obstructive
apnea index (OAI), 2. percentage of time with
SaO 2 , 90% against the total sleep time, 3. SaO 2 nadir)
and scores of ten scales on CBCL were calculated. CBCL
has 113 questions that were classified into eight categories
(I. Withdrawn, II. Somatic complaints, III. Anxious/
depressed, IV. Social problems, V. Thought problems, VI.
Attention problems, VII. Delinquent behavior, VIII.
Aggressive behavior), and caretakers selected one of the
three answers (0 ¼ not true, 1 ¼ somewhat or sometimes
true, 2 ¼ very true or often true). Internalizing
(I 1 II 1 III) and externalizing (VII 1 VIII) scales were
also scored. Results: Among thirty pairs of correlations
examined, five pairs showed a high r; OAI and I
(r ¼ 20:39, 0:05 , P , 0:1), VIII (r ¼ 0:36,
0:05 , P , 0:1), and externalizing (r ¼ 0:35,
0:05 , P , 0:1), and SaO 2 nadir and I (r ¼ 0:46,
P , 0:05), and internalizing (r ¼ 0:35, 0:05 , P , 0:1)).
Conclusion: The severity of SDB (elevation of OAI and
decrease of SaO 2 nadir) tend to correlate with decrease of
internalization and the increase of externalization in children
with SDB.
FP-N-018
Survey on the prevalence of autism in Japan
H. Shimoizumi, N. Iizuka, S.I. Saitou, M.Y. Momoi
International University of Health and Welfare, Otawara,
Japan
Objective: According to recent Japanese reports, including
one study documenting the prevalence of autism in

602
Abstracts
Yokohama at 21.1 per 10 000, the prevalence of autism in
Japan has increased. A survey was conducted to ascertain if
similar results would be obtained from a rural area of Japan.
The Nasu area is a region dependent on agriculture and
forestry, with a population of 214 323. Methods: Subjects
were 6–7-year-old children in the Nasu of Tochigi Prefecture.
Autism was diagnosed based on ICD-10 diagnostic
criteria. Results: A total of 4533 6- and 7-year-old children
(2287 boys, 2246 girls) were identified in the Nasu area in
1998. This population included ten autistic 6-year-old children
(six boys, four girls) and seven autistic 7-year-old children
(seven boys). The prevalence of autism was thus 37.5
per 10 000 (boys, 25.8; girls 17.8). Conclusions: Like the
recent Japanese reports, the results of the present survey of a
rural area demonstrated a high prevalence of autism. The
reasons could be two-fold: (1) The current diagnostic
criteria differ substantially from those used in the past;
and (2) As is the case of Nasu area, the number of falsenegative
cases is decreased in areas where early detection
and intervention systems are available for autism. The
prevalence of autism in Japan is higher than previously
reported, and improvement in prognosis for autistic children
through early detection and intervention are imperative.
FP-N-019
Relationship of social function and motor and speech
functions in children with autism
H.-C. Hsu, C.-L. Chen, C.-Y. Wu, C.-Y. Chung, M.-K.
Wong
Department of Physical Medicine and Rehabilitation,
Chang Gung Memorial and Children Hospital, Kweishan,
Taoyuan, Chinese Taipei
Autism is a pervasive developmental disorder of early
childhood characterized by severe impairment in reciprocal
social interaction, communication, and restricted repertoire
of activities and interests. This study investigated the relationship
of social function with motor and speech function
in children with autism. We collected 32 children with PDD
with the mean age 3.7 years. We used the Chinese Child
Development Inventory to assess the developmental functions
in 8 domains, including gross motor (GM), fine motor
(FM), expressive language (EL), concept comprehension
(CC), social comprehension (SC), self help (SH), personal
social (PS), and general development (GD). Children with
PDD were divided into 2 groups: group A (DQ of PS
.50%), and group B (DQ of PS ,50%) according to the
DQ of PS function. We compared the DQ between the two
groups. The correlations of different developmental functions
were analyzed by Pearson’s correlation. The PS function
was correlated with all the other seven functional
domains (r ¼ 0:6–0:9, P , 0:05), with the SC function
most highly correlated. While the PS function was not correlated
with the age, sex, and educational and economic status
of parents. Children in the group A had better developmental
functions than those in group B in all the eight domains.
Our findings suggest that autism is a multifacted developmental
disorder with impaired motor, speech and psychosocial
function. Therefore, a more comprehensive assessment
and transdisciplinary therapy are more appropriate in the
approach of children with pervasive developmental disorder.
FP-N-020
Evaluation of maturity in drawing in childhood in
neuropediatric consultation. A new test graphomotor
S.I. Pascual-Pascual
Service of Pediatric Neurology, Hospital infantile ‘LA
PAZ’, Madrid, Spain
Introduction: Tests used for measuring the ability of a
child to copy a drawing take more than 15 min and require
direct attention to the patient. This makes them almost
impossible to use in the neurological or pediatric consultation.
Objective: To present a new test of drawing copy, the
graphomotor test (GT). It consists of eight easy figures:
diamond, stair, cross, flower, clock, house, cube and
bicycle. The scoring method is simple. The maturation of
drawing in childhood and the reliability and validity of the
test are studied. Patients and methods: (A) Two studies
were done in order to assess the validity and reliability
of this test in normal childhood population of different
social (n ¼ 210) and economic (n ¼ 133) levels from 5
to 12 years of age, without selection. Drawings were
scored blindly by a neurologist and a clinical psychologist.
(B) A prospective study was made in children with varied
neurologic, mental and/or psychiatric diseases. All patients
of a neurology and psychiatric department completed the
test (n ¼ 349, aged 5–40), and repeated after 15–30 min. A
total of 276 were also studied with Wechsler test (WPPSI-
R, WISC-R or WAIS-R) and in other 53 cases the mental
level was known. The tests were scored blindly by a neuropediatrician
and a clinical psychologist. Results: (A)
Normal school children: The score system proposed is
easy to do and takes less than 1 min. The perfect test get
a score of 0, and the worst possible score is 20; Results
showed high reliability: interobserver Pearson’s correlation
coefficient r . 0:92, and reliability Cronbach alpha
coefficient ¼ 0.97; test-retest reliability r . 0:91 and
alpha ¼ 0.95.It is a valid tool to measure visuomotor
maturation in childhood. Average scores are different at
every age until 11 years. There is no difference in scores
between children of the two socioeconomic levels studied.
Results depend on mental or drawing level, not on the
socioeconomic status. (B) In children with neurologic,
mental or psychiatric diseases. The GT is a reliable tool
for measuring the visuomotor level of children with mental
levels above IQ 50: Cronbach’s alpha ¼ 0.98 (test-retest)
and alpha ¼ 0.98 (between different observers), and Pearson’s
‘r’ correlations .0.92 (P , 0:001). The repetition of

Abstracts 603
the test does not change the score. The GT is a valid tool
for measuring drawing maturation and the non-verbal intelligence
in childhood. Pearson’s correlations with verbal,
performance and total Wechsler test IQ (VIQ, PIQ and
TIQ) were significant (P , 0:01), the best of them were
with the PIQ and with the spatial capability factor of
Wechsler test (r ¼ 20:58 to 20.72, P , 0:001). GT is a
very sensitive screening test of the cognitive non-verbal
level of patients, with a high negative predictive value
(0.97). This allows selection of cases for further study
with neuropsychological tests. Conclusions: Graphomotor
test is a very useful tool to assess the drawing and performance
level of school children. It is simple, fast reliable
and valid in normal children and those with neuropsychiatric
pathology. It can be assessed in the same neurological
or pediatric consultation, while talking with the parents.
The results are related to the mental level and with age.
FP-N-021
Early identification of children with developmental
delay: an appraisal of 24 years’ experience in Hong
Kong
C.-W. Chan
Department of Paediatrics, The University of Hong Kong,
Hong Kong, China
Universal Developmental Screening of children below 5
years of age was first introduced in Hong Kong in April,
1978 as part of the White Paper on Comprehensive Observation
Scheme adopting the ‘Hong Kong Developmental
Screening System’ designed by the Department of Paediatrics,
The University of Hong Kong with data derived from
a cohort study on Growth and Development of 782 normal
Chinese children follow-up from birth to 8 years. Screening
Tests are provided free of charge at all Maternal and
Child Centres in Hong Kong to all preschool children and
to children at-risk, and at private paediatricians’ clinics.
Before 1987, tests were given at five key-ages from birth
to 5 years old but tests were re-scheduled to three stages: at
10 weeks, 9 and 36 months since 1987 for better compliance
and for more effective utilization of local resources.
At each key-age, each child is tested in major fields of
development: namely, gross motor, coordination, social
skills and language as well as hearing, vision, anthropometric
studies and physical examination. The average
coverage rate for local children is 84% per annum while
detection rate is about 10%. Children with abnormalities
identified were referred to paediatricians, relevant specialists
or to child assessment centres for confirmation of
abnormalities. All children with abnormalities were
comprehensively assessed and provided with appropriate
management and placement as arranged by the multidisciplinary
and interdisciplinary teams at Comprehensive
Child Assessment Centres.
FP-N-022
Evaluation of the crawling of disorders in brainstem
aminergic dysfunction; infantile autism, Rett syndrome,
and Down syndrome
K. Hoshino, J. Uchino, K. Hachimori, Y. Nomura, M.
Segawa
Segawa Neurological Clinic for Children, Tokyo, Japan
The brainstem aminergic neurons are known to have
important roles in functional development of the brain.
Ability and pattern of crawling reflect the development of
locomotion system modulated by the specific brainstem
aminergic neurons. To assess these neurons in infancy we
evaluated locomotion in correlating with the sleep wake
cycle (SWC), another biological phenomenon modulated
by these neurons in disorders of the aminergic neurons.
Method: Thirty-eight patients of infantile autism (IA), 42
RTT, ten Down syndrome (DS) were analyzed for crawling
and the development of SWC cycle in infancy and DQ/IQ
levels in childhood. Result: Crawling with normal pattern
was observed in 34.2% in IA, 17.5% in RTT, and none in
DS, and crawling with abnormal pattern (creeping, shuffling,
backward, etc.) were observed in 55.2, 30 and 70%,
respectively. A total of 10.5% of IA, 52.5% of RTT, 30% of
DS never crawled by the age of 12 months. IA showed delay
in development of circadian SWC in early infancy, whereas
RTT and DS showed delay in physiological decrease of day
time sleep after late infancy. The DQ/IQ were low (moderate
to severe delay) in most cases. Psychobehavioral disorders
were predominant in IA. Discussion: We previously
showed that locomotion during developmental period correlates
with the development of functional specialization of
the cerebral cortex and IQ level. The present study
confirmed the results and suggested that aminergic neurons
involved in locomotion are not those modulating circadian
SWC but are involved in reduction of day time sleep after
late infancy.
FP-N-023
Preschool screening and school performance: how
predictive is Denver II test?
B. Anlar, B.U. Bayoglu, F. Elibol, K. Yalaz
Hacettepe University Department of Pediatric Neurology,
Ankara, Turkey
The relationship between preschool developmental
screening and academic performance in the first school
year was assessed in a prospective design. Six year-old children
from two socioeconomically different areas of Ankara
were screened in the beginning of first grade with Denver II
developmental test standardized for Turkey. The results were
compared with school performance in the first grade. All
children with normal or questionable Denver II had satisfactory
school performance while 27% of those with abnormal

604
Abstracts
Denver II had low scores. Denver II results improved after 1
year at school, especially in children from low socioecenomical
status. Denver II appeared to have a good predictive
value in detecting potential school problems. These results
support screening children with disadvantaged backgrounds
at the beginning primary education, and providing supportive
programs in the 1st year at school.
FP-N-024
Using mismatch negativity (MMN) to assess auditory
discrimination and sensory memory in children with
dyslexia
R. Bombardieri, M. Terribili, L. Lopez, P. Curatolo
Department of Pediatric Neurology, Tor Vergata University
of Rome, Rome, Italy
Developmental dyslexia is a specific learning disorder,
characterized by a difficulty of decoding single letters,
generally due to insufficient phonological ability. Deficits
in phonological skills seem to be present in children with
reading disability but a topographic localization along the
auditory pathways is not yet identified. Using MMN, which
is an auditory event-related evoked potential, we want to
test the hypothesis that children with dyslexia have impairment
in auditory frequency discrimination. Twelve dyslexic
children (nine males, three female) and ten normal subjects,
matched for age and sex, were recruited. Mean age was 13.4
and 10.4 years, respectively. Children were submitted to
neurophysiological evaluation using EEG, BAEP, PEV
and ERPs (MMN). MMN responses were elicited using
two different tones (standard and deviant) and two different
conditions (‘ignore’ and ‘count’). EEG traces and evoked
potentials were normal in both groups. Data found an overlap
in 200 ms latency between the two traces (one obtained
using standard tones and the other using deviant tones) in
children with dyslexia. This could be due to attention is
activated in dyslexic children, even during ‘ignore condition’,
from both tones, as if standard tones were considered
‘new’ and worthy of attention. This result could be related to
an excessive discrimination of auditory stimuli, in dyslexic
children, and could be connected with an alteration in an
early station of the auditory pathway. Thus, MMN can be
used as a marker for the phonologic and mnemonic alteration
and can be a valuable tool for early diagnosis.
FP-N-025
A profile of pervasive developmental disorders in
Filipino children seen at the Philippine Children’s
Medical Center
E.L. Avendaño, M.A.G. Berroya, M.H. Ortiz, L.V. Lee
Child Neuroscience Division, Philippine Children’s Medical
Center, Quezon City Philippines
A total of 187 patients evaluated at the Neurodevelopmental
Section of the PCMC with a diagnosis of pervasive
developmental disorder using the diagnostics and statistical
manual (DSM III R and IV) criteria over a 10 year period
were included in the study. All but five were diagnosed to
have autistic disorder. There were more males than females
with as ratio of 5:1. Majority of the children were between 3
and 4 years old with a mean age of recognition at 1.86 years.
Mean interval between onset of symptoms and age of referral
was 2.67 years. Language delay was the most common
initial manifestation and reason for referral. In majority of
the patients, no definite underlying medical nor neurological
disorder was established. Autistic children displayed significant
impairment in the use of nonverbal behaviors, delay in
or lack of development in verbal language and preoccupation
with stereotyped and restricted patterns of interest.
Neurodevelopmental evaluation revealed marked delays in
expressive and receptive language as well as social adaptive
skills. Work-ups included EEG, BAER, cranial CT scan,
cranial MRI and urine metabolic screen which had low
positive results. Interventions included enrollment in a
special education (SPED) program, speech therapy, occupational
therapy and family counseling. The profile of Filipino
children with PDD in this study is comparable to that of
foreign data.
FP-N-026
Evaluation of cognitive functions in school-age children
with attention deficient with hyperactivity syndrome
M.B. Guryeva, O.I. Maslova, S.V. Balkanskaya, V.M.
Studenikin, M.A. Kirdyashkina
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific Center of Child Health (Russian Academy of
Medical Sciences), Moscow, Russia
Background: Attention deficit with hyperactivity
syndrome (ADHS) is nowadays one the main concerns in
pediatric neurology and psychiatry, since this condition had
been recognized to affect cognitive functions in children by
the time of school education. Aim: Our goal was to evaluate
the basic parameters of cognitive sphere in ADHS patients
of school-age. Method: Objective quantitative evaluation of
cognitive functions was performed in 45 patients (aged 6–10
year) with ADHS, utilizing the testing computer systems
(TCS ‘Ritmotest’, ‘Mnemotest’, and ‘Binatest’ of Russian
Manufacture). Forty-three normal children of identical age
were evaluated, as well, using the same diagnostic protocols.
Results: Evaluation of cognitive parameters in ADHS
patients mainly showed significantly compromised or
impaired functions in children under our observation in
comparison with their healthy compeers. The distribution
of deficient parameters was following: (1) attention
(86.6%); (2) short-term and/or long-term memory
(73.3%); (3) psychomotor activities (82.2%); and (4) analytic/synthetic
processes (80%). The results were expressed as
percentage for normal indices. The important finding is that

Abstracts 605
cognitive functions’ deficiency was more pronounced in
cases with hereditary (genetic) predisposition (ADHS in
families). Conclusion: The obtained results imply that
medicinal correction should be applied for cognitive deficit
correction in management of this condition. Hereditary
predisposition to ADHS seems to be as important as perinatal
damage, even though the precise genetic mechanisms
involved still are not completely understood.
FP-O
Psychology/Psychiatry
FP-O-001
Clinical characteristics of psychogenic diseases in
childhood
Z.-Y. Pei
The Yuncheng Central Hospital, China
Objective: To discuss the clinical characteristics and
diagnosis of psychogenic disease in childhood. Methods:
The clinical features of psychogenic diseases in 86 children
were analyzed. CT, MRI, EEG, ECG, CSF, CPK were
carried out. Results: Total 86 cases, 44 males, 42 females.
Among them, 62 cases lived in the cities, 24 cases lived in
the countryside. The onset of the disease was between 4.7
and 14 years old, and 81 cases (94.2%) were at the age of 7–
12 years old. Fifty-nine cases (68.7%) had known factors
which induced the diseases. Among them, 24 cases (27.9%)
were due to the heavy load of school work, 18 cases (20.8%)
due to the physical disease and six cases (7%) due to the bad
relation between their parents. The patients manifested as
paralysis in 23 cases (26.7%), convulsion in 13 cases, and
headache, syncope, sense disturbance, dizziness, visual
hallucination, etc., in the other cases. All the examinations
were negative, which-helped us to exclude other organic
diseases. Conclusion: The psychogenic diseases in childhood
were not rare. The heavy load of schoolwork is the
most important inducing factors, then the physical diseases
and the bad family relations. The clinical manifestation was
various, and it was likely misdiagnosed. So the pediatricians
should pay more attention to the psychogenic diseases in
children.
FP-O-002
Evaluation of visual perception and adaptive behavior in
children with Tourette’s syndrome
Z.-S. Liu, L.-Z. Yang, F.-L. Wang, J. Lin
Department of Child Neurology, Wuhan Children’s Hospital,
Wuhan, China
Objective: To explore the visual perception and adaptive
behavior of children with TS. Methods: The visual perception
and adaptive behavior of 38 children aged 8–12 years
(M 27, F 11) with TS were measured by means of Benton
Visual Retention Test (VRT) and Scale of Adaptive Behavior
for Children (SAB). Thirty healthy children were
selected as the control group. Results: All correct and erroneous
scores of VRT in the TS group showed no significant
difference compared to the control group (P . 0:05). Adaptive
behavior quotient and T score of cognitive function
factor of SAB in the TS group also showed no significant
difference compared to the control group (P . 0:05). T
scores of independent function factor and social/self-direction
factor of SAB in the TS group were significantly lower
than those in the control group (P , 0:01). Conclusion:
Children with TS had no visual perception deficit, and
possessed lower social adaptive ability.
FP-O-003
A diagnostic study and classification of attention-deficit/
hyperactivity disorder in children
H.-J. Zhu, X.-X. Wang, Q. Zhang
Department of Pediatrics, Zhejiang Provincial People’s
Hospital, Hangzhou, China
Objective: To evaluate the usage of the Children’s Attention
Tester and scales of ADHD in the diagnosis and classification
of ADHD in children. Methods: The authors tested
the attention of 380 ADHD cases aged 6.5 , 11 years with
NJ22 Children’s Attention Tester, and compared positive
rates of attention deficit test with the Children’s Attention
Tester and the DSM-IV criteria scales. The sensitivity and
the specificity of the instrument were analyzed. Results: The
ratios of the different types indicated that the predominately
attention deficit type was 57.1%, the predominately hyperactive-impulsive
type was 7.1%, and the combined type was
35.8%. The general positive rates of attention deficit in these
cases using Children’s Attention Tester was 78.4%. The
sensitivity and specificity of this instrument was 78.4 and
88.3%. The positive rates were not significantly different in
the children of different clinical types x 2 ¼ 0.78, P . 0:05,
but were significantly different in the children in whom
severity degrees was different x 2 ¼ 22.37, P , 0:01.
Conclusions: Conner’s questionnaire is a good screening
tool. The scale of ADHD by DSM-IV criteria is useful for
the diagnosis of the clinical type. The Children’s Attention
Tester can be the index for diagnosis of ADHD, and cannot
substitute of the scales.
FP-O-004
Anxiety of asthmatic children and salivary SIgA assay
L.-H. Wang, R.-P. Sun, H.-J. Zhang, M.-H. Zhang
Pediatric Department of the Second Hospital, Shangdong
University, Jinan, China
Objective: Assessment of anxiety in asthmatic children
and observation of the relationship between anxiety and
level of salivary serum immunoglobin A (SIgA), research-

606
Abstracts
ing the new method of improving asthmatic children’s
immune condition and relieving asthmatic attacks. Method:
Forty asthmatic children (asthmatic group) were tested with
Hamilton Anxiety scale (HAMA), and were tested by
measurement of skin resistance, salivary SIgA immunoradiometry
assay, and compared with 26 healthy children
(control group). Result: HAMA score was 12.03 ^ 6.01 in
the asthma group and 4.96 ^ 3.89 in the control group, the
asthmatic children had HAMA scores that were higher than
those of the normal controls (P , 0:01); Salivary SIgA level
in the asthma group was lower than that of in control group
(P , 0:01); skin resistance in the asthma group was higher
than that of the controls (P , 0:01). Salivary SIgA was
121.37 ^ 63.10 mg/ml in the asthma group with anxiety
and 208.23 ^ 166.33 mg/ml in the asthma group with no
anxiety, a significant difference being found (P , 0:05).
Skin resistance in the asthma group with anxiety was higher
than that of the asthma group with no anxiety (P , 0:05).
HAMA score in attack groups was much higher than in the
remission group (P , 0:01). Salivary SIgA and skin resistance
in the two groups were not significantly different
(P . 0:05). Conclusion: Compared with healthy children,
the asthmatics had more obvious anxiety disorders,
decreased humoral immune function, and increased function
of the parasympathetic nervous system. Humoral
immune function was lower in anxious asthmatic children.
Parasympathetic nervous excitement in asthmatic children
with anxiety was higher than in asthmatic children with no
anxiety. Anxiety in asthmatic children during an attack was
greater than that of asthmatic children in remission.
FP-O-005
Emotional disorders and related factors in in-patient
pediatrics
L.-H. Wang, R.-Q. Zhang
Pediatric Department of the Second Hospital, Shangdong
University, Jinan, China
Objective: To investigate the state of mental health of
pediatric inpatients. Methods: Thirty-four pediatric inpatients
were assessed with HAMA and Depression Status
Inventory (DSI), compared to 28 healthy children. Results:
HAMA scores were 14.91 ^ 6.10 in the inpatient group and
5.50 ^ 4.06 in the control group. The inpatients had a
HAMA score that was higher than that of the controls
(P , 0:01). The percent of the pediatric inpatients with
anxiety was 52.9%, higher than that of the controls
(P , 0:01). DSI scores were 53.01 ^ 6.75 in the inpatient
group and 47.14 ^ 6.07 in the control group. The DSI score
in the inpatient group was higher than the score of the
controls (P , 0:01). The percent of children with depression
in the two groups was not significantly different
(P . 0:05). HAMA score in female inpatients was higher
(P , 0:05); HAMA score in rural inpatients was higher than
that of urban inpatients (P , 0:05); HAMA score in inpatients
with a longer course was higher than in those with the
shorter course (P , 0:01). No significant difference was
found in the scores of depression in the pediatric inpatients
of different sexes, residence and courses (P . 0:05).
Conclusions: More obvious emotional disorders existed in
hospitalized patients than in the healthy children. The
degrees of anxiety were different in pediatric inpatients of
different sexes, residence and courses.
FP-O-006
Effects of INF on immune function in children with tic
disorder
Y.-H. Chen, W.-X. Chen, S. Chen, Y. Wang
Department of Pediatrics, Union Hospital of Fujian Medical
University, Fuzhou, China
Objective: To explore the efficacy and change in cell
immune function with complementary treating tic disorder
(TD) with IFN. Methods: An open-controlled study was
carried out. Forty children with TD were involved in the
study. Twenty of the total number of patients were treated
with routine medication (Haloperidol, Tiapiride, and clonazepam)
(routine group), the others were treated with Haloperidol
and IFN (1 £ 10 6 U/dose, i.m., qod) (IFN group).
The course of treatment was 8 weeks. There were 20 healthy
children (control group). The cell immune function was
measured by flow cytometry. Results: The cell immunological
function in children with TD was significantly
depressed, compared to that in healthy controls. At the
end of the research, the levels of lymphocytic subsets,
NKC in peripheral blood were improved in the IFN group.
The efficacy of the IFN group was 90%, which was significantly
better than the routine group (60%, x 2 ¼ 4.8,
P , 0:01). Conclusions: The present study demonstrates
that children with TD were at low cell immune functional
state, which may underlie the disease. IFN may be a new
effective and safe method for complementary treatment of
tic disorder.
FP-O-007
Consultation of parents of new cases with childhood
epilepsy
K.R. Bao, Z.P. Wang
Department of pediatric Neurology, Xin-Hua Hospital,
Shanghai, China
Objective: To find and resolve the psychological
problems in parents of childhood epilepsy who were
newly diagnosed. Methods: Eighty-two children (aged
from 6 months to 13 years) with a recent diagnosis of
epilepsy (within the previous 18 months) in our clinic
were followed up from January 1999 to December
2000.Their parents had consulted a child neurologist about
diagnosis, medical condition and prognosis for their

Abstracts 607
families. The ways of consulting a doctor were by appointment
interview, communication by letter, telephone and
giving parents a lecture. Results: (1) Parents’ attitude to
diagnosis: acceptant 75 cases, suspicious five cases, refused
two cases. (2) Parents’ attitude towards starting treatment:
acceptant 79 cases, postponed three cases. (3) Seizure
control after 1 year , 72 cases; 13 , 18 months six cases;
.18 months four cases. A significant relationship was found
between effective therapies and complying with doctor’s
orders. (4) The main psychological problems of parents
were fear of use of antiepileptic drugs and seizures which
would influence intelligence and other organ functions for
children. Conclusion: This investigation demonstrated that
psychological problems of parents of new cases with childhood
epilepsy were closely related to being submissive to
medical advice. Resolving these problems would help to
obtain efficacy of treatment. We took various steps to
cover many aspects for children with epilepsy. For example,
nutrition, movement, hobby out of class, nursing, learning
disability, etc. The purpose of our work was to open a road
to keep in touch on a regular basis with the parents of
children with epilepsy.
FP-O-008
Effects of dolphin assisted therapy (DAT) on children
who have some problems of communication
M. Ito a , M. Miyao a , K. Nihei a , K. Shirakawa b , Y. Sato b ,Y.
Sakai b , K. Fukusima c , S. Tomita c , S. Deguchi c , K. Kobari c
a Department of Neurology, National Center for Child
Health and Development, Tokyo, Japan; b Department of
Neuropsychology, National Children Hospital;
c Ocean
Wellness Foundation
Dolphin assisted therapy (DAT) is classed as one of the
animal assisted therapies. The treatment is for people who
have physical or mental problems which can be improved
by interaction with dolphins due to their charm and intelligence.
DAT is reported effective in improving patients
affected by depression, PTSD, Down syndrome (Bs
syndrome, and autism. We applied DAT to three children
(3–12 years old) who have some problems of their communication
at the Dolphin lagoon and in the playroom of the
Okinawa Marine Research Center in Okinawa Pref in Japan.
One boy, 12 years old, had physical and mental handicaps
due to sequelae of encephalitis. One boy, 8 years old, could
not take communications well with the schoolmate because
of ADHD. The other boy, 4 years old, had been treated
cruelly by the mother. Their families also participated. We
prepared clinically based DAT programs in conjunction
with the Department of Neurology of National Children
(Bs Hospital, and made comparative studies of the medical
and psychological condition of each case before and after
DAT. Consequently, DAT is effective in the improvement
of their communication through the interaction with
dolphins. Additionally the climate and natural environment
are important factors for reducing their stress.
FP-O-009
A contrasting model for deficit symptoms of
schizophrenia in leadership, with ‘sex-hormonal
dysgenesis’ in its pathogenesis?
A.G. Alias
Chester Mental Health Center, Chester, IL, USA
Associational loosening, impaired information processing,
poor gating of irrelevant stimuli, poor ability to shift
attention, poor working memory, passivity, ambivalence,
anhedonia, as well as impaired motor coordination are cardinal
features of schizophrenia but, unlike delusions and hallucinations,
they are related more to deficit symptoms. The
results of hundreds of studies during the past decades point
to a striking contrast between leadership and, say, schizotypy,
with the average human in between. Example: As
summarized by Bass (… Handbook of Leadership, NY,
Free Press, 1990), leadership correlated with ‘speed and
accuracy of thought’, ‘finality of decision’, ‘ambition, initiative
and persistence’, ‘mood control, and sense of humor’,
etc. A relationship between cognitive processes and cerebellar
and basal ganglia functions, and a role of neocerebellum
in rapidly shifting attention, have been demonstrated. The
cognitive styles, including a proficiency to quickly shift
attention, of history’s famous leaders can be telling examples:
Julius Caesar and Napoleon could dictate to up to six
secretaries simultaneously, using their exceptional working
memories, and proficiency in smoothly shifting attention
while flawlessly gating irrelevant external and internal
stimuli. I would suggest that specific brain imaging and a
variety of neuropsychological studies of accomplished,
younger, political and business leaders could illustrate this
contrast. Neurosteroids have profound effects on brain development
and functions. Though not scientifically rigorous, I
had noted positive correlations (P ¼ 0:0162) between the
self-rated ratings of voice depth (promoted by testosterone)
and of leadership, but none between those of body hair (dihydrotestosterone
dependent) and leader-ship in 47 male US
National Academy of Sciences members (Alias AG. Med
Hypotheses 2000;54:537–552), which was similar to what
Gray et al. (Psychosom Med 1991;53:375–385) found in
1709 older men.
FP-O-010
Clinical analysis and long-term follow-up survey for
Tourette syndrome
Q.-Y. Zhang
Department of Pediatrics, The 1stClinical College, China
Medical University, Nanjing Shenyang, China
Objective: To study the treatment and prognosis of Tour-

608
Abstracts
ette syndrome. Methods: Forty-three cases of Tourette
syndrome were followed up for 12–15 years. Results and
conclusions: (1) The symptoms were self limited and
abated or remitted greatly for most cases in late stage of
adolescence, and their study period and social adaptability
were normal. (2) The younger the patients, the more
serious the symptoms and prognosis. (3) Introverted
personality and relatively higher level of psychological
defense are the main characteristics of their personality.
(4) Mentality of most cases was normal. The intelligence
quotient for some cases was low. (5) The cases with
distractibility, hyperactivity, emotional disorders, obsessive-compulsive
neurosis, anxiety disorder, self-injury
behavior, mental confusion and abnormality of EEG had
a poor prognosis. (6) Severe cases should be treated by
drugs such as dopaminergic receptor blocking agent, the
mild cases or cases in remission stage can be treated by
main therapy with relaxation.
FP-O-011
The study of characteristics of sensitive children
W. Yu
Guangzhou Railway Central Hospital, Guangdong, China
Objective: To study the personality characteristics of both
inpatients and outpatients who were sensitive to infection.
Methods: Both the study group (120 cases) and the normal
control group (100 cases) were assessed with Jacson personality
questionnaire scale. Results: The E score was lower in
the study group than that in the control group (P , 0:01),
but the N score was higher in the study group (P , 0:05).
There were no significant differences in P and L scores in
both groups (P , 0:05). Sensitive patients showed introverted
and unstable characteristics of personality. Conclusion:
Frequently going to hospital would strongly influence
a sensitive child psychologically. It is necessary to carry out
early psychological intervention in sensitive children.
FP-O-012
RC Schumacher and society
N.C. Schumacher
Epilepsy Foundation, New Haven, CT, USA
Many of the problems faced by us are not understood.
Something as simple as why our dog may die, or as complex
as why our family is genetically inclined towards epilepsy
may be shrouded in mystery. One method of removing this
cloud is through education. This cannot be fully accomplished
until we tap the resources of those who suffer
from epilepsy and truly listen to what they themselves
have to tell us. As a society, we need to understand the
emotional problems they face by non-acceptance. Unless
society as a whole accepts the fact that being different is
not wrong, but an individual trait that we are blessed with,
they will fail to accept persons who have epilepsy as the
unique individuals that they are.
FP-O-013
Methylphenidate in children with attention deficit
hyperactivity disorder: evidence from a Sri Lankan
tertiary children’s hospital
S.H. Kariyawasam, H. Perera, A. Koralagama, P.
Jayawardane
University of Colombo/Lady Ridgeway Hospital, Sri Lanka
The response to methylphenidate (MPD) was assessed in
children diagnosed as having ADHD during the year 2000,
at the Lady Ridgeway Tertiary Hospital for Children in Sri
Lanka. They were managed in outpatient child psychiatry
clinics. ADHD was diagnosed according to DSM-IV
criteria and severity of the symptoms was determined
with a validated Sinhala assessment form based on the
DSM-IV criteria. The data on problems experienced by
the diagnosed children and their families was obtained
using an interviewer-administered questionnaire. The
severity of the symptoms, problems experienced by the
children and their families were reassessed at 6 weeks
and 6 months of MPD therapy. Thirty-seven new subjects
were diagnosed as having ADHD in year the 2000 and 36
of these were treated with MPD. Severity of symptoms and
the number of subjects receiving frequent complaints from
school was significantly lower 6 weeks after MPD treatment,
while frequency of other social problems was not
significantly reduced. At 6 months of treatment severity
of symptoms was not significantly reduced when compared
with the severity assessed initially and 6 weeks after. There
was no improvement in social problems at the end of 6
months of therapy. MPD did help with school-related
problems short-term, but the long-term effects of MPD
was not convincing in this group of subjects and showed
poor long-term compliance, probably due to inadequate
improvement seen in symptoms.
FP-O-014
Reassessing the efficacy of Adderall w after prolonged
treatment for attention-deficit/hyperactivity disorder
(ADHD)
P.A. Ahmann, L.R. Campbell, F.W. Theye, R.L. Berg, A.J.
Linquist
Marshfield Medical Research Foundation, Marshfield, WI,
USA
Objectives: (1) Assess response to Adderall w over time
in children 5–18 newly diagnosed for ADHD. (2) Describe
long-term behavioral efficacy of Adderall w by measuring
psychodynamic behavior in subjects in medicated and
unmedicated conditions. (3) Assess long-term safety of
Adderall w , especially growth effects. Method: Reassess

Abstracts 609
Adderall w responders after a median of 24 months of treatment.
Assess Adderall w response with Connors’ Parent and
Teacher Rating Scales; ADD-H Comprehensive Teacher’s
Rating Scale; plus parent, teacher, and child narratives.
Evaluate behavioral efficacy with Strengths and Weakness
of ADHD-symptoms and Normal-behaviors (SWAN) scale
completed on and off Adderall w . Assess height, weight at
routine visits. Results: Of 314 children completing initial
assessment, 271 (86%) showed positive response to Adderall
w . Of these, 133 enrolled in the reassessment study.
Seventy-three have currently completed reassessment,
with 47 (64%) demonstrating continuing response to
Adderall w . Thirty-six of 44 (82%) SWAN scores indicate
ADHD behaviors that worsened off medication
(P , 0:001). After a median follow-up of 2 years, the
first 44 children enrolled demonstrated highly significant
(P , 0:001) declines in growth relative to national percentiles:
Median height decreased 1.4 cm/year from the 65th
to 42nd percentile, median weight declined from 81st to
49th percentile, and median body mass index declined
from 77th to 59th percentile. Highly significant negative
correlation appeared between total dose/kg and rate of
change in height. No significant growth trend appeared
prior to therapy. Impact on growth was not significantly
related to dosing schedule. Conclusion: Adderall w response
and behavioral efficacy continue after prolonged treatment.
Clinicians should monitor growth carefully.
FP-O-015
Psychostimulants, amphetamine and
methamphetamine, are toxic to rat cortical neurons and
induce both apoptosis and necrosis
W.-T. Lee, Y.-Z. Shen
Department of Pediatrics, National Taiwan University
Hospital, Chinese Taipei
Psychostimulants, amphetamine and methamphetamine,
are both popular recreational drugs of abuse in many countries,
including Taiwan. Studies of amphetamine and
methamphetamine neurotoxicity showed the decreased
neurotransmitter levels and neurite degeneration, rather
than neuronal death. However, other studies also showed
that methamphetamine can lead to neuronal apoptosis.
Therefore, in the present study, we investigated the pathogenic
mechanisms of amphetamine and methamphetamine
neurotoxicity using primary rat cortical neuronal culture.
We found that both amphetamine and methamphetamine
induced dose- and time-dependent neuronal necrosis and
apoptosis. There was also time-dependent increase of free
radical production and decrease of ATP content in
neurons. Compared with amphetamine, methamphetamine
led to higher free radical production and ATP depletion,
and induced more necrosis and apoptosis in neurons. There
was also gradual increase of caspase-3 activity and
decrease of Bcl-2 expression following the application of
both amphetamine and methamphetamine. We conclude
that both amphetamine and methamphetamine can lead
to neuronal apoptosis and necrosis by a mechanism related
to mitochondrial dysfunction and free radical overproduction.
FP-O-016
Increased urine phenylethylamine after
methylphenidate treatment in children with attention
deficit hyperactivity disorder
Y. Yamashita a , A. Kusaga a , M. Keneko a , T. Koeda b ,M.
Hiratani1 c , S. Yamada d , T. Matsuishi a
a Department of Pediatrics and Child Health, Kurume
University School of Medicine, Fukuoka, Japan; b Faculty
of Education and Regional Sciences, Tottori University;
Tottori, Japan; c Hiratani Clinic for Developmental Disorders
of Children, Fukui, Japan; d Department of Psychiatry,
Saga Medical College, Saga, Japan
Objective: We measured urinary monoamines to clarify
the neurochemical metabolism, the pharmacological
mechanism of methylphenidate (MPH), and the correlation
between monoamine levels and clinical symptoms in children
with ADHD. Methods: After informed consent had
been obtained from the children with and without ADHD
and their parents, urine samples were collected over a 24 h
period. The urine levels of b-phenylethylamine (PEA),
MHPG, HVA, and 5-HIAA were measured; PEA by
GCMS and the others by HPLC. We investigated the correlation
between severity and the concentration of monoamines,
their difference between responders and nonresponders
of MPH, and their changes before and after
MPH treatment. Results: The urinary levels of monoamines
in 37 children with ADHD, 21 age-matched controls, 12
children with high function autistic disorder were
measured. The 22 children with ADHD were treated with
MPH, then divided into responders (n ¼ 18) and nonresponders
(n ¼ 4). The mean urinary levels of MHPG,
HVA, and 5-HIAA in children with ADHD were not
significantly different from those of controls. The PEA
levels were significantly lower in children with ADHD
(n ¼ 37, 21.7 ^ 20.5 mg/g creatinine) than in the agematched
controls (n ¼ 21, 46.6 ^ 46.6 mg/g creatinine).
No difference in urinary monoamines was found between
autistic disorder and controls or children with ADHD. PEA
levels in the responders to MPH significantly increased
after MPH therapy, however PEA levels in the 4 nonresponders
did not increase. No changes were found in
other monoamines after MPH therapy. No correlation
between the levels of PEA and ADHD severity was
observed.

610
Abstracts
FP-O-017
Clinical profile of attention/deficit hyperactivity
disorder among Filipino children in a tertiary referral
center 1987–2000
C.L.M. Cruz-Conducto, E.L. Avendano, A.L. Reyes, M.H.
Ortiz, A.L. Tanega, L.K. Ledesma
Neurodevelopmental Section, Child Neuroscience Division,
Philippine Children’s Medical Center, Quezon City, Philippines
Attention-deficit/hyperactivity disorder (AD/HD) is a
developmental disorder characterized by developmentally
inappropriate degrees of inattention, hyperactivity, and
impulsivity affecting 3–5% of school-age children. Charts
of 1355 patients seen over a 13-year period were reviewed.
Of these, 64 were diagnosed as having primary AD/HD. The
majority were in the early childhood age group with more
males affected (6:1). Language delay with heightened activity
level was the most frequent symptom in the combined
type while short attention span with language delay was o
the Inattentive type. AD/HD of the combined type was the
most common subtype (90%) across all age groups, while
AD/HD of the inattentive type was more frequent in adolescents.
The least common across all age groups was the
hyperactive-impulsive type. Diagnostic work-ups (EEG,
neuroimaging, thyroid function test, BAER) were normal.
Apart from the mental status examination, there were no
significant physical and neurological examination findings.
On neurodevelopmental examination, significant delay in
language domains (70%), fine motor (23%), and self-help
skills (7%) were noted. Neuropsycholoical evaluation
revealed cognitive functioning in the average range, delays
in the achievement test were noted in 44%. Co-morbidities
noted were developmental language disorder (95%), oppositional
defiant disorder (33%) and learning disability
(20%).
FP-O-018
A comparative study of the psychological characteristic
of epileptic and normal children
A. Aghaei
Islamic Azad University Khorasgan Branch, Iran
Objective: The purpose of this study was to compare the
psychological characteristics of epileptic and normal children.
Method: Fifty elementary school children (34 boys
and 16 girls) were diagnosed as epileptic on the basis of
neurological experimentation. These children constituted
the experimental group and were matched for sex, age,
and grade with 50 normal children (control group). A
demographic questionnaire, Child Symptom Inventory
(CSI-4), and Raven’s matrices were administered to both
groups. Both groups and their mothers were also interviewed
and all the necessary information was obtained.
Results: The results of multivariate of variance using
SPSS showed that a difference between the two groups
exists in the following characteristics: the history of family
epilepsy, gestation illness, type of birth (difficulty of delivery),
cyanosis, late talking, late urine control, enuresis,
fever and convulsion, attention weakness, hypomnesis,
sleep depth, social relations with peers, relation with
teacher, school interest, neglect of appearance, neglect of
personal affairs, jealousy, school GPA, school failure,
hallucination, ADHD, oppositional defiant disorder,
conduct disorder, generalized anxiety, voice tic, PTSD,
depression, autism and Asperger symptoms, nail-biting,
self-abuse and withdrawal at home.
FP-O-019
Psychological distress of families with children having
tics – 3 years experience of a community hospital
K.-W. Tsui, W.-C. Wong, M. Tse, K.-W. Li, K.-C. Chan
Department of Paediatrics, North District Hospital, Hong
Kong, China
Introduction: Tics are not uncommon and can be distressing.
Method: We retrospectively reviewed all patients
referred for tics from 1 July 1998 to 28 February 2002.
Data was retrieved from records, supplemented by telephone
interviews of parents with emphasis on psychological
distress at presentation and at the time of interview.
Results: Thirty patients (25 males, five females) with
median age at presentation ¼ 8 years (range: 4.5–14.5
years) and median follow up ¼ 20 months (range: 3–42
months) were recruited. Twenty-nine were successfully
interviewed. Median symptom duration was 3 months
(range: 1 month–8 years). Eleven (37.9%) had sought
two or more doctors’ advice before the first interview.
Nine patients (30%) had chronic motor tics and four
patients (13.3%) presented with vocal tics, one of which
had TS. Commonly affected muscle groups were eye blinking
(43.3%), head jerk/movement (40%), facial grimace
(23.3%) and shoulder shrug (20%). At presentation,
96.6% of parents were distressed (86.2% worried about
underlying physical illness, 31% felt embarrassed and
31% used negative comments towards children). After
consultations, their distress was alleviated (P , 0:01).
The patients with an urge to control tics also decreased
from 46.4 to 7.1% (P , 0:01). On follow up, parents
perceived a decrease in tic frequency, children being
happier and more confident in 86.2, 41.4 and 17.2% of
cases, respectively. Conclusion: Psychological distress
was not uncommon in these families. Reassurance and
counseling could reduce their distress and the potential
tension build-up between the parents and child.

Abstracts 611
FP-O-020
Clinical study of administration of fluvoxamine for
epilepsy patients with obsessive symptoms
J. Furusho a ,H.Sato a , K. Yamaguchi a , H. Ozawa b ,M.
Fukumizu c , T. Miyajima d , J. Kohyama e , Y. Iikura a
a School of Medicine, Showa University (at the present;
College of Literature, Department of Education Aoyamagakuin
University); b Tokyo Metropolitan Hachioji Children’s
Hospital, c Division of Child Neurology, National Center of
Neurology and Psychiatry; d Tokyo Medical University;
e Tokyo medical and Dental University, Japan
We performed a clinical study of administration of
fluvoxamine for epilepsy patients with obsessive symptoms.
Recently, the effectiveness of a selective serotonin reuptake
inhibitor (SSRI) has been reported in patients with
obsessive symptoms. Fluvoxamine is one of the SSRIs.
However, it has no indication for use in children under 20
years of age and patients with epilepsy in Japan because
there has been no report about their usefulness in children
and epilepsy. Therefore, we have established our own ethical
standards by referring to overseas reports, and we have
also performed a clinical study of administration of fluvoxamine
for epilepsy patients with obsessive symptoms at six
different institutions. We selected six patients in accordance
with our criteria: (1) more than two seizures per month; (2)
treated with antiepileptic drugs for at least 2 years; and (3)
having obsessive symptoms. A daily dosage of 25–100 mg
of fluvoxamine was administrated for at least 4 weeks under
their informed consent. Usefulness of fluvoxamine for
obsessive symptoms was defined using CBCL at 4 and 16
weeks after administration. Alleviation of psychiatry symptoms
was observed in two cases and clinical seizures were
decreased in two cases. No adverse effects (including
increase of seizure frequency) were observed. In view of
these results, fluvoxamine may be useful for psychiatric
symptoms even in pediatric patients with epilepsy.
FP-O-021
The importance of psychological testing in differential
diagnosis of childhood headache
I. Franula, I. Prpi, I. Vlaši-Cicvari, Z. Korotaj, E. Paui-
Kirini
University Children Hospital ‘Kantrida’ and Medical
Faculty of University of Rijeka, Rijeka, Croatia
The aim of the study was to establish whether neuropsychological
tests may be helpful in the differential diagnosis
of childhood headache. The retrospective results of
neuro-psychological testing of 54 school age children with
headache were analyzed. There were 18 boys and 36 girls
with a mean age 11.4 years (SD ¼ 2.6). No statistical difference
between the boys and the girls regarding age and social
background were detected. All tests were performed as soon
as a child was able to cooperate – on average 2 days following
the headache (minimum 0 days and maximum 9 days).
General IQ was average in 28 children, higher in 24 children
and lower in two children. The Bender-Gestalt visual-motor
perception test (Bender test) was normal in 28 children,
pathological (cerebral organic dysfunction) in 14 and inconclusive
in seven children. When applying IHS criteria for
headache differentiation we found that the majority of children
with migraine had a higher IQ (higher IQ in 15; average
in nine children) while children with other types of
headache were in the majority with an average general intelligence
(average IQ 19; higher IQ nine children). These
results were statistically significant (Hi2 ¼ 4.89,
P ¼ 0:02). Bender test was pathological in 14 children
with migraine and in one child with other types of headache.
Normal Bender test was found in four children with
migraine and 24 children with different types of headache.
These differences were statistically significant (Hi2 ¼ 21.9,
P , 0:01). Only children with migraine (six) had an inconclusive
Bender test. The results clearly show the importance
and reliability of neuro-psychological tests in differential
diagnoses of migraine and ’ordinary’ headache. In any
child with a characteristic headache attack who has a high
IQ and cerebral dysfunction on the visual-motor perception
test, migraine should immediately be suspected.
FP-O-022
Role of perinatal pathology in formation of attention
deficit with hyperactivity syndrome in children of
school-age
M.B. Guryeva, O.I. Maslova, V.M. Studenikin
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific Center of Child Health (Russian Academy of
Medical Sciences), Moscow, Russia
Background: Brain damage during prenatal and perinatal
periods of human development is believed to be one of the
principal factors in the formation of attention deficit with
hyperactivity syndrome (ADHS), as well as some genetic
mechanisms. Objective: The goal of our study was to determine
the role of pre-/perinatal pathology as a risk factor for
ADHS formation in school-age children. Method: Case
histories of 45 ADHS patients aged 6–10 years were thoroughly
analyzed (the cohort consisted of three groups in
accordance with DSM-IV classification: (1) ADHS
combined form; (2) ADHS with prevailing attention deficit;
and (3) ADHS with prevailing hyperactivity and impulsivity).
Results: Disturbances of physiological gestation course
took place in 32 cases (17.1%): severe toxemia, threatened
miscarriage, acute somatic conditions, potentially toxic
substances in the domestic environment etc. Pathology of
intranatal period was evident in 34 cases (75.5%): early
rupture of membranes (17.7%), inadequate labour activities
(20%), precipitated deliveries (24.4%). Seventeen neonates
(37.7%) were delivered preterm (gestational age ,36

612
Abstracts
weeks), and four babies post-term (GA .42 weeks). Among
term infants’ morpho-functional immaturity was present in
four cases, with intrauterine hypotrophy in 6.6% of patients,
and severe intranatal asphyxia in 12 pts (26.6%). Conclusion:
Our data provides evidence of high risk for ADHS
formation in children who experienced the influence of
pre- and perinatal pathology. The establishment of social
prognosis for pediatric patients with ADHS requires taking
into account the peculiarities of individual perinatal history.
FP-O-023
Nootropic drugs in therapy of the attention deficit
hyperactivity disorder (ADHD)
M.M. Lepessova, A.Kh. Jaxybayeva
Municipal hospital 7, Almaty, Kazakhstan
The purpose of our study was comparative analysis of
therapeutic effects from therapy by Instenon, a combination
of Instenon and Actovegin and routine form of therapy of
ADHD by Piracetam and Cavinton. During our investigation
we observed three groups of children with behavioural
problems such as ADHD. One group (20 children) had therapy
by Instenon, the second group had therapy by combination
Instenon and Actovegin, and the third group had
therapy by piracetam and cavinton. For assessment of effectiveness
of therapy we used neuropsyhological tests and
electroencephalography. During our investigation in all
the children we detected behavioural deviations such as
attention deficit, hyperactivity, impassivity, different speech
and motors delays. On electroencephalography we detected
slow posterior rhythm. After therapy by nootropic drugs we
obtained positive dynamics such as improvement in neuropsyhological
tests (cognitive and speech skills, visio-kinesthetic
perception, motor activity). In the first group we
observed positive dynamics in 12 (60%) children, in the
second group 15 (75%), and in ten of the third group
(50%). Therefore, for treatment of behavioral deviation
we recommended nootropic drugs, such as Instenon, or
Actovegin. However, treatment by combination of Instenon
and Actovegin is more effective.
FP-O-024
The investigation of social action characteristics in
hyperkinetic syndrome of childhood
D.-M. Wang, S.-M. Jia, S.-Y. Liu
No. 4 Hospital of Baotou, Baotou, Inner Mongolia, China
During the period 1998–1999, in order to understand the
characteristic of social adaptability of hyperkinetic
syndrome of childhood, we investigated the differences
between 46 children diagnosed as having hyperkinetic
syndrome of childhood and 50 normal children. Everyone
examines EEG which expresses normal EEG and critical
EEG. Their examinations of nerval system are normal.
Adopting ‘Wei’s measuring chart of child intelligence’
revised by Yao-Xian Gong and ‘The measuring chart of
child adaptable action’ compiled by Shu-Qiao Yao, two
groups of children were estimated by the intelligence quotient
(FIQ) and adaptation quotient (ADQ). Result: (1) There
is no significant difference in FIQ between the two groups
(100; 102, P . 0:05). (2) There is no significant difference
in ADQ between the two groups (105.4; 105.8, P . 0:05).
But the social/self control factor of the normal children is far
higher than one of sick children (57.6; 40.3, P , 0:05). The
independent factor and cognition factor of the normal children
is far lower than ones of the sick children (that is 52.4;
60.5, P , 0:05:49:3; 57.8, P , 0:05). Analysis: the sick
children is normal in FIQ and ADQ although independent
and cognition Factors maintain certain levels, they are low
value in social/self control Factor, which lead to pay no
attention to what you are doing. Likely, their studies and
lives are irregular. Their social action is unusual.
FP-O-025
A study on the effects of EEG biofeedback on the
cognitive function of ADHD children
R.-H. Jiang, Y.-F. Wang
Institute of Mental Health, Peking University, Beijing,
China
Objective: To study the effect of different sessions of
EEG biofeedback training on the cognitive function, especially
executive function of ADHD children. Methods:
Thirty-eight cases diagnosed as ADHD according to the
criteria of DSM-IV, aged 7–16 years, and 38 normal
controls well matched by gender and age with ADHD children
were involved in the study. Conner’s behavior rating
scale-parent questionnaire, Chinese-Wechsler intelligence
scale for children (C-WISC), Wechsler memory scale,
number cancellation test, Stroop test, CPT and Raven’s
standard progressive Matrices were administered pre and
post-EEG biofeedback treatment (20 and 40 sessions) and
after 6 months of treatment. No other interventions were
used to the ADHD children during the treatment. Results:
(1) Before treatment, ADHD children performed worse than
normal controls on all psychometric tests except picture
recollection, association memory in Wechsler memory
scale (WMS) and Stroop color-word test. (2) After 20
sessions of treatment, the scores of hyperactivity factor
and index of Conner’s behavior rating scale decreased
significantly. Number cancellation test, rate of omission
errors in CPT, visual reproduction, tactile memory, logical
memory, short-term memory in WMS improved significantly,
and were similar to normal children. Memory quotient,
digit span and Stroop word interference effect improved
significantly, but were not as well as normal children. (3)
After 40 sessions treatment, the scores of behavior and
learning factors and hyperactivity index of Conner’s behavior
rating scale decreased significantly. Memory quotient,

Abstracts 613
number cancellation test, Stroop word-interference effect
improved significantly, and was similar to normal children.
Instantaneous-memory and Stroop word test improved
significantly but was not as well as normal children. Performance
IQ, total IQ and B factor improved significantly.
There is no improvement on the Raven’s standard progressive
matrices. (4) Compared the ADHD children who
finished 20 sessions treatment only (20 session group)
with whom finished 40 sessions of treatment (40 session
group), 20 session group was better on clinical symptoms
before treatment, and improved more significantly than 40
session group. Before treatment, 20 session group
performed better than 40 session group on number cancellation
test, CPT. After 20 sessions treatment, 20 session group
performed still better on number cancellation test, CPT, and
worse on association memory. (5) On follow-up, reaction
time of CPT improved continuously, the others were similar
with post-treatment. Discussion: (1) Vigilance and sustained
attention, response inhibition, working memory, EF and
psychomotor speed of ADHD children started to improve
after 20 sessions of EEG biofeedback treatment. Vigilance
and sustained attention, response inhibition, non-verbal
working memory were as well as normal children. (2) All
of the cognitive functions improved continuously after 40
sessions treatment and were similar with normal children
except verbal working memory, planning and psychomotor
speed. (3) The effect was maintained when the treatment
stopped. Conclusion: (1) The EEG biofeedback was effective
both on clinical symptoms and cognitive function of
ADHD. (2) Cognitive functions began to improve after 20
sessions EEG biofeedback treatment, and improved
continuously after 40 sessions, but there is still some difference
on cognitive function between ADHD children and
normal controls, especially on executive function. The
effect was maintained and psychomotor speed improved
continuously when the treatment was stopped.
FP-O-026
Association of 5-HT 2A receptor T102C polymorphism
and attention deficit hyperactivity disorder in children
J. Li, Y.-F. Wang, Q.-J. Qian, B. Wang
Institute of Mental Health, Peking University, Beijing,
China
Objective: To detect the genetic association between
attention deficit hyperactivity disorder and a T-C polymorphism
at nucleotide 102 of the serotonin receptor
2A(5-HT 2A ) gene. Methods: A case-control study which is
based on 182 normal control and 323 children diagnosed
with ADHD according to DSM-IV criteria, along with a
transmit/disequilibrium test (TDT) which is based on 195
nuclear families is used to analyze the association of 5-HT 2A
receptor T102C polymorphism with the whole and all kinds
of phenotypes of ADHD in children. Results: As for the
patients of ADHD combined subtype, the genotype of
T102T is sparse (22.3 versus 33.5%, odds ratio,
OR ¼ 0.569, P ¼ 0:028, 95% CI 0.344 , 0.943) and the
genotype of T102C is excessive (64.0 versus 47.3%,
OR ¼ 1.987, P ¼ 0:003, 95% CI 1.264 , 3.124), when
compared with normal control, moreover, for the patients
of girl ADHD combined subtype, there is biased transmission
of the alleles of T102C polymorphism gene locus to the
probands. Conclusion: To ADHD combined subtype, the
genotype of T102T is a protective factor and the genotype
of T102C is a risk factor, besides, to the girl ADHD
combined subtype, the allele C102 is the disease-predisposing
gene.
FP-O-027
Study on developmental and circumstantial
characteristics of ADHD children with and without ODD
J. Liu, Y.-F. Wang
Institute of Mental Health, Peking University, Beijing,
China
Objective: To compare developmental and circumstantial
characteristics among ADHD children, ADHD/ODD children
and normal controls. Method: Based on DSM-IV
criteria, matched by age, sex and ADHD subtypes, we
sampled 34 pure ADHD cases, 34 ADHD with comorbid
ODD cases and 34 normal controls, and collected information
about their developmental and circumstantial features.
Results: (1) General information: compared with normal
controls, two case groups had significantly higher rate of
self-medical expense, but lower percentages of partial
state reimbursement, father with graduate education, father
being professional, mother with Han nationality and mother
being official. (2) Perinatal stage: two case groups had fewer
mothers with satisfactory mood during pregnancy, the new
born babies cried shorter immediately after their birth, but
more mothers got pregnant complications, mental stress in
late stage of pregnancy and work frustration than control
group; more mothers in ADHD group got too much weight
(more than 15 kg) than normal group; more pregnant
mothers in ADHD/ODD group got medication, drank and
had mental stress in early stage of pregnancy than control
group; ADHD/ODD group had more neonates with intracranial
bleeding than other two groups. (3) Development in
infancy and early childhood: two case groups had lower
rates of good physical development, and higher rates of
hyperactivity after 1 year of age; more children in ADHD
group had poor physical development than normal children.
(4) School life: two case groups had fewer children with
intimate peer relationship, or could maintain friendship for
more than 1 year, or could get helps from peers when having
difficulty, but more children had just a so-so peer relationship
or could last friendship for less than half a year. (5)
Relative factor for current behavior: the teachers or parents
had less efficacy for children’s behavior by way of oral
criticism in two case groups; additionally, they adopted

614
Abstracts
more measures of physical punishment, privilege abolishment,
ignorance, connivance or avoidance. (6) Health
history: more ADHD/ODD children had awkward in bold
movement than normal controls. (7) Living circumstances:
two case groups had worse parental relationship, and more
children were afraid of their fathers; there was higher rate of
harmonious parent-child relationship in normal group than
ADHD group, and that of the latter group was higher than
ADHD/ODD group; when meeting difficulty, families in
ADHD/ODD group had worse social support than the
other two groups. (8) Family history: the rate of positive
family history of mental disorder was higher in ADHD/
ODD group than ADHD and normal group. Conclusion:
Children with ADHD had disadvantaged developmental
and circumstantial features than normal children, particularly
ADHD children with comorbid ODD had many even
worse situations than pure ADHD children.
FP-O-028
A study on the cognitive function of attention deficit
hyperactivity disorder
Y.-X. Liu, Y.-F. Wang
Institute of Mental Health, Peking University, Beijing,
China
Objective: Our goal was to explore cognitive impairments
of ADHD children. Methods: Participants were children
referred for symptoms of over activity, inattention, and
impulsivity, and 94 children as normal control. Using
DSM-IV diagnostic criteria, we classified the 317 ADHD
children into three subtypes, and assessed comorbidities. All
participants received a series of neuropsychological tests
which consisted of C-WISC, WMS, the number cancellation
test, the Stroop test and the Raven’s standard progressive
matrices. Results: (1) Although children with ADHD have
normal intelligence, their C-WISC, WMS, the reverse digit
span scores were all less than their counterparts. In the
number cancellation test and the Raven’s standard progressive
matrices, ADHD children displayed worse than the
control. ADHD children spent more time on each part of
the Stroop test and made more effort to eliminate the interference
of word meaning; at the same time, they made more
errors on C part (P all ,0.05). ADHD girls had lower scores
on information subtest of C-WISC than ADHD boys
(P , 0:05). However, ADHD girls displayed a tendency
to be superior to that of their counterparts in the Stroop
test (P all ,0.1). There were no significant differences
between each subtype of ADHD children in neuropsychological
tests, though they were well matched by age and sex.
ADHD children combined with learning disability
(ADHD 1 LD) were significantly older than children
suffered from ADHD only (ADHD group). ADHD 1 LD
were significantly more impaired on the Freedom From
Distractibility factor (C factor) and comprehension subtest
of the C-WISC, the comprehension and reverse digit span
subtest of WMS, the number cancellation test (P all ,0.05).
(2) Logistic regression analysis showed that age and
decreased C factor were risk factors for combining with
LD. Gender, subtype and executive functions had little
effect on the onset of LD among ADHD children. C factor
significantly related to WMS, the number cancellation test,
the Stroop test and the Raven’s standard progressive
matrices. The highest relationships were found between C
factor and short term memory and memory quotient. The
middle relationship was found between C factor and the
number cancellation test. We did not find the discrepancy
of C factor with other factors in C-WISC. (3) The Stroop test
had little relationship with the C-WISC
(r ¼ 20:107 , 0:200). It suggested that the traditional
intelligence test did not assess the selective inhibition of
irrelevant stimulus or the control of impulse which were
assessed by the Stroop test appropriately. Full scale intelligence
quotient had little effect on variables standing for
executive functions. Conclusion: ADHD children have
impairments in tests of intelligence, memory, attention
and executive functions such as selective inhibition, working
memory and plan. Compared with ADHD boys, girls
with ADHD displayed a tendency of greater intellectual
impairment; however, their selective inhibition probably
maintained better than that documented in boys with
ADHD. Three subtypes of ADHD have the similar cognitive
model. When ADHD children grow up, maybe they would
combine with LD more easily. ADHD 1 LD children have
poorer C factor, working memory and attention level.
FP-O-029
A study of dopamine candidate genes and attention
deficit hyperactivity disorder
Q.-J. Qian, Y.-F. Wang, R.-L. Zhou
Institute of Mental Health, Peking University, Beijing,
China
Objective: To investigate the relationships between
ADHD and some candidate genes of dopamine system,
such as dopamine D4 receptor (DRD4) gene, dopamine
transporter (DAT) gene and catechol-O-methyltransferase
(COMT) gene in Han Chinese population. The distribution
of gene polymorphisms in ADHD phenotypes and different
genders, and potential gene-gene interactions were also
analyzed. Methods: The samples were comprised of 340
ADHD children, 224 unrelated controls and 202 integrated
ADHD trios (included proband and biological parents). The
diagnoses and subtypes were ascertained according to
American clinical diagnostic interviewing scales (CDIS), a
structured, interviewer-administered interview based on
DSM-IV criteria. The polymorphisms consisted of 48 bp
VNTR in exon 3 of DRD4 gene, 40 bp VNTR in the 3 0
untranslated region of DAT gene, the restriction fragment
length polymorphisms of Val158Met of COMT gene. Associations
of polymorphisms with ADHD and its subtypes

Abstracts 615
were examined by: (i) comparing cases and controls; and (ii)
using family-based association study in an extension of
TDT and haplotype-based haplotype relative risk (HHRR).
Results: (1) For the association analysis of 48 bp VNTR of
DRD4 gene with ADHD, the frequencies of alleles and
genotypes of long repeat tandem (4 , 6 repeats) in ADHD
boys were significantly higher than in the male controls. The
similar results were also obtained in ADHD-C subtype and
ADHD fulfilling ICD-10 criteria. But the frequency of long
repeat alleles was significantly lower in ADHD girls than in
female controls. The repeat numbers of 48 bp are 2 , 6, we
found no 7 or more repeat alleles among our samples. TDT
and HHRR revealed no preferential transmission of either
allele to ADHD probands. (2) For the 40 bp VNTR of DAT
gene, TDT analysis revealed preferential transmission of 11
repeat allele to ADHD-C probands and nine-repeat to
ADHD-I children. The frequencies of alleles and genotypes
of long repeat (11 , 12 repeats) in ADHD probands were
significantly higher than in the controls. The similar results
were also obtained in ADHD-C, pure ADHD subtype, and
ADHD fulfilling ICD-10 criteria. (3) In the family-based
association of COMT gene in ADHD, TDT and HHRR
suggested the association of COMT gene and ADHD
boys. The low enzyme activity Met158 allele preferentially
transmitted to ADHD boys. This association was particularly
marked among pure ADHD boys, especially the
ADHD-I subtype. The case-control study revealed that
high enzyme activity Val158 allele were more frequently
in ADHD girls fulfilling ICD-10 and DSM-IV criteria than
in the female controls. (4) Binary logistic regression analysis
with the sample of refined phenotype showed that long
repeat genotypes of DRD4 gene, DAT gene, and male
gender were risk factors to ADHD. There was gene-gene
interactions between DAT and DRD4 genes. In the sample
of pure ADHD, there was gene-gene interaction between
COMT and DAT genes. Conclusions: (1) The molecular
genetic mechanisms of ADHD display gender difference.
(2) The length polymorphisms of genes influence ADHD
liability. (3) There are gene-gene interactions which are
associated vulnerability to ADHD.
FP-O-030
The difference of attention-deficit disorder with or
without hyperactivity: a 1 H-magnetic resonance
spectroscopy study
L. Sun a , Z. Jin b , Y.-F. Zang a , Y.-W. Zeng b , G. Liu b , Y.-F.
Wang a
a Institute of Mental Health, Peking University, China;
b Center of fMRI, Hospital 306, Beijng, China
Objective: Using proton magnetic resonance spectroscopy
( 1 H-MRS) to investigate the possible neurometabolic
difference among the predominantly inattentive subtype
(ADHD-I subtype), the hyperactivity-impulsive subtype
(ADHD-C subtype) and normal controls. Method: Proton
spectra were acquired bilaterally on the globus pallidus in
20 schoolboys having ADHD and ten matched controls. The
boys having ADHD were divided into ADHD-C subtype
group (n ¼ 10) and ADHD-I subtype group (n ¼ 10)
according to DSM-IV criteria. The peaks of N-acetylaspartate
(NAA), choline moieties, myo-inositol, creatine (Cr)
and a-Glx were measured and their ratios to Cr were calculated
and compared. Results: (1) Though the NAA/Cr ratios
in both ADHD groups were lower than the value in normal
controls, however only the ADHD-C subtype group showed
a significant difference, not the ADHD-I subtype group. (2)
In the right globus pallidus, the NAA/Cr ratios in the
ADHD-C group was significantly lower than the ADHD-I
group. In the left globus pallidus, the NAA/Cr ratios in the
ADHD-C group also showed a decreased tendency
compared to the ADHD-I group. Conclusion: Since NAA
is a useful marker of neuronal function, these findings
suggest that the basal ganglia neuronal dysfunction exist
in ADHD children, the neuronal dysfunction of the
ADHD-C group was more severe than that of the ADHD-I
group.
FP-O-031
Response inhibition in two subtypes of children with
ADHD in a stop signal task
Y.-H. Wang a , X.-L. Zhou a , Y.-F. Wang b , Y.-X. Zhang a
a Department of Psychology, Peking University, Beijing,
China; b Institute of Mental Health, Peking University, Beijing,
China
Objective and method: A stop signal task was used to
investigate two kinds of response inhibition, response
conflict and response stopping. Subjects were two subtypes
of children with ADHD (predominantly inattentive and
combined) and normal controls. Results: The results
showed that ADHD children were deficient in both kinds
of response inhibition compared with normal controls. In
response stopping, it was more difficult for ADHD children
to withhold response when responding with left hand,
which means that they have significant deficit in the right
hemisphere. No significant differences were observed
between the combined and inattentive ADHD group. In
response conflict, normal children did not show significant
conflict effect, suggesting that their strong conflict control
ability can overcome the mild conflict manipulation. The
inattentive ADHD group showed a significant conflict
effect when responding with left hand, suggesting the
impairment in control functions of the right hemisphere
and the anterior cingulated. The combined ADHD group
were more impaired than the inattentive ADHD children,
showing a larger conflict effect when responding by either
left or right hands. Conclusion: This suggested that the two
types of ADHD children were impaired to the different
extent in neurocognitive functions.

616
Abstracts
FP-O-032
Effects of distractors on sustained attention in children
with attention-deficit hyperactive disorder (ADHD)
Y. Xu a , X.-L. Zhou a , Y.-F. Wang b
a Department of Psychology, Peking University, Beijing,
China; b Institute of Mental Health, Peking University, Beijing,
China
Objective and method: Using an experimental design
combining the sustained attention task (CPT, SART) and
the flanker task, we investigated: (1) whether children
with ADHD have deficits in their sustained attention; (2)
whether distractors have different effects on the response to
targets at different sustained attention levels; and (3)
whether different subtypes of ADHD children show different
patterns in sustained attention. Results: The results
support the view that ADHD children have deficits in
their sustained attention, reflecting the deficits in brain
development. More importantly, this study found that the
effects of distractors in sustained attention can be dissociated
according to the level of demand on sustained attention:
distractors interfered with responses to targets when
the demand on sustained attention was low while they facilitated
responses to targets when the demand on sustained
attention was high. There were no significant differences
between ADHD-inattentive type and ADHD-combined
type in their deficits in sustained attention. Conclusion:
ADHD children have deficits in their sustained attention,
reflecting the deficits in brain development.
FP-O-033
Investigation of ADHD comorbidities in a Chinese
clinical sample
L. Yang, Y.-F. Wang
Institute of Mental Health, Peking University, China
Objective: To investigate the comorbid rate between
ADHD and other childhood psychiatric disorders and
analyze the influence of gender and age in a Chinese clinical
sample. Method: The study included 423 ADHD children
consecutively diagnosed by specialists at the Institute of
Mental Health, Peking University. All subjects were
assessed by structured diagnostic interviews with parents
for comorbidities based on DSM-IV. Results: The comorbidity
of recruited ADHD children with disruptive behavior
disorder is 35.1% (ODD 28.2%, CD 6.9%), with multiple
anxiety disorder 8.3%, mood disorder 10.1%, Tics disorder
(including Tourette syndrome, TS) 14.4%, and learning
disorder 37.9%. Girls with ADHD were more likely than
boys to have the anxiety disorders (P , 0:001), especially
the special phobia (P , 0:05) and the general anxiety disorder
(P , 0:01). In addition, adolescents with ADHD (12–16
years) were more likely than children (6–11 years) to meet
the criteria for mania (P , 0:05), and less likely to have
chronic tics disorder (P , 0:05) and learning disorder
(P , 0:001). Conclusions: The high likelihood for ADHD
children to comorbid other psychiatric disorder makes it a
clinical problem that should not be neglected. It is necessary
to routinely evaluate, diagnose and manage the comorbidities
in clinical work. The sex and age differences of comorbidity
implicated different pathogenisis.
FP-O-34
The investigation of social action characteristics about
hyperkinetic syndrome of childhood
D.-M. Wang, S.-M. Jia, S.-Y. Liu
No. 4 Hospital of Baotou, Aogan Str., Qingshan District,
Baotou 014030, Inner Mongolia, China
To understand the characteristic of social adaptability of
hyperkinetic syndrome of childhood, we had carried out the
investigation between diagnosed hyperkinetic syndrome of
childhood 46 and normal child 50 in 1998–1999. Everyone
examines EEG which expresses normal EEG and critical
EEG. Their examinations of nerval system are normal.
Adopting (Wei’s measuring chart of child intelligence)
revised by Yao-Xian Gong and (The measuring chart of
child adaptable action) compiled by Shu-Qiao Yao. Two
groups of children were estimated intelligence quotient
(FIQ) and adaptation quotient (ADQ). Result: (1) There is
no significant difference in FIQ between the two groups
(100; 102, P . 0:05). (2) There is no significant difference
in ADQ between the two groups (105.4; 105.8, P . 0:05).
But the social/self control Factor of the normal children is
far higher than one of sick children (57.6; 40.3, P , 0:05).
The independent factor and cognition factor of the normal
children is far lower than ones of the sick children (that is
52.4; 60.5, P , 0:05:49:3; 57.8, P , 0:05). Analysis: the
sick children is normal in FIQ and ADQ although independent
and cognition Factors maintain certain levels, they are
low value in social/self control Factor, which lead to pay no
attention to what you are doing. Likely, their studies and
lives are irregular. Their social action is unusual.
FP-O-035
Multidisciplinary Tourette syndrome clinic
V. Gross-Tsur, A. Zohar, M. Sadeh, F. Benarrouche
Neuropediatric Unit, Shaare Zedek Medical Center, POB
3235, Jerusalem 91031 Israel
Tourette’s syndrome (TS) is a multifaceted disorder characterized
by waxing and waning tics, both motor and vocal.
ADHD, learning disabilities, OCD and other psychiatric
phenomena are strongly associated with the syndrome. In
order to address the multiple handicaps of children with TS,
our patients are treated by a multidisciplinary team of pediatric
neurologists, psychiatrists, clinical and experimental
psychologists and a social worker. In this presentation we

Abstracts 617
report the clinical characteristics and presenting signs in 60
children (49 boys and 11 girls, ages 9.5 ^ 3.0 (mean ^ SD).
Age of presentation was 5.3 ^ 1.5 years, the full syndrome
(vocal and motor tics for 1 year) became apparent at the age
8.7 ^ 2.3 while the diagnosis was made about 1 year later.
Tics were the presenting sign in 57% of children: 30% had
motor tics, 10% vocal tics and a combination of motor and
vocal tics were seen in the rest. ADHD was the presenting
problems in 30% while OCD was rarer, the presenting sign
in only 3% of children. The first motor tics were eye blinking
(38%) or other facial tics (37%) and the first vocal tics
were throat clearing (53%). During their illness, complicated
motor tics were found in 53% and copralalia in 5%.
Although learning disabilities, interpersonal problems and
behavioral disorders were not presenting symptoms in TS,
they were very prevalent appearing in 65 and 58% respectively.
OCD and ADHD were also very common, occurring
in three quarters of the children. Therapy was multimodal,
58% of TS children received medication and 72% psychological
therapy sometime during their illness. The familialgenetic
element was evident in that family history of TS was
found in 10%, tics in 20% and OCD 6.6%. In conclusion, we
found that the data of our TS patients as a group is very
similar to that of TS worldwide despite the differences in
cultural background and the known heterogeneity in the
characteristics of individuals with TS.
FP-O-036
Clinical features in adult attention-deficit/hyperactivity
disorder among substance abusers
Y. Ding, Y.-F. Wang
Institute of Mental Health, Peking University, China
Objective: The present study is conducted to explore the
clinical presentations, psychiatric comorbidities and
psychosocial functioning in adults with ADHD among
non-alcohol substance use disorder population. The authors
reason that if the adult diagnosis of the disorder is a valid
clinical entity, it should be similar to the childhood ADHD
with regard to patterns of psychiatric findings. Methods:
Thirty adults with ADHD and thirty non-ADHD controls
were selected from Beijing Compulsory Detoxification
Center, matched with sex, age, and education. Data collection
included the following: (1) self-report measures of
developmental history, employment history, social history
and ADHD clinical symptoms. (2) Psychiatric interview
using structured assessments included the CDIS for DSM-
IV. (3) C-WAIS for adult used to evaluate the cognitive
function. Results: (1) The mean age of ADHD group was
28.97 (SD ¼ 5.90), and the mean age of control group was
29.33 (SD ¼ 6.18). There was no significant difference in
age (P ¼ 0:815). (2) The clinical core symptoms (such as
attention deficit, restlessness, and impulsive symptoms)
among adulthood ADHD were similar the childhood
ADHD except for some hyperactivity symptoms. (3) The
ADHD subjects were more likely to be with current or past
(childhood) DSM-IV symptoms of ADHD (attention deficit,
restlessness, and impulsiveness) than control subjects
(P , 0.05). (4) ADHD subjects were more likely to diagnosed
with comorbid ODD (60 versus 20%, P ¼ 0:008), CD
(76.7 versus 23.3%, P , 0:001) and APD (60.0 versus
3.3%, P , 0:001) than that in non-ADHD controls. There
was no excess risk for anxiety and affective disorder in
ADHD subjects than control. Furthermore, adult ADHD
group was more likely to having an early onset of age of
non-alcohol substance use disorder (23.53 versus 25.48,
P ¼ 0:069). (5) There was no differences of marital status
and occupation between the two groups. There was no
significant differences in speeding violation, licenses
suspended, crashes between the ADHD group and the
control group. The frequency of drunk driving in ADHD
subjects was significantly higher than that in the control.
Conclusions: (1) The similarities and persistence of
ADHD symptoms from childhood to adulthood collaborate
the validity of the ADHD diagnosis for adults. (2) Adult
ADHD prefer to have more antisocial behaviors including
ODD, CD, and ASPD. (3) It is difficult to distinguish the
psychosocial functioning impairment between ADHD and
substance use disorder.
FP-P
Medical Informatics
FP-P-001
Neurologic: a web-based tutorial for teaching an
anatomical approach to the neurological examination
P.D. Larsen a , S.S. Stensaas b
a University of Nebraska School of Medicine, Nebraska
Medical Center, Omaha, Nebraska, USA; b University of
Utah School of Medicine, Salt Lake City, UT, USA
The first principle of neurological diagnosis is regional or
anatomical localization. A physician must learn the
elements of the neurological examination and how to logically
and systematically interpret the findings in order to
localize the site of neurological disease. In order to teach
these skills we have developed a web-based tutorial that is
available on the Internet for medical schools and neurology
training programs. The tutorial has seven modules: an introductory
module and six modules based on the components
of the exam (mental status, cranial nerves, coordination,
sensory, motor, and gait). Each module is divided into
four sections. (1) Review of anatomical pathway(s) and
structures being examined; (2) video demonstration of that
portion of the exam; (3) Videos of patients showing corresponding
abnormalities of that portion of the exam; and (4)
self-evaluation: At the end of the tutorial there are unknown
cases for the student to practice on. The tutorial is designed
for use on a fast (T1) Internet connection, not a modem. It is
highly interactive and uses progressively downloaded

618
Abstracts
QuickTime movies. Written text is kept to a minimum and
ease of navigation within the site is a priority. Movies come
with a £ 2 option so that they can be projected in the classroom.
The site lends itself to being adapted to the teaching
needs of classroom instruction as well as self-study. The
tutorial will be demonstrated and discussed at the meeting.
FP-P-002
Can a distance learning programme provide effective
training in paediatric neurodisability?
V.A. Harpin, C.R. Pullen, H.A. Davies, S.M. Gentle, D.M.
Hall
Sheffield Childrens Hospital, Sheffield, UK
Training in paediatric neurodisability in the UK was felt
to be patchy and difficult to obtain for many trainees. This is
incompatible with an increasing clinical need. In Sheffield
we have developed a 2 year distance learning programme to
provide core training in a co-ordinated way. Twenty
Students began the Course in September 2000, supported
by 14 tutors. The Course consists of 12 written modules (one
every 2 months) and four residential courses with local
practical visits. Trainees are evaluated by a written assessment
per module and three further assessments. Trainees
evaluated each module for usefulness and level of teaching
(1–6) and each residential session for relevance and enjoyment
(1–5 scale). Tutors are also providing feedback. Overall
feedback has been very positive. The course is felt to be
demanding and thorough. Most students consistently feel
material is at the right level and useful/very useful. Individual
students find different modules most useful/hardest
depending on experience and their own needs. Residential
sessions scored consistently highly. Teaching content
scored very good to excellent throughout and the chance
to meet other trainees and tutors was highly valued. Eighteen
of 20 students remain on the course. One is retraining in
child psychiatry. One has deferred to the next group because
of personal reasons. We will present details of course development
and evaluation. A distance learning course can
provide a good core training in neurodisability and we
feel this kind of format could be used by other specialties
and in other countries.
FP-Q
Oriental Medicine
FP-Q-001
Acupuncture and facilitation technique in the treatment
of cerebral palsy in children
J.-G. Cao, X.-Z. Guo, H.-Y. Lu
Faculty of Pediatrics, Shanxi Medical University, Taiyuan,
China
Objective: To explore the effect and influential factors of
acupuncture and facilitation technique in CP children.
Methods: A total of 246 CP were treated by acupuncture
and facilitation technique. Movement function was evaluated
before and after treatment. Results: Ninety-five cases
had significant effect, and 127 cases had effect. Among
them, 34 cases were normalized. The effective rate was
90.2%. Curative effect is related to the age of starting treatment,
sustained course, type, severity of diseases, and
complications. Conclusion: Combined acupuncture with
facilitation technique can promote rehabilitation in CP.
The earlier and the longer the treatment, the better will be
the effect.
FP-Q-002
Successful prevention of relapse in a child with multiple
sclerosis using oriented herbal medicine (Sairei-to)
S. Yamazaki, H. Yoshikawa, T. Abe
Department of Pediatrics, Niigata City General Hospital,
Niigata Japan
Since the age of 3, a 9-year-old Japanese girl developed
recurrent aseptic meningitis, optic neuritis, brainstem
dysfunction and convulsions. At the age of 4, she suffered
from aseptic meningitis and optic neuritis, and brain MRI
revealed high intensity lesions in the white matter on T-2
weighted images. The titers of anticardiolipin antibody was
also elevated. She was diagnosed as having MS associated
with positive anticardiolipin antibodies. In each episode of
MS relapse, the administration of corticosteroids was effective
in stopping the symptoms. However, corticosteroids
had no preventive effects for MS relapse. At age 6, with
the informed consent of her parent, we tried using the oriental
medicine, Sairei-to to prevent MS relapse. Following
this, over a period of two years she experienced no relapse
of MS. Some oriental herbal medicines have been reported
as being effective for MS therapy. Seirei-to has anti-inflammatory,
immunoregulatory, anti-allergic, diuretic, and steroid-like
effects, it has also been reported that Sairei-to is
also effective against antiphospholipid antibody syndrome.
These pharmacological properties could work in the prevention
of MS relapse of this patient, who had a positive anticardiolipin
antibody. Sairei-to is considered to be a
worthwhile trial in the prevention of MS relapse.
FP-Q-003
Study on antiepileptic action and immunological
mechanisms of CaoGuo ZhiMu Tang
H. Zhang, L. Wang
Department of Neurology, Baotou Central Hospital,
Baotou, China
Objective: On the basis of ‘fu zheng gu ben’ of Chinese
traditional medicine, the related immunological mechanisms
of the antiepileptic effect of CaoGuo ZhiMu Tang

Abstracts 619
(CGZMT) were studied from gross, cellular and molecular
levels with audiogenic seizure (AGS) rat model. Method:
First, ten Wister rats and 30 AGS rats were randomly
divided into four groups and were peritoneally injected
NS10 ml/kg, CGZMT 10 g/kg, phenobarbital (PB) 50 mg/
kg, respectively. Rats were treated continually for 7–14
days. We measured the antiepileptic effect. Second, the
immunological mechanisms of the rats were studied by
the colorimetry, QHS, 3H-TdR incorporation, MTT analysis
colorimetry. Results: Comparing with model control,
CGZMT could enhance activity of macrophage (MF)
(P , 0:05); accelerate biological activity of IL-2
(P , 0:05). It could enhance proliferation of T lymphocyte
stimulated by concanavalin-A (ConA) (P , 0:05), but its
proliferation of T lymphocyte to ConA was lower than
that for normal control rats (P , 0:05). It could increase
RBC-C3bRR and RBC-ICR and lower proliferation of B
lymphocyte stimulated by LPS. It could also decrease the
levels of antibody forming cell and hemolysin antibody.
Conclusion: CGZMT had antiepileptic effect. It could accelerate
the activity of MF, cell immune function and RBC
adhesion function. This suggested that one of its antiepileptic
mechanisms is improving immune state of the body, i.e.
‘fu zheng gu ben’.
FP-Q-004
Clinical study on efficacy of traditional Chinese medicine
bushen combined with special training program on
mental retardation (MR)
F.-Z. Du, Y.-J. Dou, J.-F. Ding
Rehabilitation Center, Gansu Traditional Chinese Medical
College, Lanzhou, Gansu Province, China
Objective: To observe the effect of traditional Chinese
medicine (Bushen) combined with special training program
on MR. Method: Patients of MR were divided into two
groups (A and B) according to age. Group A (N ¼ 105)
received special education in the assistant study school.
Group B (N ¼ 42) were treated with western medicine,
such as Vitamin B1 and lysine. Group A was randomly
divided into two subgroups (A1 and A2). The 55 cases of
group A1 were treated with Bushen medicine-Xingnaoyizhi
granule (XNYZG) 10 g per dose, which consisting of herbs
including lujiaoshuang, roucongrong, and tianzhuhuang,
etc. This drug was taken orally three and two times a day
in patients above and below 12 years, respectively for 3
months. The 25 cases of group B1 were treated with
XNYZG 5 g per dose, three times a day for 3 months.
Groups A2 and B2 were control groups. IQ and clinical
data were collected before and 3 months after treatment.
Results: The rate of improvement was 21.8% in group A1
and 12.0% in group B1.The rate of improvement was 87.3%
in group A1 and 60.0% in group B1. Both were significantly
higher than in the groups A2 and B2 (P , 0:01). The rate of
improvement in group A1 was significantly higher than that
in the group B1 (P , 0:01). Conclusion: Although there is
no effective method to treat MR, traditional Chinese medicine
Bushen combined with special training program may
be regarded as an acceptable method for treating MR.
FP-Q-005
Effect of ‘Xi Feng Jing Ning Decoction’ on blood
dopamine and immunological function in Gilles de la
Tourette syndrome
Z.-Y. Yu, J. Wang
Department of Pediatrics, China-Japan Friendship Hospital,
Beijing, China
Objective: To study the mechanism of ‘Xi Feng Jing Ning
Decoction’, a traditional Chinese medicine decoction
consisting of 12 kinds of herbs, in treating Gilles de la TS.
Methods: Forty TS were divided into two groups, Xi Feng
Jing Ning Decoction treatment group (XF group, 30
patients) and control group (C group, ten patients). A clinical
observation table was set up to compare the changes
before and after 60 days of treatment. A comparison was
made in the change of blood DA level and IgA, IgG before
and after 60 days of treatment. Results: The response rate in
the XF group was 93.3%. There was no significant difference
between the two groups (P , 0:01). The blood DA
content in both groups was lower after treatment
(P , 0:01). IgG and IgA of the XF group were increased
(P , 0:01) after treatment, but those of the C group had no
increase (P . 0:05). Conclusion: Xi Feng Jing Ning Decoction
can relieve the symptoms and adjust the DA systems
and immunological status of patients with TS. The Decoction
can also decrease attacks of upper respiratory tract
infection and reduce the possibility of TS relapse. This
showed that the traditional Chinese medicine had a promising
future in treating TS.
FP-R
Neurosurgery
FP-R-001
A case report about melanotic neuroectodermal tumor
of infancy in skull
N. Wang
The children’s hospital of Zhejiang, Hangzhou Zhejiang,
China
This article reports a rare tumor named ‘melanotic
neuroectodermal tumor of infancy (MNTI)’. MNTI is
found more than 200 cases in the world from 1918. Our
case is a male 4 months child. There is a growing mass on
his left temple. After CT and MRI examining, it is diagnosed
‘meningioma’. But it is MNTI in pathology. We
check this tumor with immunohistology, and review it in
literature. This case is the fifth in China, and is the first

620
Abstracts
occurred in frontal bone. Generally, MNTI is low grade
malignity. Our case has not recurred about 13 months
after operation. Our conclusion is that thorough operation
is the most important thing in MNTI’s therapy.
FP-R-002
Children’s tuberous sclerosis associated with brain
tumor
H.-M. Jin, L.-P. Sun, N. Bao, J.-M. Zhu
Department of Pediatric Surgery, Xin Hua Hospital, Shanghai
Second Medical University, Shanghai, China
Objectives: To explore the diagnosis and treatment of
TSC associated with brain tumor in children. Clinical materials:
Six tuberous sclerosis complexes, four male, two
female, 4–12 years old, have been confronted during last
10 years. CT and MRI scans showed brain tumors located in
lateral ventricle near foramen of Monro. Four were in left
and two in right. Tumors were excised completely in three
cases, partially in two patients. The pathologic findings were
subependymal giant cell astrocytoma. One child who had
concomitant renal tumor accepted gamma radiotherapy.
Results: All cases were followed 1–7 years. Three children
are disease-free after complete tumor removes. One of two
patients with partial resection died of recurrence, and
other’s residual tumor size does not increase. One tumor
has not changed after gamma radiation. Conclusions: TSC
associated with brain tumor can be well diagnosed by CT
and MRI. Craniotomy is the only effective treatment. The
patients with TSC should be followed in order to detect and
intervene brain tumor earlier.
FP-R-003
Microsurgical treatment for hypothalamic hamartoma
causing epilepsy in children
C.-D. Li, S.-Q. Luo, Z.-Y. Ma, Y.-Q. Zhang, Q. Bai
Department of Neurosurgery, Tiantan Hospital, Beijing,
China
Object: To discuss the surgical treatment for hypothalamic
hamartoma causing epilepsy in children. Methods: Six
boys and five girls with epilepsy and hypothalamic hamartoma,
age ranged from 2 to 15-years-old, were reported. The
onset ages were 2 months–14 years. Ten patients presented
with gelastic seizure, six of them also with generalized
tonic-clonic seizures and three with drop attacks. All
patients were treated by microsurgery via a pterional
approach except one via a laminaterminalis approach and
four patients underwent depth electrode encephalography
with electrodes implanted into the hypothalamic hamartoma.
Result: Two hamartomas were totally removed without
any complications, eight hamartomas were gross
partially removed and one was partially removed. All
patients were followed by 16–87 months after operation,
two were completely seizure-free, nine were significantly
reduced the frequency of seizures. Conclusion: With
removement of hamartoma we can effectively treat epilepsy
caused by hypothalamic hamartoma in children.
FP-R-004
Pediatric craniopharyngiomas: surgical treatment and
protection of the hypothalamus
Y.-Q. Zang, C.-C. Wang, Z.-Y. Ma, S.-Q. Luo
Department of Neurosurgery, Beijing Tiantan Hospital,
Beijing, China
Object: With the protection of the hypothalamus,
craniopharyngiomas can be in children safely removed,
total or subtotal. Methods: A total of 142 pediatric cases
were operated with the transcallosal-interseptal-interforniceal
approach, the transfrontal-longitudinal fissure
approach, and other approaches. The methods of protection
and treatment of hypothalamic functions were used
before and after the operations. Results: A total of 133 out
of 142 tumors (93.7%) were removed totally and subtotally.
The complications were diabetes insipidus 129
(90.8%), hypernatremia or hyponatremia 127 (89.4%),
and seizures 4 (2.8%). However, the seizures were fatal
complication. One patient (0.7%) died postoperatively due
to seizures. Conclusion: For total or subtotal removal of
the pediatric craniopharyngiomas, it is important to protect
the hypothalamic functions before, during and after the
operations. The tumors should be operated directly under
a microscope.
FP-R-005
Evaluation of mild closed traumatic brain injury in
children with CT scan: prospective international study
A. Murgio a , D. Fong b , E.J. Herrera a , G. Hotz c , F. Romeo d ,
D. Rocco d , S. Mutluer e , A.F. de Andrade f
a Quintana 1256 – Mar del Plata (7600), Argentina; b China;
c United States; d Italy, e Turkey, f Brazil
Introduction: Traumatic brain injury (TBI) is the leading
cause of death and disability in pediatric trauma. Although
the incidence and mechanism for TBI in children 0–15
years of age varies through the developmental levels,
early diagnosis and treatment may ameliorate the morbidity
and mortality associated with significant intracranial
injury. Objectives: The principal idea was to evaluate,
using an international and multicenter population, the relationships
between severity of injury, risk factor and
imaging findings by attending physicians. Methods: The
present study was a multicenter prospective, randomized,
study of children who serially presented to emergency
departments with head injury. The organizing group and
administrator study is the International Study of Head
Injury Project, which was created in 1996. A total of

Abstracts 621
9460 children were seen between 1996 and 2000, and it
sample was divided in two groups: Phase I (1996–1998)
with 4690 patients and Phase II (1999–2000) with 4770
patients. Each child was seen by medical staff and evaluated
during acute care. A standardized data base sheet was
completed to include general demographics, medical
status, Glasgow Coma Scale score and Pediatrics Score,
neuroradiologic study. Indicators consisted in frequency
counts and tabulations. On follow-up each subject was
given a Glasgow Outcome Score and asked set standardized
questions considered detect functional level and
quality of life. The baseline information and subsequent
outcome score were compared and evaluated. Results: A
total 9460 children, we observed not statistical difference
between two phases, by number and sex. Boys made up
60% (Phase I) and 61.8% (Phase II) of the sample. The
distribution by age was: birth to 2 years (77.1%: first phase
and 40.8% at the second phase); 3–9 years (18.7 and 46%,
respectively) and 10–15 years (4.1 and 13.2%). Use of the
GCS-PGCS score to rate severity of the injuries indicated
that 79.1% were milds TBI (Phase I) and 96.4% (Phase II).
P ¼ ns. The most frequent mechanism of injury was a fall,
which reported in approximately 70% in a two Phases, road
accidents accounted for 20% (Phase I) and 16.8% (Phase
II), the mechanisms in the remaining 10% hit by object,
crush injury, and others. By comparison, head X-rays were
performed in 89 and 88.6% of the children, 89% of the first
group giving normal and 85.1% with 453 (10% ¼ Phase I)
and 629 (14.9% ¼ Phase II) pathologic results. With
respect the CT scan that was ordered, we found difference
between the two groups: 14.3% in Phase I and 53% Phase
II, were 65% of which were normal with 35% labeled
‘abnormal’ and the second phase 71.4% were normal and
the remaining 28.5% labeled ‘abnormal’. Seven children
died (0.15%) at the first phase and three (0.06%) of the
second phase, and 81 underwent some neurosurgical intervention
(1.7% Phase I) and 130 (2.7% Phase II). Most of
the children 91% of the Phase I and 99.8% (Phase II) were
re-evaluated at the follow-up after 3 months. The vast
majority 99 and 99.7% had scores of 5, i.e. good recovery.
Each center participating demonstrated the same tendency
for the vast majority of children to get good recovery
ratings at the local follow-up assessment. Conclusion:
The advantage of the multicenter study is that allows us
a glimpse of practice in varied settings and makes it possible
to compare these experiences with our own. The
present study seems to suggest that some of the beliefs
that govern us in decision-making need review, for example:
the use of ‘older and familiar’ technologies (X-rays) to
determine the need for a more complex evaluation, including
CT. Until more definitive information is available, clinicians
should be liberal in their use of CT so that early
identification of significant intracranial pathology can be
obtained and appropriate management of the injuries
initiated.
FP-R-006
Is a separate cerebrospinal fluid access device useful in
the management of childhood hydrocephalus?
T.-Y.M. Lo, L.M. Myles, R.A. Minns
Department of Child Life and Health, University of Edinburgh,
Edinburgh, UK
A retrospective study was undertaken to determine the
long-term risks and benefits of a separate CSF access device
in the management of 52 patients with shunted childhood
hydrocephalus. We compared the usage and complication
after separate reservoir insertion with a pre-reservoir period.
The risks were assessed in terms of the frequency of: (1)
ventriculoperitoneal (VP) shunt infection and ventriculitis;
(2) shunt blockage; (3) access device failure; and (4) other
potential complications such as porencephaly, epilepsy,
hemiplegia, visual and cognitive deficits. The benefits of a
separate CSF reservoir were gauged from the attendances
where the reservoir was accessed to diagnose, treat or
exclude, raised intracranial pressure (ICP) or shunt infections
and ventriculitis. There was no mortality from raised
ICP or CNS infection over a median follow-up period of
19.05 years. Significantly fewer episodes of ventriculitis
(P ¼ 0:0091) and shunt blockage (P , 0:0001) were
found in the post-reservoir study period. There was no hemiplegia,
epilepsy, visual or cognitive loss from the additional
cortical puncture. The reservoir was used for access in 344
attendances for diagnosis or treatment of raised pressure or
CNS infection. We conclude that a separate CSF access
device is very useful in the long-term management of
patients with shunted hydrocephalus and is without mortality
or significant morbidity.
FP-S
Diagnosis/Treatment
FP-S-001
Different dosage regime of penicillamine and urine
copper for hepatolenticular degeneration
Z.-C. Wen, R.-M. Wang, W.-X. Sun, X.-Y. Wang
Shandong Provincial Hospital, Jing Wu Road 324, Jinan,
China
Objective: To determine the dosage of penicillamine in
treating patients of hepatolenticular degeneration. Methods:
Penicillamine with different dosages are administrated to
patients with hepatolenticular degeneration, group 1
(N ¼ 17) small dose (5 , 10 mg/kg per day); group 2
(N ¼ 17) using moderate dose (11 , 16 mg/kg per day).
The followings are monitored: extrapyramidal symptoms,
liver function, hypersplenism, average amount of daily
urine copper of 8 weeks; and observe side effects (including
fever, white blood cell, skin rash, nausea). Results: (1)
Copper excretion in the moderate dose group was better

622
Abstracts
than the small dose during the first 6 weeks of treatment
(P , 0:01). (2) After 6 weeks, the copper excretion effects
of the two groups were the same (P . 0:05). (3) The side
effects of the two groups were not different. (4) The clinical
symptoms were identical with above three conclusions.
Conclusions: We recommend using moderate dose of penicillamine
in treating patients with hepatolenticular degeneration
in the acute stage (the first 6 weeks) and small dose
after acute stage (after 6 weeks).
FP-S-002
Comparison of topamax with valpronic acid,
carbamazepine and phenobarbital in the treatment of
childhood epilepsy
Y.-Q. Zhong, J. Wu, M. Wu, X.-L. Xie, W.-G. Hu, W.-Z.
Zhou
Chengdu Children’s Hospital, Sichang, China
Objective: To compare the efficacy of adverse reactions
of TPM, VPA, CBZ and PB in children. Method: Single
drug regimens were used to treat 194 newly diagnosed
cases of epilepsy in a prospectively designed non-randomized
parallel control trial (TPM 63, VPA 61,CBZ 34 and
PB 36 cases). The dosage of TPM was 0.5 , 1 mg/kg per
day initially, increased gradually to 2 , 8.5 mg/kg per day
later. The median follow-up duration was 12.2 months.
Therapeutic efficacy and adverse reactions of the drugs
were closely observed during follow-up period. Result:
Complete control of epileptic attack was observed in 58.7,
57.4, 50 and 52.8% of cases in TPM, VPA, CBZ and PB
groups, while unfavorable results were seen in 6.3, 4.9, 2.9
and 8.3%, respectively. The original regimens had to be
discontinued in 19.1% cases owing to unsatisfied efficacy,
untoward reaction or economic reason. The untoward drug
reaction incidence of TPM was similar to CBZ and PB, but
slightly higher than VPA, whereas the severity of which
seemed to be the least among the four drugs. Conclusion:
The use of TPM alone in the treatment of childhood
epilepsy showed similar efficacy to other commonly used
drugs, while its untoward reaction was relatively mild.
FP-S-003
Family rehabilitation on 15 children with cerebral palsy
Chun-Xiang Li, Xue-Mei Liu, Ning-Bo Tang, Qing Chu, Li-
Xia Li
Department of pediatrics, Yantai Yuhuangding Hospital,
Sandong Province, Yantai China
Objective: To study the effect of family rehabilitation on
children with cerebral palsy. Methods: The destination and
scheme of rehabilitation were drawn up after 15 children
with cerebral palsy had been evaluated systematically.
Rehabilitation education and operation training were carried
on to family trainers. Training contents included proper
posture of holding in the arms and lying and training of
exercise and speech. The patients were followed up once
a month, whereas for those outside this city were followed
up once every 2 months. When the patients were seen again
they were evaluated again, their treatment effects were
observed and the training scheme corrected if necessary.
All cases were followed-up 1 year. Results: Three cases
were nearly normal, ten cases were effective and two
cases ineffective. Conclusions: Family rehabilitation is an
effective, economic, and easy method for children with
cerebral palsy.
FP-S-004
An open trial of flunarizine on therapy in children with
migraine
C.-Y. Zeng
First Hospital, Guangzhou Medical College, Guangzhou,
China
Objective: To assess the efficacy and safety of flunarizine
(sibelium) in children with migraine. Methods: Flunarizine
was administered to 30 children with migraine, 5 mg every
night for 2 months–1 year. Results: The effectiveness was
20% after 1 week, 50% after 4 weeks, 80% after 12 weeks,
TCD abnormalities were improved in 18 cases after 3
months of therapy. Conclusion: Flunarizine is an effective
therapeutic drug in children with migraine.
FP-S-005
Efficacy of topiramate (TPM) in refractory childhood
epilepsies
L. Cvitanovic-Sojat a , B. Mucic-Pucic a , S. Miskov b ,Z.
Sabol c , T.F. Hajnžić a , M. Mataija a , T. Sojat a
a Departments of Pediatrics and b Neurology, UH ‘Sestre
milosrdnice’, c Outpatient Clinic ‘Dr. Sabol’, Zagreb, Croatia
Objective: To analyse the efficacy and tolerability of
TPM in children with refractory epilepsies. All patients
treated with TPM had first failed trials of conventional antiepileptic
drugs, some of them were treated with LTG, VGB,
GPB, corticosteroids and IV globulins. Subjects and methods:
We performed a retrospective study of 35 PTS patients
(14 girls and 21 boys). The age range was 3, 5–17 years in
32/35 and 8–10 months in 3/35. In 22 patients, epilepsy was
complex partial, in two myoclonic, five had Lennox-Gastaut
syndrome, multiple seizure types in three children and three
had infantile spasms (IS). Epilepsy was symptomatic in 17/
35 and idiopathic/cryptogenic in 18/35. TPM was administrated
as add-on therapy, it was titrated to target dose of 6–
9 mg/kg per day. The duration of follow-up was 6 months–3
years. During the titration and maintenance phase we evaluated
the seizure frequency, side effects and EEG in all
patients, and visual evoked potentials in 20/35. Results:

Abstracts 623
Seventeen/35 patients became seizure-free, among them
one had IS. A seizure reduction .75% was observed in 6/
35 patients, between 50 and 75% in 4/35, less than 50% in 4/
35. Two/35 patients had seizures only during febrile illness
and two patients with IS had their seizures unchanged.
Twelve/35 patients were switched to monotherapy from
add/on therapy. Side effects noted were transient change
in appetite in 2/35, hyperactivity in 1/35, and hypochidrosis
in 2/35. Conclusion: This report indicates that TPM was
well tolerated, no serious side effects were noted. TPM
was effective as add-on and monotherapy in refractory
childhood epilepsies.
FP-S-006
Treatment of self-induced photosensitive epilepsy with
dopamine agonist
M. Horikawa a , R. Iwanaga b , E. Ohtaki c , T. Yamada a ,K.
Tominaga a
a National Higashi-Saga Hospital, Saga, Japan;
b Asou
Iizuka Hospital, Iizuka, Japan; c Koguma Gakuen, Ogouri,
Japan
We report a 9-year-old girl with self-induced photosensitive
epilepsy in which bilaterally synchronous 3–4 Hz
spike and wave discharges (SWDs) were induced by twirling
of her hands in front of her face while facing the sun.
She drooled in an absent-minded manner after turning her
hands round and round, and on a few occasions she showed
a transition to generalized tonic-clonic spasms. Selfinduced
photosensitive epilepsy is notoriously resistant to
therapy. We used dopamine agonist. A total of 5 mg
bromocriptine mesilate, three times/day, was added to the
previous medication (valproate 1 carbamazepine). After
that, she lost consciousness less frequently and became
unable to develop generalized convulsions although the
hand movements persisted. However, the SWDs induced
by the hand movements were still observed on EEG. It has
been reported that high-frequency photic stimulation in
cats significantly reduces the cortical release of dopamine
and that this is not a phenomenon restricted to the visual
cortex. It is thought that decrease in cortical release of
dopamine during photic stimulation, as observed in cats,
is probably a crucial physiopathological step in epileptic
photosensitivity in man.
FP-S-007
Topiramate add-on or monotherapy in infantile spasms
D.-C. Jiang
Children’s Hospital, Dalian, China
Objective: To study the effect, dosage, and adverse
events of TPM in treating infantile spasms, either used as
monotherapy or an add-on therapy. Methods: Eleven
patients with infantile spasms were treated with TPM to
a course of 4–13 months. Seven cases were used TPM as
monotherapy. The patients were started at a dose of 12.5
mg per day, which was increased by 12.5 mg every 3 days.
Four cases used TPM as add-on therapy, but they were
substituted by VPA and clonazepam for undesirable
control. The clonazepam was used at a dose of 0.66 mg/
day and increased gradually. Results: (1) Three gave up
treatment due to the side effect and unknown reason. (2)
There were a 50% or greater reduction from baseline in
seizure frequency shown in 91% cases, and seven cases
showed no free seizure including four cases using TPM
and clonazepam. (3) Adverse reactions: one patient had
impairment of verbal language, and another failed to
sweat in hot days. Conclusion: TPM is an effective and
safe drug used as monotherapy or with clonazepam in
infantile spasms.
FP-S-008
Immunoregulation and therapeutic effect of biological
agent in intractable childhood epilepsy
Z. Xu, X. Tian, J.-Y. Lan, D.-F. Sun
Department of pediatrics, the second clinical college of Jilin
University, Changchun, China
Objective: We treated intractable childhood epilepsy
(ICE) with biological agents, IL-2 and IV IG, and investigated
their immunoregulatory and therapeutic effects.
Methods: 28 children with ICE were treated with IL-2
and IV IG. Before and after treatment, serum IgA, IgG,
SIL-2R, IL-6, T-cell subsets and anti-brain anti-body
(ABAB) were examined, respectively. According to
frequency of seizures, changes of EEG and improvement
of immunological parameters, the immunoregulatory effect
and therapeutic effect of IL-2 and IV IG were evaluated.
Results: (1) The immune state: the serum IgA levels in
epilepsy group were significantly lower than those in
controls (P , 0:01). SIL-2R and Serum Il-6 levels in
epilepsy group were significantly higher than those in
controls (P , 0:01). The CD 4 /CD 8 ratio in epilepsy group
was significantly lower than those in controls (P , 0:01).
The positive rate of ABAB in epilepsy group was 35.7%
(10/28). The parameters after treatment were statistically
significant difference compared to pretreatment (P , 0:01
or P , 0:05). (2) Therapeutic effect: the patients had been
followed up for 0.5–2 years. The improved rate of EEG
was 82.2%. To assess the parameters together, the markedly
effective rate was 53.5%, effective rate 35.5% and
total effective rate 89.2%. Conclusion: (1) The patients
with ICE may present with disturbance of immune function.
(2) IL-2 and IV IG have immunoregulatory effects on
the immune abnormalities in patients with ICE. (3) Biological
products, IL-2 and IV IG, have therapeutic effect for
ICE.

624
Abstracts
FP-S-009
Study on the diagnosis of childhood paroxysmal
headache
G.-Q. Li, H.-W. Hu, X.-Y. Ye, Z.-D. Lin, J.-W. Zhao, D.-K.
He
Yuying children Hospital, Wenzhou Medical College, Wenzhou,
Zhejiang, China
Objective: To investigate the differential diagnosis of
childhood paroxysmal headache. Methods: Clinical data of
122 patients with paroxysmal headache in our hospital
outpatient service were analyzed. The case history, physical
examination, EEG video electroencephalography (V-EEG),
and transcranial Doppler (TCD) were analyzed. The patients
were followed up. Results: Migraine was found in 85 of the
122 patients, tension headache in 35, headache epilepsy in
one, and intracranial tumor in one. Most EEG and V-EEG
was normal. There was increase of diffuse slow wave, and
only one of above abnormal EEG showing scattered epileptic
waves. In 74 patients, TCD showed significant increase
of cerebral blood flow velocity. Conclusion: Migraine and
tension headache were frequent cause of childhood paroxysmal
headache. The diagnosis should depend on the clinical
manifestation, EEG and TCD findings. Headache
epilepsy is rare. We need to differentiate headache epilepsy
from paroxysmal headache only.
FP-S-010
Choice of anti-epileptic drugs for epilepsy caused by
hypoxic-ischemic encephalopathy
S.-W. Yang, H.-L. Yu
Department of Pediatrics, Xianning Central Hospital, Xianning,
China
Objective: To improve the quality of life in patients with
epileptic caused by HIE, to select suitable AED and to
improve control the epileptic attacks. Methods: Retrospective
analysis was performed on 46 cases to detect the different
effect of different anti-epileptic drugs (AED). Results:
Among 19 cases of partial seizure treated with CBZ,
seizures were completely controlled in two cases, in six
other cases the seizures decreased more than 75%. Of the
27 cases of generalized seizure, only three out of 21 who
were treated with PB alone were completely controlled, and
two cases had decreased seizure frequency more than 75%.
In six cases of infantile spasm, five were treated with CZP,
resulting in none being completely controlled or decreased
his seizure more than 75%. One case was treated with Topamax
had its seizure frequency decreased more than 75%. Six
out of 8 cases of partly seizure treated with the addition of
Topamax were completely controlled and two other cases
were decreased their seizure more than 75%. Similar results
were achieved by adding Topamax in both generalized
seizure and infantile spasm. Conclusion: Topamax is an
ideal anti-epileptic medicine to treat secondary epilepsy
caused by HIE.
FP-S-011
Long-term response to zonisamide monotherapy in West
syndrome
Y. Suzuki a,b , H. Ueda a,b , Y. Toribe a,b ,K.Imai a ,T.
Matsuoka a , T. Mano a , T. Nagai a
a Collaborate Clinical Research Group for Pediatric
Neurology, Osaka University Medical School; b Division of
Pediatric Neurology, Osaka Medical Center and Research
Institute for Maternal and Child Health, Izumi City, Osaka,
Japan
Aim: To determine the long-term efficacy and safety of
zonisamide (ZNS) monotherapy in patients with West
syndrome. Methods: In our previous study, ZNS (3–12
mg/kg per day) was administered to 54 newly diagnosed
patients with West syndrome (symptomatic 40). Overall,
11 of 54 infants (20%) had cessation of seizures and disappearance
of hypsarrhythmia. These 11 ZNS responsive
patients (symptomatic seven) were the subjects of this
study and eligible to enter a long-term follow-up period.
Results: During the follow-up period (24–79 months,
mean 53 months), this response was maintained in seven
patients (symptomatic three, relapse rate 36%). Seizures
recurred in the remaining four symptomatic patients; three
patients (patients 8—10) had relapses of spasms within 4–
10 weeks after cessation of seizures, and one developed
complex partial seizure 4 years after the initial suppression.
The seven sustained responders had favorable prognosis;
ZNS was discontinued without relapse in two, and five
had normal psychomotor development or only mild impairment
(DQ .70). No serious adverse reactions were noted.
Conclusion: ZNS may be effective and well tolerated for
patients with West syndrome.
FP-S-012
Clinical diagnosis of tuberous sclerosis in children
J.-X. Liao, L. Chen, B. Li, T.-S. Huang
Epilepsy Center and Department of Pediatric Neurology,
Shenzhen Children’s Hospital and Shenzhen Institute of
Pediatrics, Shenzhen, China
Objective: To guide the practical use and to evaluate the
diagnostic criteria for tuberous sclerosis syndrome made by
the National Tuberous Sclerosis Association, the United
States. Methods: The clinical data of 31 children with
TSC features were studied. Results: The age ranged from
2 months to 12 years (median age 2 years 1 month). The
main clinical features of children with TSC were epilepsy,
psychomotor delay, dermatologic lesions (hypomelanotic
macules and facial angiofibromas), subependymal nodule,
cortical tuber, cardiac rhabdomyoma and renal angiomyoli-

Abstracts 625
poma. Of 31 cases 26 had definite TSC, three had probable
TSC and 2 had possible TSC. Conclusion: The commonly
encountered clinical features related to the diagnostic
criteria are dermatologic lesions (hypomelanotic macules
and facial angiofibromas), subependymal nodule, cortical
tuber, cardiac rhabdomyoma and renal angiomyolipoma.
FP-S-013
Topiramate monotherapy in epilepsy of children
W. Wang, J.-M. Li, Y. Chen
Harbin Children’s Hospital, Harbin, Heilongjiang
Province, China
Objective: To observe the effect, dose and side-effects of
topiramate (TPM) monotherapy in children with epilepsy.
Methods: The study was an open-labeled research in 62
children with epilepsy. TPM was administered as monotherapy.
Result: Sixty-two cases were treated with TPM to an
average course of more than 6 months. A total of 88.7% had
50% reduction in seizure attacks and 50% with no attack
occurred. The curative effect of primary epilepsy was
obviously better than secondary epilepsy. TPM monotherapy
may have satisfactory effects. The adverse reaction was
slight and it decreased the titration of dose. Conclusion:
TPM proves to be effective and safe monotherapy with
few side-effects for all types of seizure in children with
epilepsy.
FP-S-014
An open-labeled trial with topiramate in 46 children
with epilepsy
X.-H. Cheng, C. Zhu, C.-Z. Liu
Department of pediatrics, Xiaogan central hospital, HuBei
province, China
Objective: To investigate the efficacy and side-effect of
topiramate as monotherapy and concomitant therapy in children
with different kinds of epilepsy. Methods: An openlabeled
and self-controlled study was conducted in 46 cases
of pediatric epilepsy treated by topiramate. Monodrug therapy
was used in initiative patients and concomitant therapy
was adopted in refractory epilepsy. Results: (1) The effective
rate was 84.8% of all patients with the treatment of
topiramate, seizure attacks decreased 50–74% in two
cases, 13 cases reduced 75–99% and 24 cases were completely
controlled. (2) Seven cases did not have any effect. In
that seven cases, five cases lost contact, one case quitted
because of aggravating of epilepsy. (3) Thirty-seven cases
were treated by monotherapy of topiramate, 33 cases
(89.1%) had satisfied effect; nine cases were under the treatment
of concomitant therapy, four cases (44.4%) had
evident effect. (4) Adverse reaction: dyspepsia and nausea
occurred in 22 cases (47.8%), summer fever-like syndrome
in 22 cases (47.8%), apathy and hypomnesia in 15 cases
(32.6%), lethargy in 11 cases (23.9%), dizziness and gait
imbalance in four cases (8.7%), weight reduction in four
cases (8.7%), polyuria in two cases (4.4%) and rash in
1case (2.2%). Conclusion: Topiramate is a safe and efficient
new anticonvulsant drug. It produced a good curative effect
to different kinds of pediatric epilepsy, and it can be used as
a first-line anticonvulsant drug in children.
FP-S-015
Pallister-Killian phenotype. Diagnostic difficulties
A. López-Lafuente, J. Campos-Castelló, M.-J. López-
Rodríguez, E. Miravet-Fuster, N. Clusellas-Casals, J.
Antich-Femenías, M.-L. Martínez-Frías
Hospital Clinico San Carlos, Calle Martin Lagos S/N,
Madrid, Spain
Introduction: Pallister-Killian syndrome is a sporadic
disorder with multiple congenital anomalies and a wide
clinical spectrum. These anomalies include coarse face,
hypertelorism, epicanthic folds, wide nasal bridge, prominent
upper lip, dysplastic ears, temporal balding, hypotonia
at birth, pigmentary skin anomalies, epilepsy and severe
mental delay. Lymphocytes show a normal karyotype but
a tissue-specific mosaic distribution of an additional
isochromosome 12p often confined to fibroblast is found.
Objective: We report four cases (three males, two of which
were brothers, one female) with Pallister-Killian phenotype.
Karyotype of lymphocytes was normal in all. Chromosomal
analysis in fibroblasts showed tetrasomy 12p in 84.4% of
them in first case, only in 2% in the second case and was
normal in the other two cases. FISH showed three spots in
75% of nuclei in first case, in 2% of nuclei in second and
third cases and was unremarkable in the fourth. These data
confirmed the diagnosis only in the first case but were not
significant enough in the second and third cases. The fourth
case only had a compatible phenotype. We review the clinical
and cytogenetic characteristics of 50 cases reported in
the literature. Discussion: The high percentage of cytogenetic
abnormalities required to make the diagnosis could
seem too strict. The possibility to decrease the diagnostic
criteria should be considered when a compatible phenotype
coexists.
FP-S-016
Diagnosis of attention deficit hyperactivity disorder with
visual evoked potential
L.-Y. Cai, M.-Z. Zhou, J.-H. Li, L.-L. Huang, S.-Z. Hu
Department of Neurology, Jiangxi Children’s Hospital,
Nanchang, China
Objective: To investigate the clinical value of red flash
visual evoked potential (FVEP) used as an index in diagnosing
ADHD. Methods: In 15 cases of ADHD diagnosed by
clinic (CCMD-3-R), red FVEP was tested. Trigger rate was

626
Abstracts
1.2 Hz. Amplitude was weighted 100 times by computer
automatically. Results: According to FVEP P100 amplitudes,
we divided the 15 cases of ADHD into four grades.
Grades I and II (normal) (N ¼ 2 cases); grade III (mild
disorder) (N ¼ 7 cases) and grade IV (N ¼ 6 cases). Diagnoses
by both Red FVEP and CCMD-3-R can be confirmed
in 86.67% of the cases. Conclusions: Red FVEP is a relatively
objective index in diagnosing ADHD.
FP-S-017
Evaluation of medical treatment of late onset vitamin K
deficiency with intracranial hemorrhage
Y.-K. Zhu, Z.-Y. Ren, Y.-H. Liu, L.-J. Dong, Y. Liu, Q.-Z.
Xu, F.-R. Lu
Hospital of Chinese People’s Armed Police Forces, Harbin,
China
Objective: To summarize the prognosis and factors in
medical treatment to late onset vitamin K deficiency with
intracranial hemorrhage, and explore new treatment methods
to change the prognosis. Methods: We studied 124 patients
who suffered late onset vitamin K deficiency with intracranial
hemorrhage in our hospital from 1996 to 2001. Thirty
patients were followed up. Results: Seventy-two cases
(59.5%) were given up for being worse in 1 of 2 days, 12
(9.6%) cases died. Forty cases (32.2%) recovered. Among
patients recovered, thirty were followed up, ten cases DQ
$70 (33.3%), the rest were with low DQ or with cerebral
paralysis, the prognosis was correlated with the severity of
the lesions found by cranial CT. Conclusion: Patients with
late onset vitamin K deficiency with intracranial hemorrhage
had bad prognosis. The doctors in the primary hospitals were
lack in experience of this disease, it was difficult for them to
diagnose and treat this disease at an earlier stage. Medical
treatment cannot get rid of the factors which were closely
related to the prognosis, such as severe intracranial hypertension,
cerebral hernia, and cerebral embolism. Therefore,
early surgical invention may be more important than medical
treatment for improving the affected infants’ living quality.
FP-S-018
The diagnosis and treatment of childhood moyamoya
disease in 19 cases
J. Hong
Beijing Children Hospital, Beijing, China
Objective: To explore the clinical characteristics and prognosis
of childhood moyamoya disease (MMD) and the diagnostic
value of neuroradiological findings. Methods: We
analyzed the clinical data of 19 inpatients with MMD
admitted to our hospital between December 1989 and January
2000. Patients in this study consisted of 13 males and six
females, aged from 2 years to 13 years and 2 months, of
whom 12 cases were beyond 5 years old (63%). We followed
up them for 9 months to 5 years and 4 months (mean 3 years
and 3 months) in 13 cases in terms of residual neurological
symptoms and activities of daily living, etc. Results: The
main clinical symptoms were motor weakness of extremities
or hemiplegia (17 cases), motor-aphasia (11 cases), and
headache (ten cases). Stenosis or occlusion at the terminus
of the siphon portions of internal carotid arteries (ICA) and
proximal portions of anterior or middle cerebral arteries
(ACA or MCA), and abnormal vascular networks in the
brain were noted by the digital subtraction angiography
(DSA) or magnetic resonance angiography (MRA). The
medical treatments were also given in 18 cases. One case
underwent a surgical treatment. We followed up 12 cases
with medical treatments. Neurological impairment was
found in ten cases, motor impairment in six, transient
ischemic attack (TIA) or headaches in two, epilepsy in two,
motor-aphasia in one and intellectual deterioration in five.
The other two cases had a relatively favorable outcome without
neurological sequelae. Of the 12 cases, nine could live on
themselves. The patients with surgery remained absent of
any neurological sequelae or intellectual impairment during
a 2-year and 5-month follow-up period. Conclusions: Motor
weakness of extremities or hemiplegia, motor-aphasia and
headache were the predominant clinical features of MMD.
The findings of MRA or DSA were crucial to the early diagnosis
of the disease. And early diagnosis and timely treatment
contributed to the improvement of the prognosis of MMD.
FP-S-019
Successful treatment of osteopenia with bisphosphonate
in Duchenne muscular dystrophy. Case report.
S. Christerson
Department of Pediatrics, Örebro University Hospital,
Sweden
Introduction: Children with DMD often develop osteopenia
from inactivity which is accentuated with loss of ambulation
and the treatment with cortisone make this effect
stronger. Fractures are reported in several studies. Case
report: A 14 year-old boy with DMD was treated with cortisone
0.35 mg per kilo from age 5:5 years until 10:8 years. At
10 years he stopped walking and at 11:1 years he had a
fracture of his right arm. He fell 5 months later and got a
femur fracture. Dual energy X-ray absorbometry (DXA) at
11:6 and 12:6 years showed a BMD 21.9 SD for age at
lumbar spine. He was treated with dinatrium pamidronate
15 mg i.v. once a month for a year. DXA 1 year later showed
an improvement in BMD at lumbar spine with 40%. Side
effects were, at first dose 30 mg, fever, stomach pain and
diarrhea for 2 days. With half that dose there were no side
effects. Discussion: Larson and Hendersson (2000) found
significant decreased bone density in 41 boys with DMD
with loss of ambulation. A total of 44% of the boys had
sustained a fracture and four of nine boys stopped walking
after fracture. Long-term effects of biphosphonates show

Abstracts 627
normal linear growth and lamellar structure of bone. Side
effects exist but are transient. Conclusion: Osteopenia in
DMD can be treated with bisphosphonates, which might
lessen the risk for fractures.
FP-S-020
Idebenone reduces protoporphyrin IX and improves
cardiac function in Friedreich’s ataxia
G.M. Buyse, I. Matthijs, L. Mertens, B. Eyskens, F.
Weidemann, G. Di Salvo, J.L.K. Van Hove
Department of Paediatrics (Neurology, Cardiology, Metabolic
Diseases), University Hospital Gasthuisberg, Leuven,
Belgium
Background: In Friedreich’s ataxia (FRDA) no treatment
is currently available despite causative gene and protein
identification. The pathophysiology includes iron-induced
increase in oxidative stress, with secondary deficiency of
mitochondrial Fe-S cluster-containing enzymes such as
ferrochelatase. A recent pilot study in three patients showed
a reduction of hypertrophic cardiomyopathy with idebenone,
a coenzyme Q10 analogue and a free-radical scavenger.
Methods: We conducted a 1 year prospective
therapeutic study with idebenone (5 mg/kg per day) in
seven FRDA patients (age 8.5–27 years). The purpose was
twofold: first, to investigate whether erythrocyte protoporphyrin
IX (normally metabolized by ferrochelatase) is a
therapeutic marker in FRDA, second, to study prospectively
idebenone treatment in FRDA. Neurological function was
assessed using the Cooperative Ataxia Group Rating Scale,
and cardiac function by standard echocardiography and, for
the first time, strain rate and strain imaging, a new method to
quantify regional myocardial function. Results: Baseline
levels of erythrocyte protoporphyrin IX were elevated in
six out of seven patients, and levels decreased significantly
during treatment in all. Idebenone did not improve neurological
function. Long-term idebenone improved left ventricular
mass index, cardiac ejection fraction, and some
regional myocardial function. Changes in protoporphyrin
IX levels did not correlate with changes in cardiac parameters.
Conclusions: Thisisthefirst report showing that
erythrocyte protoporphyrin IX is a potential therapeutic
marker in FRDA. Our results indicate that long-term idebenone
can improve cardiac function in FRDA. Cardiac strain
rate and strain imaging is a sensitive method for therapeutic
assessment in FRDA.
FP-S-021
Selective serotonin reuptake inhibitor (fluvoxamine)
treatment for post-encephalitic Kuver-Bucy syndrome
T. Usuugi, Y. Saito, S. Yanagaki, O. Mayu, E. Yazaki, H.
Mithizu, H. Katumori, K. Hayashij, M. Osawa
Department of Pediatrics, Tokyo Women’s Medical University
Tokyo, Japan
Kluver-Bucy syndrome (KBS) is a rare amalgamation of
cognitive, emotional and behavioral symptoms seen
following insult to the bilateral temporal lobes. To date,
effective treatment for KBS has not been established in
children or in adults. We encountered a 3-year-old-girl
suffering from KBS following acute encephalopathy with
prolonged status epilepticus. She demonstrated most
features of KBS, including hyperorality (i.e. a strong
urge to put non-food items into her mouth), visual agnosia,
language disorder with aphasia, placidity and memory
dysfunction. MRI revealed marked bilateral frontotemporal
atrophy. TRH (1 mg/day) injection therapy for
2 weeks or daily carbamazepine (maximum 20 mg/kg per
day) therapy failed to improve these behaviors. We treated
her with a selective serotonin reuptake inhibitor, fluvoxamine,
for the KBS-associated symptoms. After treatment
with fluvoxamine (maximum 2 mg/kg per day) plus carbamazepine,
the hyperoral behavior markedly decreased
within 1 month, and fluvoxamine could be stopped after
3 months without relapse. Although the precise pathophysiological
mechanisms of KBS symptoms have not been
elucidated, it is hypothesized that these neurobehavioral
symptoms result from disturbance of specific temporolimbic
networks, including the medial and lateral limbic
circuits. The temporo-limbic networks interface with multiple
cortical and subcortical circuits that care modulate
emotional and behavioral symptoms. The serotonergic
systems of these networks might play a role in the KBSassociated
neurobehavioral symptoms, particularly in
hyperorality in children.
FP-S-022
The clinical features and treatment of cerebral
cavernous angiomas in children
J. Xie, Z.-Y. Ma, S.-Q. Luo
Department of Neurosurgery, Beijing Tiantan Hospital,
Beijing China
Objective: To discuss the clinical features, diagnosis
and treatment of paediatric cerebral cavernomas. Methods:
Over the past 11 years, 30 children with cerebral cavernous
angiomas were reviewed. Twenty-seven were operated.
Results: There were 25 boys and five girls. The
average age was 9.4 years. Epilepsy and focal neurological
deficits due to repeated hemorrhage of lesion were the
most common symptoms. CT scan did not show the
special characters. MRI appearances were mainly high
or complex abnormal signal, with ‘ring sign’ of low signal
on T1, especially T2 around the lesion. DSA did not
reveal the feeding and drawing vessels. The results of
operation were satisfactory. Conclusions: MRI examination
is the best method for diagnosis and follow-up of
cerebral cavernomas. Operation is the first choice for
paediatric lesions.

628
Abstracts
FP-S-023
Paroxysmal dyskinesias: clinical features and etiologic
studies
X.-H. Bao, Z.-Y. Pei, J. Qin, X.-R. Wu
Department of Pediatrics, First Hospital of Peking University,
Beijing, China
In order to disclose the clinical feature and the potential
etiology of each kind of paroxysmal dyskinesias, we
reviewed cases with diagnosis of paroxysmal dyskinesias
in the recent 5 years with respect to characteristics of attack,
etiology and treatment response. We evaluated 12 patients’
clinical manifestation, inducing factors, remission, frequencies,
treatment response, etc. Etiologic studies include
video-EEG monitoring, brain MRI/CT, serum ceruloplasmin
level etc. Six belong to paroxysmal kinesigenic choreoathetosis
(PKC), six paroxysmal dystonic choreoathetosis
(PDC). There are family history in one PKC and one PDC.
All patients with PKC were induced by sudden movement,
had shorter duration and higher frequency than that with
PDC. Video-EEG test found epileptic discharge in three
with PKC, no abnormalities were found in patients with
PDC. Antiepileptic drugs were very effective to PKC, and
not useful in PDC. Brain MRI/CT and serum ceruloplasmin
level were normal in all. In conclusion, PKC is different
from PDC in respect of inducing factors, frequency, remission
and the treatment. For PKC, epileptic discharges and
the dramatic response to antiepileptic drugs indicated it
might probably be associated with epilepsy.
FP-S-024
Allopurinol neurocardiac protection trial in infants
undergoing heart surgery using deep hypothermic
circulatory arrest
R.R. Clancy, S.A. McGaurn, J.E. Goin, D.G. Hirtz, W.I.
Norwood, J.W. Gaynor, M.L. Jacobs, G. Wernovsky, W.T.
Mahle, J.D. Murphy, S.C. Nicolson, J.M. Steven, T.L. Spray
National Institute of Neurological Disorder and Stroke
Bethesda, MD, USA
Objective: We conducted a single center, randomized,
placebo controlled trial of allopurinol, an inhibitor of
oxygen free radical production, in infants who underwent
heart surgery using deep hypothermic circulatory arrest
(DHCA). Methods: Allopurinol was administered before,
during and after surgery. Adverse events, including
seizures, cardiac events and coma were monitored. Results:
A total of 318 infants, of whom 131 had hypoplastic left
heart syndrome (HLHS) and 187 had other cardiac disease
(non-HLHS) underwent heart surgery using DHCA. There
was a non-significant treatment effect for death, seizures,
and coma. When cardiac events were added to the primary
endpoint, the allopurinol treatment group experiencing
fewer events (38 versus 60%) in the HLHS stratum,
compared with the non-HLHS stratum (30 versus 27%). In
HLHS surgical survivors, 40 of 47 (85%) allopurinol-treated
infants did not experience seizures, coma or cardiac
events compared with 27 of 49 (55%) controls. Allopurinol
did not reduce endpoint events in non-HLHS infants.
Conclusions: Allopurinol provided neuro-cardiac protection
in higher-risk HLHS infants who underwent cardiac surgery
using DHCA. No benefits were demonstrated in lower risk,
non-HLHS infants. No significant adverse events were associated
with allopurinol treatment.
FP-S-025
Relationship between effect and serum concentration of
loading dosage of phenobarbital in status epilepticus
R.-F. Shi, J. Tian, K.-L. Wang, S.-Z. Sun
Children’s Hospital of Hebei Shijiazhuang, China
Objective: Tofind out the relationship between curative
effect and serum concentration of PB with loading dosage
PB in status epilepticus. Methods: We treated status epilepticus
with intravenous loading dosage of PB at 15 mg/kg
(total dosage #200 mg). We examined serum concentrations
after 10, 15, 25, 30, 60 and 120 min. Results: Sixtyfour
cases were treated, 51 were seizure free in 30 min.
Among them, seizures stopped within 10 min in three;
within 10–15 min in seven; within 15–20 min in 23; within
25–30 min in 18. In 18 cases, seizure frequencies decreased
in 60 min. Six cases showed no responses. In our study, we
examined 180 blood samples. After treatment, serum PB
concentration reached the therapeutic blood level in 15
min. Seizures were controlled after the serum concentration
was above this level. Conclusion: Loading dosage of iv was
highly effective in treating status epilepticus. After 15 min
serum PB concentrations reached the therapeutic blood
level.
FP-S-026
Effects of computerized ultrasonic wave and medium
frequency therapeutic in peripheral facial neuritis in
children
P. Zhang
Department of neurology, Children’s Hospital, Chongqing
University of Medical Sciences, Chongqing, China
To explore a new physical treatment which would be safe
and effective for peripheral facial neuritis in Children. Methods:
Forty-one patients with peripheral facial paralysis were
randomly divided into two groups: 25 patients as experimental
groups, received computerized ultrasonic wave and
medium frequency therapeutic apparatus, and 16 as
controls, received electrical acupuncture treatment. The
course of diseases ranged from 1 to 8 weeks. The cure
rate, general effective rate and side effects were recorded.
We considered 20 times treatment as a treatment course and

Abstracts 629
evaluated three times totally for each patient. Facial nerve
function was evaluated according to revised House Brackmann
(H-B) criteria. Results: (1) The general effective rate
in the control group was 62.5% and among them the cure
rate was 37.5%; whereas in the experimental group, these
were 84 and 76%, respectively. Comparison of cure rate
between the two groups showed the difference was significant
(x 2 ¼ 3.8567 , 11.91, P , 0:05 , 0:01). (2) All
patients in the control groups suffered from pain and the
obedience was poor, while nobody in the experimental
groups were upset and the patients expressed good obedience,
as well as their parents. (3) During the treatment, no
infection occurred in two groups. Conclusion: Computerized
ultrasonic wave and medium frequency therapeutics
was better than electrical acupuncture in peripheral facial
neuritis in children. The patients with the apparatus avoid
secondary infection and the danger of pin breaking. There
was nearly no pain. It is a safety and effective physical
treatment for peripheral facial neuritis in children.
FP-S-027
Management of cerebral palsy in Egyptian children with
botulinum toxin type A (BTX-A)
A. Raouf, R.A. Hakim
Police General Hospital, Agooza, Cairo, Egypt
Background: Cerebral palsy is the commonest cause of
physical handicap in children in Egypt, occurring in 3–4 of
every 1000 live births. The most common form of cerebral
palsy is spastic type (88%). Objective: The safety and dosage
of botulinum toxin type A (BTX-A) in children under 2 years
of age were examined in a 18 month period at Police General
Hospital, Cairo, Egypt. Methods: Twenty-two children with
diplegia, aged between 11 and 24 months, were studied. The
average dose was 4–6 u/kg body weight and the interval
between injections was 3–6 months. Results: Significant
improvement in both Ashworth score (P , 0:001) and
ROM (P , 0:01) was achieved and the safety record for
these treatments was excellent. The most commonly reported
adverse effect, apart from local hematoma, was muscle weakness.
Conclusion: Children who respond to BTX-A treatment
have improved physical functioning and gait, and are able to
sustain these results long term.
FP-S-028
Influence of anticonvulsants on carbamazepine diurnal
fluctuation and plasma protein binding in epileptic
children
B. Steinborn
Chair and Department of Developmental Neurology K.
Marcinkowski University of Medical Sciences, Poznań,
Poland
CBZ is one of the most commonly used AED. It is
frequently prescribed in combination with other AEDs,
leading to drug interactions. Aim of study was to estimate
pharmacokinetic parameters of CBZ plasma protein binding;
assess diurnal fluctuations in total and unbound CBZ of
epileptic children treated with VPA, LTG, VGB or TPM.
Eighty children, aged 3–18 years were included to the study.
The doses of CBZ did not differ statistically. Blood samples
were taken in steady state before the morning dose and
subsequently every 3 for 24 h. Diurnal fluctuations of
serum concentrations of total and free CBZ during CBZ 1
VPA, CBZ 1 LTG, CBZ 1 TPM and CBZ 1 VGB were
synchronous and in therapeutic range. The fluctuation
index (FI) of total CBZ for polytherapy CBZ 1 VPA and
CBZ 1 LTG were higher than for combination of CBZ 1
TPM or CBZ 1 VGB. Diurnal fluctuations of total CBZ
were prominent than fluctuations of unbound CBZ in comedication
with VPA, LTG or VGB. FI for free and total CBZ
in bitherapy with TPM did not differ statistically. Mean
CBZ free fraction in bitherapy with LTG was 16.4%, with
VGB 15.9% with VPA was 18.2% and with TPM – 21.5%.
The FI for CBZ was the highest in LTG. The increase of free
CBZ in patients treated with VPA or TPM may be a result of
pharmacokinetic interactions between these AEDs.
FP-S-029
Gene therapy to murine MPS VII by human neural stem
cells transplantation
X.L. Meng a , J.S. Shen a , T. Ohashi a,b , S.U. Kim c , Y. Eto a,b
a Department of Gene Therapy, Institute of DNA Medicine,
The Jikei University School of Medicine, Tokyo, Japan;
b Department of Pediatrics, The Jikei University School of
Medicine; c Division of Neurology, Department of Medicine,
University of British Columbia, Canada
Cell therapy to murine MPS VII, authentic model of
human Sly syndrome, caused by the deficiency of the lysosomal
enzyme, b-glucuronidase, was studied. Immortalized
human neural stem cell line, HB2F3, was labeled with EGFP
and transplanted into the lateral ventricles of newborn normal
mouse brain. At 2 weeks after transplantation, EGFP positive
cells were observed to be lining the surfaces of the ventricles.
At 4–6 weeks, some cells migrated to the olfactory bulb, the
striatum and cortex, differentiated into neurons, astrocytes
and oligodentrocytes in vivo. Further, we studied if the
human neural stem cell transplantation is effective for the
treatment of CNS pathology of MPS VII. Human neural
stem cells over-expressing 80 times more b-glucuronidase
activity in vitro were generated by retroviral transduction
which encoding a human b-glucuronidase cDNA under the
CMV promoter. After neonatal intraventricular transplantation,
transplanted cells expressed biological active b-glucuronidase
in the MPS VII mouse brain, which was elevated to
5% that in the wild type mouse in whole brain homogenize at
3 weeks after transplantation. The distribution pattern of the
transplanted cells was similar to that of EGFP expressing

630
Abstracts
cells. Pathological improvement is currently under investigation.
FP-S-030
Topiramate monotherapy in childhood epilepsy
M. Mazurkiewicz-Bel⁄dzińska a , A. Matheisel a , J. Wendorff b ,
G. Ircha b , R. Chmielewski c
a Department of Developmental Neurology Medical University
of Gdańsk, Gdańsk, Poland; b Department of Child
Neurology, Institute of Polish Mother Health Centre,
L⁄ ódź, Poland;
c Johnson and Johnson Pharmaceutical,
Warszawa, Poland
Introduction: Topiramate is a new antiepileptic agent
with multiple mechanisms of action. It is a broad spectrum
of antiepileptic effect. It can be used for therapy of partial,
generalized seizures and epileptic syndromes. The efficacy
of TPM on monotherapy was evaluated in several clinical
studies, but there are still limited data according to use of
topiramate in monotherapy in children. Materials and methods:
In order to evaluate the efficacy and tolerability of TPM
monotherapy of childhood epilepsy, we analyzed prospectively
two patients on TPM therapy as a first line antiepileptic,
and 16 in whom previous AED was switched to TPM
monotherapy. There were 12 children with symptomatic and
six with cryptogenic epilepsy. Majority of the patients had
partial evolving to secondarily generalized seizures. We
administered TPM according to the slow-titration schema:
initial dose was 0.5–1 mg/kg per day, the dose increased
0.5–1 mg/kg every 10–14 days. The dosage of TPM at the
steady state was 7–11 mg/kg. The mean time on TPM
monotherapy was 10 months. Results: The mean frequency
of seizures before TPM therapy was 1 per month to 2–3 per
day. Seizure reduction was observed in 17 patients, nine of
them were seizure free during the observation. Observed
adverse events were mostly mild and transient. In one this
led to discontinuation of treatment. Conclusions: The use of
TPM as a first-line monotherapy in children with epilepsy
seemed to be valuable and promising.
FP-S-031
Response to topiramate in children with catastrophic
epilepsy syndromes
R. Chmielewski a , M. Mazurkiewicz-Bel⁄dzińska b ,A.
Matheisel b , B. Mańkowska b
a Johnson and Johnson Pharmaceutical, Warszawa, Poland;
b Department of Developmental Neurology Medical University
of Gdańsk, Gdańsk, Poland
Aim of the study: Several epileptic syndromes in children
are characterized by treatment resistant seizures, progressive
loss of higher intellectual functions and characteristic electroencephalographic
abnormalities. It is well known that
control of seizure results in better intellectual outcome.
The long-term efficacy and tolerability of medication are
important especially in epilepsy. Materials and methods: In
order to estimate the long-term effectiveness of TPM therapy
in children with drug-resistant epilepsy syndromes, medical
records of children with West syndrome (3), Lennox-Gastaut
syndrome (38), myoclonic-astatic epilepsy (3) and severe
myoclonic epilepsy (Dravet syndrome) (3) treated with
TPM were analyzed. Mean age of the patients was 6.8
years (range 7 months–16 years). TPM was administered as
add on therapy with doses ranging 6–10 mg/kg per day. The
mean age of treatment was 12 months and follow up was 18
months. In order to estimate retention rates and factors
predicting successful treatment with TPM we used
Kapplan–Meyer analysis and Gehan tests. Results: A total
of 50% remained in TPM therapy after 12 months of treatment.
The factors which influenced long-term retention rates
were younger age presence of tonic or absence seizures.
Forty-one percent remained in TPM therapy after 18 months
of treatment and seven (37%) of them were seizure free.
Conclusions: Our results showed valuable and long-term
efficacy of TPM in treating drug resistant epileptic seizures
in childhood.
FP-S-032
New antiepileptic drugs in childhood epilepsy – long
term efficacy, tolerability and quality of life
M. Mazurkiewicz-Beldzińska a , A. Matheisel a , B. Mankowska
a , J. Biclicka-Cymerman b
a Department of Developmental Neurology, Medical University
of Gdansk, Gdans, Poland; b Department of Neurology,
Children’s Hospital, Warszawa, Poland
The long-term efficacy and tolerability of medication are
important in epilepsy. Since 75% of first epileptic seizures
occur before 18 years of age, it is important to have an idea
which drug might have the greatest chance of success in children.
There are a few methods of assessing quality of life in
children with epilepsy. Considering the available methods of
assessing quality of life in epilepsy, we have constructed and
validated a questionnaire for parents children with epilepsy to
estimate the influence of epilepsy on the main aspects of life in
their children, and subsequently, to assess family dynamism.
A retrospective chart review was conducted for epilepsy
patients from our departments who were on lamotrigine
(LTG) (157), vigabatrin (VGB) (79) and topiramate (TPM)
(101). We calculated the retention rates for each antiepileptic
usingKapplan–Meyeranalysis.Thecorrelationbetweentherapeutic
effect and type of epilepsy, age of onset, etiology and
concomitantantiepileptictreatmentwasanalysed.Thetimeof
follow-up was 21 months. After 1 year 69% stayed on TPM,
68% on LTG and 48% on VGB. After 2 years, the retention
rates were 57, 61 and 30%, respectively. The seizure free rates
after 1st year of treatment were 31% for TPM, 28% for LTG
and22%forVGB.Therewasstrongcorrelation between good
LTG effect and absence of CBZ in concomitant therapy, and

Abstracts 631
the presence of generalized seizures. For VGB, correlation
was good between the presence of psychomotor retardation
and better seizure outcome. For TPM, we noticed better
outcome in patients with symptomatic or cryptogenic epilepsies.
The greatest improvement in quality of life in patients
was when their seizures were controlled. Interestingly, it did
not reduce the fear of the parents about child’s general health.
Improvement in the quality of life was reached earliest with
TPM therapy but it lasted longer on LTG therapy. Our results
showed that new antiepileptic drugs can serve as a good treatment
strategy for many childhood epilepsies. They can
provide seizure remission or significant seizure reduction.
They could be considered (in many cases) as the first-line
antiepileptic medications.
FP-S-033
Use of trihexiphenydyl (THP) in dystonic cerebral palsy
– a study of 14 patients
V. Udani, M. Khadye
P.D. Hinduja National Hospital and Medical Research
Centre V.S. Marg, Mahim, Mumbai, India
Dystonic cerebral palsy is a significant problem. High dose
trihexiphenydyl has been used successfully in dystonia due to
other causes. This study was undertaken to assess the effect of
this drug in dystonic cerebral palsy (DCP). Methods: DCP
were identified from our OPD. Those with progressive
diseases were excluded. Our therapist assessed the dystonia
as being functionally handicapping before inclusion. Video
recording was performed using a predetermined plan and
THP started at a minimum dose which was increased if
needed. After 3 months a repeat video recording was
performed. Both video were undated and reviewed by a
blinded observer using the Barry-Albright scale. Results:
Fifteen children (M:F 8:7), median age 8 months were
recruited. Etiology was prenatal in two, perinatal in nine
and postnatal in three. One child was excluded when follow
up revealed a progressive disorder. THP was started at a
median age of 12 m (range 5–144 m). Mean daily dosage
was 9.9 mg/day (range 3–30 mg). Significant improvement
was noted in scores in 13 children though one stopped treatment
due to fever. One child worsened. The main improvement
was in upper limb and neck dystonia. Functional
improvements were noted in all 12 patients who continued
the drug. Conclusion: High dose THP seemed to be effective
for dystonic CP. It seemed to improve functional status and
was well tolerated.
FP-S-034
Infantile spasms in Down syndrome: good response to
short course of vigabatrin therapy
R. Nabbout a,b , I. Melki a , B. Gerbaka a , O. Dulac b ,C.
Akatcherian a
a Department of Pediatrics, Hôpital Hôtel Dieu de France,
Université St Joseph, Beirut, Lebanon,
2 Department of
Neuropediatrics, Hôpital St Vincent de Paul, Paris, France
Purpose: To evaluate the efficacy of VGB in treating
infantile spasms associated with DS and to assess the feasibility
of early withdrawal in order to reduce the possibility
of retinal toxicity. Patients and methods: Five DS children
presenting with IS were treated with vigabatrin as first line
monotherapy in an open prospective study. The mean age of
VGB treatment was 9.9 months (range ¼ 7–14.6 months).
The short term response was evaluated based on clinical
response of disappearance of spasms and of paroxysmal
interictal activity on EEG. VGB was continued in responders.
The treatment was tapered gradually over 1 month
after 6 months in those who were still spasms free. Results:
Four children became spasms free on VGB (75–100 mg/kg
per day) and three responded within 1 week. This response
was maintained during the 6 months in VGB treatment.
After VGB withdrawal with follow-up ranging from 2 to 4
years, none of the responders experienced spasms recurrence
or other types of seizures. Conclusion: Our study
confirmed the efficacy of VGB in Infantile Spasms associated
with Down Syndrome. Moreover, the duration of
VGB treatment can be reduced to 6 months without relapse
of IS. This short treatment might reduce the risk of developing
visual field constriction.
FP-S-035
Analysis on myelin basic protein level in serum and CSF
of infant with intracranial hemorrhage
Y.-Y. Yang, X. He, S.-D. Yang
Neurological and Rehabilitating Department, Guangzhou
Children’s Hospital, Guangzhou, China
Objective: To investigate myelin basic protein (MBP) in
CSF and serum in intracranial hemorrhage (ICH) infant,
study the relation between ICH and quantitative MBP.
Methods: Thirty-eight cases were studied with easy MBP-
ELISA method; study group I (n ¼ 16) were substantial (or
combined with ventricular) hemorrhage, while group II
(n ¼ 22) were non-substantial. There were ten cases in
control group their quantitative MBP in CSF and serum
were tested, then repeated and compared after treatment
for 4–5 weeks. Results: Quantitative values MBP in CSF
and serum in both study groups were significantly higher
than control group (P , 0:01), while values in substantial
group were also significantly higher than non-substantial
one (P , 0:01). MBP levels did not decrease after treatment
for 4–5 weeks. Conclusion: Quantitative test for MBP in
CSF and serum can be used as a biochemical guideline for
the diagnosis to this disease, and helpful in predicting prognosis.

632
Abstracts
FP-S-036
Significance of serum S-100B and NSE of children with
head injury
L.-L. Jin
The pediatric of Tian tan Hospital, Beijing, China
Objectives: To study the significance of measurement for
S-100B and NSE in serum of children with head injury.
Methods: Blood samples were taken shortly after the trauma
(12–24 h) 18 with severe head injury patients (GCS ,8) and
18 minor head injury (GCS .12). Blood was allowed to clot
and after centrifugation for 30 min (3000 g, 4 min) serum
was stored at 2308C for later analysis. Serum protein S-
100B and NSE were analyzed by use of immunoluminometric
assays. In 16 healthy children these markers were
also measured. S-100B and NSE concentrations in serum
of patients and controls were compared. Results: The differences
of serum S-100B and NSE in each group (group 1,
group 2, and controls) are significant. The values of mean
serum S-100B and NSE are as follows: group 1 S-100B:
1.05 ^ 0.52 mg/l, NSE; 69.13 ^ 37.86 mg/l group 2 S-
100B: 0.47 ^ 0.21 mg/l, NSE: 26.14 ^ 13.46 mg/l, controls,
S-100B: 0.19 ^ 0.09 mg/l, NSE: 14.43 ^ 7.70 mg/l.
Compared between groups 1 and 2 S-100B (t ¼ 4:57,
P , 0:01), NSE (t ¼ 4:54, P , 0:01); group 2 and control
S-100B (P ¼ 4:724, P , 0:01); NSE (P ¼ 3:059,
P , 0:01), group 1 and control S-100B (t ¼ 6:569,
P ¼ 0:00); NSE (t ¼ 5:665, P ¼ 0:00). Conclusion: S-
100B concentration was higher in severe head injury than
minor head injury. Median NSE concentration was higher in
patients than controls and severe head injury higher in
patients than that of minor head injury patients. S-100B
and NSE is a useful marker for brain damage and neuron
damage in severe and minor head injury.
FP-S-037
Evaluation of visual-motor Bender-Gestalt perception
test (Bender test) in diagnosis of childhood migraine
I. Frančula, I. Prpić, I. Vlašić-Cicvarić, Z. Korotaj, E.
Paučić-Kirinčić
University Children Hospital ‘Kantrida’ and Medical
Faculty of University of Rijeka, Rijeka, Croatia
According to IHS 3–5 attacks of headache is required to
fulfil criteria for migraine, which sometimes means a period
of year or more. In order to speed up the diagnosing of
migraine we have analyzed the results of Bender test. Retrospectively
results of the Bender test were analyzed in 24
children with definite migraine diagnosis (according IHS
criteria). There were nine boys (mean ¼ 9.8 age) and 15
girls (mean ¼ 11.8 age). Boys were statistically significantly
younger than girls. No difference between boys and
girls regarding their general intelligence and social background.
The children performed Bender test at an early
phase of the headache (mean ¼ 2 days, SD 1.5 day) and
repeated in a period without headache (mean ¼ 6 days,
SD 2.5). At an early stage after the headache, suspected or
definite organic cerebral dysfunction was found at 20 children
while four had normal results. Bender test performed in
phase without headache revealed only three children with
organic cerebral dysfunction, and the rest of the children had
normal results. There was statistically significant difference
between the results of first and second Bender test measurement
(Fisher test: 0.00015). Our results revealed that visualmotor
capacity is disturbed at children with migraine in an
early phase of headache and it took at least 6 days to normalize.
These findings demonstrated that Bender test may be
used as a rapid and useful diagnostic tool in migraine diagnosis.
One can make safe conclusion of migraine accompanied
by disturbed visual-motor perception. Furthermore, it
was shown that children with migrainous attack need 4–8
days to regain their visual-motor ability which is important
information considering child’s daily school obligations,
particularly reading and writing skills.
FP-S-038
Pontine/extrapontine myelinolysis due to dysequilibrium
syndrome
Ö. Faruk Aydın a ,Ç.Üner b ,N.Şenbil a ,K.Bek c ,Ö. Erdoǧan c
a Departments of Pediatric Neurology and c Pediatric
Nephrology; b Division of Radiology, Dr. Sami Ulus Children’s
Hospital, Ankara, Turkey
Neurological disorders may be seen in end-stage renal
disease patients due to complications of haemodialysis or
peritoneal dialysis. A dysequilibrium syndrome may be
seen, usually soon after or towards the end of dialysis. We
report a patient with pontine/extrapontine myelinolysis due
to dysequilibrium syndrome. The patient had depressed
consciousness, agitation and tremor towards the end of the
second peritoneal dialysis with stupor and hyperactive deep
tendon reflexes. Brain CT scan showed hypodense lesions in
pontine and extrapontine locations without radiocontrast
medium enhancement. After 2 days, the patient had only
memorial deficits. A follow-up Brain CT scan 1 week
later showed a decrease of the lesions in pontine and extrapontine
locations. Central pontine/extrapontine myelinolysis
should be suspected and investigated in the acute
neurological disorders of dialysis patients.
FP-S-039
Complex metabolic therapy at newborns affected by
central nervous system damage
T.A. Djumabekov, B.D. Zhurkabayeva, C.V. Malinina
Children’s Hospital No. 1, Almaty, Republic of Kazakhstan
Objectives: Determining efficiency of a complex hyperbaric
oxygenation and neurometabolic therapy at newborns

Abstracts 633
with hypoxic and ischemic damage of CNS Methods: Eighty
newborns with clinic damage of CNS and with manifestation
of consciousness disturbance of CNS, spoor-35 children,
coma-25 children. Blood circulation and breathing
disturbance, edema of brain symptoms have been identified
by the most of children. The hyperbaric oxygenation had
been prescribed to the main group of children (50 patients)
during 30 min in combination with neurometabolic medicines:
Instenon 1 ml in 5% of glucose solution together with
Actovegin 1000 mg in 24 h. The therapy has been effecting
about 5–10 days. The control group children had only
hyperbaric oxygenation. Results: During complex hyperbaric
oxygenation and neurometabolic therapy had been determined
improvement of rehabilitation processes: the positive
dynamic in neuroreflection sphere, recovery of consciousness,
stabilization of hymodynamic indexes and spontaneous
breathing. The critical condition recovery of
newborns reduced by 2–3 days, lethality was lowered
(comparing with control group) Conclusion: Carrying out
hyperbaric oxygenation and neurometabolic therapy in
combination with Instenon and Actovegin at newborns
with hypoxic and ischemic damage advanced the date of
rehabilitation and also critical Condition recovery. In the
result of this lethality was lowered, staying patients in clinic
was reduced by 3 days. That approves that this method is
advisable for widely using.
FP-S-040
Treatment of movement disorders introducing
homeopathic medicine of original method
T. Osipenko, O. Osipenko
Scientific medical Center on treatment and prophylaxis of
neurologic disability in children, Moscow, Russia
Six courses of treatment have been done in a half year’s
time during 3 years 20 children with movement disorders
from city Pavlodar in country Kasahstan. There were children’s
cerebral paralyses spastic and spastic-hyperkinetic
forms. Complete course of treatment includes: (1) microinjections
biologically active medicaments – homeopathics
medicines by cerebrum compositum, Traumell, coenzyme
compositum (Heel, Baden-Baden) in the perineural, periganglonal,
miomeres and scleromeres points in the spinal
card segmentes, which permits to correlate differentially the
pathological posotonic and motor functions. (2) Scleromere-point
pharmacomassage by Traumel pointment.
After first injection course the changes were fast. Spastic
syndrome and hyperkinesis are significantly decreased. At 3
years children are independently walking and talking.
FP-S-041
Diagnostic criteria for Rett syndrome in children
N.V. Andreyenko, O.I. Maslova, V.M. Studenikin
Division of Psychoneurology, Research Institute of Pediatrics,
Scientific center of Child Health (Russian Academy of
Medical Sciences), Moscow, Lomonosovsky, Russia
Background: A hereditary disease, known as Rett
syndrome, that affects exclusively girls, remains a point of
interest both for neurologists and psychiatrists since it has
been described in 1966 by A. Rett. Goal: The goal was to
determine clinical and diagnostic criteria for Rett syndrome
in pediatric patients. Method: For that purpose we have
observed 22 female patients (aged 1–7 years), using conventional
neurological diagnostic methods, including EEG, CT
and MRT, ultrasonics, etc. Results: Eighteen patients were
diagnosed as having Rett syndrome in our clinic (81.8%).
The main clinical findings in Rett patients were the following:
(1) motor development delay (100%); (2) mental retardation
(100%); (3) manual stereotypy (100%); (4) mild
respiratory distress: episodes of hyperventilation and/or
apnea (86.4%); (5) muscular hypotonicity (86.45); (6)
seizures (72.7%); (7) microcephaly (72.7%); (8) ataxia
(68.2%); (9) induced screams and/or laughter (68.2%);
(10) hypersalivation (68.2%); (11) autism (autistic features)
(54.5%); and (12) bruxism (50.0%), and autoagression
(18.2%). Other (additional) symptoms were not so frequent
or evident. MRI and CT scans revealed cortical atrophy in
frontal-temporal areas of 12 patients (54.5% of cases). Characteristic
EEG findings in Rett patients included quick
complexes ‘peak-slow wave’ configurations with predominant
localization in central areas of the brain. Conclusion:
Our data allow us to conclude, that combination of manual
strereotypy, respiratory paroxysms (apneic spells) and
seizures against the background of developing microcephaly
and progressing disturbances of psycho-motor sphere
should be considered as pathognomonic diagnostic criteria
for establishing the diagnosis of Rett syndrome in children.
FP-S-042
Thallium-201 SPECT in pediatric brain tumors
V. Humbertclaude, J. Chevalier-Tessier, N. Leboucq, A.
Roubertie, B. Echenne, P. Coubes
Department of Pediatric Neurology and Neruosurgery,
CHU Saint Eloi-Gui de Chauliac, Montpellier, France
We retrospectively studied 59 children (4 months–16
years of age). To determine whether Thallium 201 (T1)
SPECT could detect malignant brain tumors and provide
relevant information during follow-up that could not be
derived from MRI imaging. A total of 209 T1 SPECT
were performed. Histological diagnosis was obtained in
each case. Results of T1 SPECT were compared to clinical,
histological and MRI Results. Thallium uptake was considered
significant when the ratio to a homologous region of
interest was equal or superior to two. Twenty-five patients
were studied at the moment of initial diagnosis. Thallium
uptake was present in 16 cases with high grade malignant
tumor. Nine cases with low grade tumor were Thallium

634
Abstracts
negative. A total of 184 T1 SEPCT were realized postoperatively
and/or during medical therapy. SPECT scans
(147) realized in children with absence of tumor recurrence
or evolution on MRI showed absence of Thallium uptake
(true-negative results). In one of these cases, T1 SPECT
permitted the diagnosis of radionecrosis. Thirty true-positive
results were obtained. Thallium uptake was present in
29 SPECT scans realized in children with MRI in favor of
tumoral activity. In one supplementary case. T1 uptake
revealed tumor recurrence 1 month before MRI. False negative
results were observed in two cases, and false positive
results in five cases. Thallium 201 SPECT provided useful
information for the diagnosis and the follow-up of pediatric
malignant brain tumors, even in posterior fossa location,
technically more difficult to study by SPECT.
FP-S-043
Clinical study of treating cerebral palsy with Chinese
and Western medicine
Z.-H. Liu, P.-G. Pan, M. Ma
Pediatric Neurology, Department of Recovery Hospital for
Women and Children, Nanhai, Guang Dong, China
Objective: To investigate the better effective CP rehabilitation
mode and suit the condition of our country. Methods:
To implement modern rehabilitation (western
medicine 1 physic therapist) 1 tradition rehabilitation
(acupuncture, traditional Chinese medicine) 1
rehabilitation mode of home rehabilitation. A total of 300
CP patients of 1–7 years old were randomly divided into two
grouts for clinical study. Results: In the recent period of
treatment group (3 months) there were obvious effects.
Value of big motion, fine action DQ, MQ, GMFM ascended
more than before treatment (P , 0:01). Cranial CT cerebral
atrophy, cerebromalacia recovery normal tare was 26.5%.
There was significant difference (P , 0:01). Conclusion:
Chinese and western rehabilitation mode is better effect
and suit the condition of our country CP rehabilitation
mode.
FP-S-044
Role of botulinum toxin in cerebral palsy
P.A.M. Kunju
Department of Pediatric Neurology, Medical College
Trivandrum, Kerala, India
We have tried botulinum toxin A (BT) for spasticity
(diplegic, hemiplegic or choreoathetosis) management in
15 children with cerebral palsy. Ten of them had severe
spastic diplegia, three had hemiplegia and two had athetosis.
All children were undergoing physiotherapy with monitoring
of their baseline status for 6–24 months before BT injection.
Six of them were walking with varying disability and
nine were either sitting and standing with support only.
Twelve had calf muscle spasticity leading to equinus deformity,
ten had scissoring due to adductor spasm and nine had
flexed knee posture due to hamstring spasm. Two of them
were having very limited range of movement at ankle and
knee. Target muscles identified by functional spasticity
assessment or by EMG guidance. The mean follow-up
time was 25 months. Post injection, intensive physiotherapy;
splinting orthoses and other appliances were done
either by the advice of physiatrist or by the author himself.
Response parameters included changes in muscle tone
assessed by the modified Tardieu scale, periodic observation
of longitudinal gait parameters as well as parental assessments
of improvement. Gait recording was done on videotape
for eight patients. The effect was evident as early as 3–5
days. The peak effect was noticed by 1–2 weeks in the
majority; the effect lasted for 3–10 months. In five of
these patients a repeat injection was done during a varying
period of 4–9 months by the suggestion of relatives, as they
wanted to consolidate the positive response in spite of the
high cost of the treatment. All children except two with
fixed contractures experienced decreased spasticity scores,
scissoring, equinus deformity and improved mobility. Three
non-ambulatory children became ambulatory with assistance
and five with assisted ambulation became more independently
mobile. Video analysis revealed comparative
improvement. Ability to rise from sitting to standing also
demonstrated improvement. No positive response to choreoathetosis
noticed. Remote effect like improvement in
drooling and swallowing also noticed. None of the children
had any untoward side effects. Botulinum toxin is useful in
treating spasticity of CP. Pretreatment patient observation
and assessment is very important, Post treatment physio and
appliances helps in consolidating the results.
FP-S-045
The efficacy of a newly-developed of a newly-developed
package for the promotion of motor patterns
M.T. Joghataei, G.R.H.P. Nezhad
University of Social Welfare and Rehabilitation Sciences,
Tehran, Iran
How to deal with spasticity and flaccidity in the upper and
lower limbs of the cerebral palsy child has always been a
very unquenchably controversial issue in the field of rehabilitation
of the cerebral palsy client. A demanding research
area has been the comparison of various inhibitory and
facilitatory techniques in dealing with tone disorders. The
commonly practiced methodologies such as the Bobath
method are well known to the profession. More recent
attempts have, however, turned toward the promotion of
more active functioning on the part of the child, whose
main premise is that sound rehabilitation depends less on
passive or assistive measures and more on independence
through the implementation of client-controlled selfinitiated
goals. The advent of sensory rooms has been a

Abstracts 635
great revolution in the field. In such rooms, the child is
presented with a multitude of sensory stimuli and feedbacks
in order to receive compact sensory stimuli and provide
proper responses to those various stimuli. However, a
demerit of sensory rooms is that they demand general movement
patterns (combinations of motions in different joints of
the upper limb, for instance), which are not necessarily
remedial to the pathological patterns and movements
made by the child, but detrimental in many cases as the
main goal of the use of sensory rooms is mere mobility
and there is no strict control of the abnormal patterns,
where the child tries to, for instance, touch a certain pad
to have a specific sound heard, and may over try and make
the involved muscles more spastic than they actually are.
The present article is the report of the results of a 2-year
research project conducted at the University of Social
Welfare and Rehabilitation Sciences in Iran to develop an
electronic package which aimed to have the merits of a
typical sensory room and dispose of its demerits. As regards
the description of the package, it consists of an attachment
kit, which is a combination of pads and straps attached to the
neighboring levers of the given joint (for example, forearm
and arm in the case of the elbow). The pads contain certain
sensors which will record the initial posture of the joint and
signal the flexion/extension attempts of the client after the
‘game’ starts. The ‘brain of the package’ consists of a
keyboard which gets the orders from the therapist. Orders
might include the assignment of the type of feedback to be
presented when a certain motor attempt was reported. These
include beeps, bells ringing, songs, and recorded messages
such as ‘well-done’ as auditory reinforcers and lights of
different color to be displayed on the light-boards of the
package mounted onto the wall in the child visual field as
visual feedbacks (other accessory parts provide the child
with tactile feedbacks such as a breeze blown out of a
small fan). The keyboard also gets orders as to what movement
patterns to reinforce (flexion or extension). Packs of
different sizes have made the kit versatile for all the joints of
the extremities. Utilizing the above package as the instrument,
a true experimental study was carried out to investigate
the performance of this package compared with that of:
(1) traditional facilitatory/inhibitory techniques; and (2) a
typical sensory room. The study was designed for the
following purposes: (1) to find out the possible challenges
to the use of the package. (2) To compare the efficacy of the
package with that of other measures in diminishing spasticity.
(3) To compare the efficacy of the package with that of
other measures in increasing mobility. (4) To cross-compare
the effect of only auditory/only visual/and auditory and
visual feedbacks combined. Ninety-six spastic CP children
of both sexes aged between three and nine from twelve
clinics located in southern and northern parts of the city
were selected and assigned to the experimental group and
the two control groups. The experimental group received a
treatment of a 3-month period of rehabilitation with the
above package, while one control group received rehabilitation
in a sensory room and the other by facilitatory and
inhibitory techniques. The three groups were matched on
mobility, spasticity/flaccidity, and normal patterns as the
dependent variables of the study. At the end of the treatment
period, different scales of the above variables were then
plotted, cross-compared, and interpreted. A variety of statistical
techniques were employed to tackle the questions of
the study. These ranged from simple correlations and
ANOVAs through factor and regression analyses to structural
equation modeling and path analysis. Results: Showed
that the mentioned package specifically significantly
reduces spasticity, increases mobility, and enhances motor
pattern normality differently from the two other measures of
the study. Also, it was found that a combination of auditory
and visual feedbacks proved more effective than the other
two choices. Implications and applications are expounded.
FP-S-046
Selective tibial neurotomy for relief of spasticity
focalized to the ankle in children with cerebral palsy
Y.-B. Yu, L. Zhang, Z.-S. Zhou
Department of Neurosurgery, China-Japan
Hospital, Beijing, China
Friendship
Objective: To study the effectiveness of selective tibial
neurotomy for relief of spasticity focalized to the ankle in
children with cerebral palsy. Methods: Fifty-three feet of 37
cases of ankle spasticity in children with cerebral palsy were
treated by microsurgical selective tibial neurotomy from
February 2000 to June 2001. Results: At follow up evaluation
(mean duration: 8.6 months), this study showed that
100% cases experienced disappearance or notable regression
of the spasticity right after operation, and the percentage
in follow-up duration was 94.34%. The improved
motor capacities right after operation were found in 100%
cases, while 94.34% in follow-up duration. One hundred
percent cases had better quality of life by follow-up studying.
Postoperative complications were few, including
dysaesthesias of foot in two cases. Muscle weakness was
not found in all the cases. Conclusions: Selective tibial
neurotomy is an effective and safe microsurgical method
for the treatment of ankle spasticity in children with cerebral
palsy.
FP-S-047
Early curative effect for neurophysiological treatment of
cerebral palsy
Y.-X. Ma, X. Wu, J.-J. Zhang
Harbin Children’s Hospital, Harbin, Heilongjiang Province,
China
Objective: To investigate early curative effect for neurophysiological
treatment of cerebral palsy. Methods: Diagnose
and type classification of cerebral palsy depend on the

636
Abstracts
standard of the first national meeting of cerebral palsy.
There are Spastic type 27 cases, athetosis type five cases,
dystonic type 1 case. We use Vojta method to bring out the
rolling reflex and bow crawl, and use Bobath method to
promote establishing normal posture and reflex. We pay
more attention on motor function, operative ability, and
speech functional training. We evaluate the effect of treatment
according to the GMFM every half a month. Comparing
the results of two evaluation, it is obvious effect if the
score increase more than 20 points; effective if the score
increase 10 , 20 points; no effect if the score increase
,10 points. Results: Obvious effects were observed in 3
cases (all ,1 year old, spastic type), effective in 27 cases
(23 cases ,2 years old, including spastic type 23 cases,
athetosis type four cases). No effect were observed in
three cases (all .3 year old, spastic type 1 case, athetosis
type 1 case, dystonic type 1 case). Conclusions: Early curative
effect for neurophysiological treatment of cerebral
palsy is relative good, especially for the spastic type. The
older children with good speech and intelligence have better
effect.
FP-S-048
Efficacy of GPi stimulation on pantothenate kinase
associated neurodegeneration (PKAN) related dystonia
P. Castelnau a , A. Gannau b , L. Cif b , S. Hemm b , P. Evrard c ,P.
Coubes b
a Pediatric Neurology Service and INSERM 316, Clocheville
University Hospital, Tours, 37044 France; b Department of
Pediatric Neurosurgery (Research Group on Movement
Disorders in Children), Gui de Chauliac University Hospital,
Montpellier, 34295 France;
c Pediatric Neurology
Service and INSERM E-9935, Robert Debre University
Hospital, Paris, 75019 France
We evaluated the efficacy of GPi stimulation on the
dystonia of three patients presenting pantothenate kinase
associated neurodegeneration (PKAN) (formerly Hallervorden-Spatz
syndrome). PKAN related dystonia usually
worsens dramatically and becomes life-threatening. Pharmaceutical
therapies are poorly efficient. Our group has
shown long-lasting efficacy of GPi stimulation in DYT1
patients. All three patients (two boys and one girl – mean
age 16 years old) had a typical PKAN clinical history with a
characteristic eye-of-the tiger sign in both pallidum on cerebral
MRI and a progressive generalized dystonia. Pallidal
electrodes were implanted using a Leksell stereotactic frame
with MRI guidance and were activated as previously
reported. Functional improvement of the patients was
assessed using the Burke-Marsden-Fahn dystonia rating
scale (BMFDRS). Patient 1, a boy, was implanted at the
age of 20. Pre and postoperative BMFDRS scores were
respectively 55/120 and 27.5/120 after 9 months followup.
Patient 2, a girl, was implanted at the age of 17. Pre
and postoperative BMFDRS scores were respectively 52/
120 and 21.5/120 after 11 months follow-up. Patient 3, a
boy, was implanted at the age of 10. Pre and postoperative
BMFDRS scores were respectively 86/120 and 55.5/120
after 31 months follow-up. No adverse events were
reported. GPi stimulation provides a long lasting efficient
treatment to limit the progression of PKAN related dystonia.
This procedure is reversible (unlike pallidotomy), it
preserves cognition and remains compatible with other
treatments. Thus, GPi stimulation should be early considered
in such drug-resistant progressive dystonia. The identification
of a pantothenate kinase-based pathogenesis might
provide additional therapies.
FP-S-049
A infantile case of completely treated multiple
intracranial tuberculoma
E.Y. La, C.W. Lee, H. Bang
Department of Pediatrics, WonKwang University College of
Medicine, Iksan, Korea
Although the incidence of intracranial tuberculosis in
children have decreased with development of antituberculous
therapy, mortality rate is high as formerly, if not early
diagnosed and treated. Authors report a case of a 5 months
old male with multiple intracranial tuberculoma who have
been accompanied generalized miliary tuberculosis and
tuberculous meningitis. He was hospitalized at the pediatric
department of Wonkwang University Hospital with mild
fever and persistent cough. The brain MRI was performed
and showed multiple intracranial micronodular densities,
perinodular edema and diffuse leptomeningeal enhancement
which were disappeared completely after antituberculosis
chemotherapy for 12 months and he had completely recovered
without sequale.
FP-T
Others
FP-T-001
Physiological basis and significance of yawning in
clinical practice
K.B. Kulkarni
Sanjivani Nursing Home, Gwalior (M.P), India
Yawning is an index of dopaminergic function. Dopamine,
a precursor of norepinephrine, is converted to norepinephrine.
The hypothalamus, brain stem, reticular
formation are rich with norepinephrine while dopamine is
abundant in nerve terminals of corpus striatum which derive
from cell bodies in the substantia nigra. Corpus striatum is
involved in complex unconditioned reflexes with somatic
and vegetative components; the latter through direct
connections between subcortical nuclei, reticular formation
and hypothalamus. The corpus striatum contains neurons

Abstracts 637
influencing both sympathetic and parasympathetic nuclei of
hypothalamus. Hence, yawning in diverse and opposite
situations like sleepiness and over excitation, hunger and
empty stomach. My decade long observations on yawning
in children from newborn to 12 year age convalescent from
serious illness – shock of any origin, dehydration, convulsive
disorders, meningoencephalopathy and comatose
states, head injury and hyperpyrexia – indicate that yawning
is a favourable sign meaning the worst is over. Yawning is
non-repetitive, once twice maximally thrice, and mother
rarely notices it hence pediatrician’s careful observation is
vital. Tone of their eyeball muscles improves after yawning
and they remain awake taking interest in surroundings.
Those who sleep do so comfortably like normal children.
Yawning should be considered on different lines in normal
healthy children and those convalescent from serious
illness. Is it a conditioned reflex out of internal environment
like temperature and pH changes, changed osmolality and
osmolarity, effect of prostaglandins and hormones? All this
appears to be grey area in the pathophysiology of yawning.
The role of glomus cells of carotid sinus and Herring’s nerve
also is discussed.
FP-T-002
Neurocutaneous melanosis (a case report and literature
review)
Y. Sun, W.-H. Du
Department of Pediaetrics, The People’s Hospital in
Xinjiang, Urumqi, China
Introduction: Neurocutaneous melanosis is an infrequent
condition characterised by the presence of numerous gigantic
cutaneous naevi and melanocytic infiltration of the CNS
and/or the leptomeningeal layers. Different clinical features
may be seen: intracranial hypertension due to hydrocephalus,
cranial nerve paralysis, myelopathy, seizures, etc. The
prognosis is considered to be malignant. Case report: A7-
year-old boy with widespread pigmented nevi, with benign
self-limiting epilepsy in early childhood and ataxia. He was
followed up for 6 years. Cerebral CT and MR scans were
abnormal. Serial magnetic resonance imaging scans demonstrate
T1 shortening and T2 shortening signals in the hippocampus
and pons, with atrophy of pons and cerebellum.
Conclusions: The clinical course of this boy was relatively
benign. MRI should be done periodically.
FP-T-003
Transient parkinsonism following the ingestion of tea in
a child with familial idiopathic cerebral calcification
H. Yoshikawa, S. Yamazaki, T. Abe
Department of Pediatrics, Niigata City General Hospital,
Niigata, Japan
We report here a normally developed 12-year-old girl with
familial idiopathic cerebral calcification (FICC), who exhibited
transient parkinsonism (TP) over a period of 20 days
following the ingestion of tea. The patient drank a cup of
tea during her elementary school graduation party, this was
her first experience of drinking tea. The next morning, symptoms
which suggested the condition known as parkinsonism
developed, these included progressing rigidity, akinesia, a
mask-like face and dysphagia. These symptoms gradually
worsened. Although routine laboratory examination
revealed no abnormal findings, brain CT revealed multiple
calcification. No other specific etiology was proven, but a
brain CT of her older sister with no symptoms revealed similar
multiple calcification. Therefore, she was diagnosed as
having FICC. After the 6th day following the onset of her
symptoms, her extrapyramidal symptoms gradually faded
and by the 20th day they had disappeared completely.
Patients with FICC have been reported as displaying various
movement disorders, such as parkinsonism or paroxysmal
dystonic choreoathetosis (PDC). Although caffeine is
known to have a possible protective effect against parkinson’s
disease, the TP which developed in this patient could
conceivably have been caused by the ingestion of caffeine,
which was compounded by emotional excitement and fatigue.
On the contrary, caffeine induced PDC, which is different
condition to the one reported on here. There have been no
reports of TP induced by caffeine in FICC, and the mechanism
which caused TP in this patient remains unclear.
FP-T-004
The influences of methylprednisolone pulse therapy on
eurological tatus in children with nephrotic syndrome
C.-Y. Chen, D.-K. Chen
Capital Institute of pediatrics, Beijing, China
Objection: To evaluate the impact of pulse methylprednisolone
treatment (PMT) on the mental and neurological
status in the children suffering from NS. Method: Twentytwo
administrated NS patients needed PMT. We recorded
the change of their stiffness, Babinski sign, Kernig sign,
Brudzinski sign, knee jerk, communication and sleepingtime
before and after PMT. The methylprednisolone dose
was 15–20 mg/kg per day and was given through intravenous
infusion within 1.5–2 h daily for 3 days. Results: No
patients had any changes except one complain mild headache.
Conclusion: PMT is safe for NS children if the dose of
methylprednisolone and the duration of PMT are proper.
FP-T-005
The analysis of detecting in children vascular headache
by transcranial Doppler ultrasound (TCD)
Y. Chen, J.-J. Zhang, S.-H. Li
Harbin Children’s Hospital, Harbin, China
Purpose: To explore the clinical significance of TCD in

638
Abstracts
detecting children vascular headache. Methods: TCD,
TC2020 made in EME Company of Germany was carried
out in 132 patients of children with vascular headache.
Recording the Vm of their MCAACAPCAVA BA and
their PI. Results: In 132 patients detected with TCD128 is
abnormal. The abnormal rate is 96%. The mean blood flow
velocity (Vm) of the basal cerebral arteries is abnormal 104
is high (81%) 24 is low (19%). A total of 936 blood vessels
of 104 children whose Vm is high was detected in total.
MCA is abnormal in 176 of them ACA is abnormal in
141 PCA is abnormal in 73 of them VA is abnormal in 60
of them BA is abnormal in 43 of them. The abnormal rate is
MCA (19%) . ACA (15%) . PCA (8%) . VA
(6%) . BA (4.5%)in turn. A total of 1152 blood vessels
of 132 patients was detected by TCD in total. PI is abnormal
in 34 of them including 14 of them is lower and 20 of them
is higher and the changes of them is not regular. Conclusion:
TCD as a method to detect children vascular headache without
damage simple and reliable. It has the great clinical
value for the diagnose classification and observing the effect
of treatment.
FP-T-006
Diagnosis and follow-up of acute high level cervical
myelopathy in children
B.-M. Wu, H. Wang, W. Zhang
Department of Pediatrics, The Second Clinical College,
China Medical University Shenyang, China
High level cervical myelopathy is uncommon, which
may be clinically manifested as dyspnea and high fever
in addition to symptoms as caused by lesions of spinal
cord. In present paper, 30 cases diagnosed as high cervical
myelopathy by laboratory tests of CSF and spine MRI and
intervened in our department from April, 1989 to February
were reviewed. Eighteen were male and 12 female with
age ranging from 11 months to 14 years old. Of the
patients, 21 patients were diagnosed as acute myelitis,
five as anterior spinal artery syndrome, two as hemorrhage
of the spinal cord, two as extramedullary arachnoid cyst.
All the patients showed abrupt onset with tetraparesis,
sensory disturbances, bowel and bladder incontinences.
Eighteen patients had dyspnea, and 14 had fever at the
early stage of the conditions, among whom five cases
manifested as persisted high fever with flush countenance
and dry skin. In 24 patients experienced spine MRI, 14
patients exhibited swelling of cervical spinal cord, two
hemorrhage of the spinal cord, and two extramedullary
arachnoid cyst. Six revealed normal images. In 18 patients
received 1-year follow-up spine MRI scan, six retained
normal, 12 showed slight spinal atrophy. Physical examination
in clinical follow-up showed that 16 patients had
muscle atrophy, upper limb dyskinesia of different degrees,
and normal lower limb.
FP-T-007
Long term follow up after acute stroke in childhood, a
retrospective study
M. Steinlin, K. Roellin, G. Schroth
University Hospital Inselspital, Bern, Switzerland
There are only few studies concerning long term neurological
and neuropsychological outcome of children after
acute stroke, but revealing significant problems. Methods:
Retrospective chart review followed by a questionnaire and
clinical examination, concerning initial presentation,
aetiology and long term outcome of children suffering a
stroke between 1985 and 1999, retrieved by a computer
search at the University Children’s Hospital of Bern.
Results: Twenty-three children (15 boys) suffered 20
acute arterial ischaemic events, two sinuous venous thrombosis
and one combination. Aetiology was detected in 15,
suspected in six and unknown in two. Follow up after 1–15
years (median 7 years) was possible for 18 children, three
had died and two were lost to follow. Only four were
completely healthy, five suffered mild, six moderate and
three severe handicap, 11 children presented combined
neurological and neuropsychological problems. Neurological
problems were mild to moderate hemisyndrome in 11,
dysphasia, epilepsy and others in six each. Mild to severe
neuropsychological problems were detected in 14 children,
school problems in nine, attention deficits in ten and behaviour
problems in seven. Headache and increased fatigue in
six each. Recurrence was observed in three, all due to
progressive underlying disease. Outcome was most
affected by combined cortical/subcortical and best with
only subcortical infarction. Size of infarction showed a
tendency to influence prognosis. Epilepsy affected neuropsychological
outcome. Conclusion: Neurological and especially
neuropsychological problems influence the life of
children after acute stroke significantly. Careful long
term follow up is mandatory and more prospective and
exact data on outcome are necessary.
FP-T-008
Sea blue histiocytosis with progressive neurologic
damnification in three cases
C.-H. Ding
Beijing Children Hospital, Beijing, China
Objectives: The cardinal clinical findings of sea blue
histiocytosis (SBH) patients were hepatosplenomegaly,
anemia and thrombocytopenia. The diagnosis and differential
diagnosis of SBH with chronic progressive neurologic
damnification were discussed. Methods: Clinical and
laboratory examinations were performed in three cases
with chronic progressive neurologic damnification. Results:
The clinical findings of three patients were mental deterioration,
paresis of vertical gaze, speaking slowly and not

Abstracts 639
clear, and unsteady walking. The second and third one had
difficulty in swallowing and torsion dystonia. The second
and third one were sisters. Physical examination for three
patients revealed dysarthria, ataxia, paresis of vertical gaze,
pyramidal and cerebella signs were positive. The second
and third one had mild splenomegaly. The serum ceruloplasmin
level was normal. The antibodies of measles virus
and rubella virus were negative in cerebrospinal fluid.
Examination of bone marrow revealed sea blue histiocytes,
no others specific findings. Liver function studies of the
first patient were slightly abnormal. The serum lactic
acid and pyruvate levels were abnormal in the first one.
Conclusions: The features of SBH with neurologic damage
were progressive psychomotor deterioration with vertical
gaze paresis, liver function abnormal, and splenomegaly.
SBH with neurologic damnification should be distinguished
from Wilson’s disease, demyelinating disease,
mitochondrial encephalomyopathy, and slow virus infection
of central nervous system.
FP-T-009
Magnetic stimulation and new method of study of a blink
reflex arch functional condition at children
A.G. Remnev
Altai Diagnostic Center, Barnaul, Russia
The purpose of research: to estimate a functional condition
of a trigeminal-facial complex (TFC) – blink reflex
arch at children. Research of a functional condition of a
blink reflex arch carried out by electrical stimulation (ES)
of a supraorbital nerve and transcranial magnetic stimulation
(TMS) of a cerebral cortex. The caused answers
registered at a level of the eye circular muscles. At realization
of researches used a complex of the equipment:
Magstim-200 (Magstim, UK), Sapphire 2M (Medelec,
UK). We investigated 45 healthy children with an age
between 5 and 15 years: 16 children with an age between
5 and 9 years (1st group), 29 children with an age between
10 and 15 years (2nd group). The received results testify
that TMS was characterized by steady registration of
components of a blink reflex – early (R1) and late (R2).
Latency of the R2 was similar at ES and TMS; latency of
the R1 at ES and TMS had difference. The latency of the
R1 at TMS was more, than at ES (13 % on the average).
The distinction in latency of the R1 can be explained by
distinction of the reflex channels, on which there is a
realization of excitation at TMS and ES. Thus, the noninvasive
technique of the TMS can be useful at an estimation
of a functional condition of various sites of a TFC
(facial nerve, trigeminal nerve and cerebronuclear tracts of
a facial nerve).
FP-T-010
Auditory early and long latency evoked potentials in
children and adolescents with headache
M. Zgorzalewicz, B. Zgorzalewicz, R. Nowak
Chair and Department of Developmental Neurology,
University of Medical Sciences, Poznań, Poland
Auditory evoked potentials were studied in 60 children
and adolescents in 8–18 years old with different types of
headache. The examination was carried out in interictal
phase of the disease. Diagnoses of disease were performed
according to the categorization of IHS. The aim of the study
was to investigate the changes in BAEP and auditory ERP in
migraine and non-migraine headache. The obtained results
were compared with healthy controls at the matched age.
The evoked potentials were recorded by Multiliner (Toennies-
Germany) according to the IFCN standards. For BAEP
latencies of waves I–V and inter-peak I–III, III–V, I–V were
measured. The ERP was recorded with the oddball paradigms.
The following parameters of ERP were measured.
The N1, P2 latencies were evaluated from the responses to
the non-target stimuli. The latencies of N2 and P300 parameters
were estimated to target tasks. The inter-peak amplitudes
were measured too. In majority of migrainous subjects
prolongation of ERP latencies was found. No modifications
of the P300 components were found in the tension-type
headache subjects. There were also no differences in
BAEP parameters between children with headache and the
control group. The results were correlated with the clinical
picture of the disease in the examined patients. An impairment
of memory and attention processes in migrainous
patients based on neurophysiological results was found.
The ERP is a non-invasive diagnosis tool in young patients
with headache when signs or symptoms may not be characteristic.
FP-T-011
Encephalopathy with intracranial calcification,
dwarfism, leucodystrophy and neuropathy: a new
clinical entity?
F.M. Aynacı a , F. Celep b , A. Ahmetoglu c
Karadeniz Technical University, Faculty of Medicine,
a Department of Child Neurology, b Genetics and c Radiology,
Trabzon, Turkey
Microcephaly, motor and mental retardation, cataract
(two cases) dwarfism and intracranial calcification occurred
in five cases in large consanguineous families between 3 and
6 months of age. The patients died between 4.5 and 8 years
of age variably. Detailed investigation was done for two
cases. Neither pleocytosis in CSF nor parathormon deficiency
was determined. Peripheral neuropathy was determined
in both. Muscle biopsy was performed in one case
and any mutation for MELAS, MERRF, Leigh diseases was

640
Abstracts
not determined. Photosensitivity and pigmental retinopathy
were not described in all cases. We think that these patients
most resemble the first group of Fahr syndrome. But,
compared with progressive intracranial calcification cases
in the literature, we found in our cases autosomal recessive
inheritance, early onset, severe pectus carinatus and dwarfism
without growth hormone deficiency, cataract, peripheral
neuropathy and progressive calcification without CSF pleocytosis.
FP-T-012
Neurologic complications of cyanotic congenital heart
disease
O.M. Luz
University of Santo Tomas Hospital, Quezon City, Philippines
Objective: This paper aims to identify the neurologic
complications associated with cyanotic congenital heart
disease and characterize the lesions in relation to different
variables. Methodolegy: Retrospective study of patients 18
years and younger with cyanotic congenital heart disease
who presented with neurologic complications. Conclusion:
Most patients presented with cerebral abscess and cerebral
infarct (45% each). Infarcts tend to be multiple and
abscesses tend to be solitary with predilection for the parietal
area. Alteration of consciousness and seizures carries a
grim prognosis.
FP-T-013
Selected risk factors in ischaemic strokes on migraine in
children
E. Pilarska a , A. Bakowska b , A. Kubik c , S. Kroczka c
a Department of Developmental Neurology Medical University
of Gdańsk; b Department of Immunopathology Medical
University of Gdańsk; c Department of Pediatric Neurology
Faculty of Medicine, Jagiellonian University of Kraków,
Poland
Aim: The investigations were made to pronounce the
value of examination of antiphospholipid antibodies and
thrombomodulin in explanation of the pathogenesis of
ischaemic strokes and migraine. Material and method:
We investigated 30 children, who suffered ischaemic
stroke at the age range between 2 and 15 years and 30
children with migraine aged 8–15 years, 19 had migraine
with aura and 11-without aura. The control group consisted
of 30 healthy children at the some age. Anticardiolipin
antibodies aCl -IgG, IgM, IgA and b2 glycoprotein and
thrombomodulin were evaluated. Results: Both in ischaemic
strokes and migraine there were increased concentrations
of thrombomodulin and antiphospholipid antibodies
compared to control group. The highest values were
observed in children with ischaemic strokes. In none case
there were increased b2-glykoprotein levels. Conclusion:
Anticardiolipin antibodies and thrombomodulin, especially
aCl- IgG, IgM maybe the important causative agents in
childhood strokes and migraine. This paper was supported
by a grant No 4PO5E 150 18 of the State Committee of the
Scientific Research.
FP-T-014
Event-related potential N270 and its distribution in
adults and school-age children
L.-P. Li, Y.-P. Wang, H.-J. Wang, L.-L. Cui, S.-J. Tian
Department of Neurology, Xuanwu Hospital, Capital
University of Medical Sciences, Beijing, China
To compare event-related potential N270 and its scalp
potential distribution in adults and in school-age children,
and to explore the development of conflict processing
system in human brain, pairs of colored numbers were
sequentially present on screen to subjects tested. They
were instructed to discriminate whether the color or magnitude
of the pairs of number was identical, and ERPs were
recorded at the same time. In adults, a negative potential
peaking at 270 ms (N270) was elicited when the second
stimulus (S2) conflicted with the first stimulus (S1) of task
relevant and irrelevant attributes. N270 was widely distributed
on the entire scalp in adults. In children, N270 was
only elicited by conflict in task-related attribute, the distribution
of N270 in color conflict was on bilateral sides of
the scalp and N270 elicited by magnitude conflict was on
the left central-frontal site. Conclusion: In school-age children,
event-related potential N270 was elicited under the
control of attention. Its distribution in scalp depends on the
attributes of stimulus pairs.
FP-T-015
Questionnaire study: what is the role of child
neurologists who advise caregivers of disabled students
at special schools?
M. Aoyama a , M. Shibata a , M. Yamamoto a , O. Nitta a ,K.
Miura c , M. Miyao d , Y. Iwasaki e
a School of Nursing, b School of Physical Therapy, c School of
Occupational Therapy Tokyo Metropolitan University of
Health Sciences;
d Division of Develop. Psychology,
National Children’s Medical Care Center; e Division of
Child Neurology, Tokyo Metropolitan Yotsugi Rehabilitation
Center, Japan
In 2000, we administered a questionnaire study to
parents of neurologically disabled students. The questionnaire
addressed such issues as medical, co-medical and
social problems, all pertaining to neurologically disabled
students. We found that some caregivers, mainly mothers,
complained about poor explanations from doctors on the
future outcomes of their children. They were also

Abstracts 641
concerned about how to properly raise their children under
the given circumstances. We were interested in what the
doctors thought about these issues in 2001, so we conveyed
a questionnaire study again. We collected 137 responses
out of 202 child neurologists, who are all councilors with
the ‘Japanese Society of Child Neurology’. A total of 81%
of doctors ranged from the ages of 41 , 60 and 78% had
over 20 years experience. Fifty-nine percent were clinical
doctors, 64% of them examined the children between 10
and 20 min (per child) and 82% of the doctors worked over
twice a week at the outpatient clinic. Twenty-five percent
of doctors indicated that their explanations were insufficient
to please the caregivers, and 20% of doctors worried
about the inadequate satisfaction of the parents. A total of
83% of them hoped to ask other staff, chiefly clinical
psychologists, to help them with children’s care.
FP-T-016
Electroencephalogram as a follow-up indicator in
pediatric moyamoya diseases
D.-S. Kim a , T.-S. Ko a , Y.-S. Ra b , C.-G. Choi c
a Department of Pediatrics, b Neurosurgery and c Radiology,
Asan Medical Center, University of Ulsan, College of Medicine,
Seoul, Korea
Moyamoya disease is one of the most common cerebrovascular
diseases in childhood. The EEG is known to
show the characteristic ‘rebuild-up phenomenon’. In
order to evaluate the usefulness of EEG as a follow-up
test, we performed the statistical analysis between clinical
improvements and EEGs after indirect anastomosis. Total
31 patients below 15 years old, undergone the surgery
from March 1995 to March 2001, were included. The
ratio of male to female was 1.58:1 (19:12) and the median
age was 6 years. The clinical manifestations were TIA
(n ¼ 27), seizure (n ¼ 8), hemiplegia (n ¼ 4), and headache
(n ¼ 4). The analysis for the total patients did not
show statistical significance between clinical and EEG
improvements (P ¼ 0:142). However, the analysis only
for cases with the initial abnormal EEG (n ¼ 23) did
show significance (P ¼ 0:04). In addition, we performed
the correlation analysis. We divided clinical and EEG data
into three groups: (a) no; (b) mild; and (c) marked
improvements, respectively. For the cases with abnormal
initial EEG findings (n ¼ 23), we found the statistical
significance between the clinical and EEG improvements
(r ¼ 0:466, P ¼ 0:025). Our results suggest the EEG can
be used as a non-invasive follow-up test after the bypass
surgery in Moyamoya disease, when the initial EEG was
abnormal.
FP-T-017
Ischemic stroke in a pediatric population risk factors
and clinical aspects
A. Schteinschnaider, C. Romero, F. Meli, R. Lagos, S.
Ameriso, S. Intruvini, J. Pociecha, M. Szlago, M.G.
Alvarez, M. Segalovich, G. Garate, M. Massaro
Raúl Carrea Institute of Neurological Research, FLENI,
Buenos Aires, Argentina
Objective: Ischemic stroke are relatively uncommon in
children and their etiology may differ from the adult population.
We report the characteristics of all pediatric strokes
seen at our institution in a 6-year period. Background:
Ischemic stroke are being more frequently diagnosed in
childhood in part due to greater physicians awareness and
also due to the development of non-invasive diagnostic
procedures such as magnetic resonance angiography, transcranial
Doppler or molecular genetic techniques. Higher
survival rates of patients with cardiological or oncologic
diseases allows for increased time for exposure to specific
risk factors. Design/methods: This was an observational,
retrospective study of institutional chart reviews of every
patients with a discharge diagnosis of ischemic stroke
between January 1995 and December 2000. Results: During
the time period studied there were 21 patients with a diagnosis
of ischemic stroke. There were four girls and 17 boys
aged 6.5 years (1 month–16 years). Acute hemiparesis (16)
was the most frequent clinical presentation followed by
altered consciousness (6), headache (3), and ataxia and/or
gait disturbances (3). Sixteen patients (76%) had a predisposing
risk factor: congenital heart disease (4), prothrombotic
state (4), vascular abnormalities (3), and traumatic
carotid dissection (3). Four patients had a positive family
history of stroke. There were no stroke related deaths and
only two patients suffered recurrent stroke. Twelve patients
had secuelar neurological deficit. Conclusions: Ischemic
stroke continues to be uncommon in the pediatric population.
Nevertheless the cryptogenetic group has certainly
diminished based on a complete and detailed work-up.
Most patients have at least one predisposing factor. A thorough
investigation of potentially treatable causes of the
stroke is warranted and may have been responsible for the
low recurrence rates observed.
FP-T-018
Auditory induced somatosensory cortex activations
identified by magnetoencephalography (MEG) in
patients with cortical lesion
M. Kubota, Y. Sakakihara, H. Hirose, I. Kimura
Department of Pediatrics, University of Tokyo, Tokyo,
Japan
A single kind of stimulus usually activates a single specific
sensory cortex such as the auditory cortex activation by tone

642
Abstracts
stimulation, but it rarely activates multiple sensory cortices
in various conditions. Anatomically, reciprocal connections
between different cortices is known to be involved in various
information processing. We here investigated the pathophysiological
significance of auditory induced somatosensory
cortex (SI) activation in patients with cortical lesions or
dysfunction. Subjects were two patients with regional encephalitis,
one with juvenile myoclonic epilepsy (JME) and
four with focal epilepsy. Using MEG, we analyzed SI activation
in auditory evoked magnetic fields which was induced
by 1 kHz/90 dB pure tone stimuli. At first, all patients showed
bilateral responses of the primary auditory cortex (AI) ,100
ms after the stimuli (N100m). In addition, the SI activation
was found in both hemisphere in patients with regional encephalitis
and JME, and in the same hemisphere as interictal
paroxysm in patients with focal epilepsy. Our findings
suggest that unmasking of normally-inhibited inputs from
AI to SI occurred after encephalitis or epileptogenic dysfunction
(intercortical reorganization). This is the first report of
auditory induced SI activation in human, although the precise
mechanism of the multimodal sensory activations is not
known.
FP-T-019
Regional variations in bone mineral density in children
with cerebral palsy using dual energy X-ray
absorptiometry
J. Shiragaki a , N. Iwasaki b , J. Nakayama b , K. Fujita c ,T.
Ohto b , A. Matsui b
a Kyushu University of Health and Welfare, Nobeoka, Miyazaki
Japan; b Department of Pediatrics and c Institute of
Disability Science, University of Tsukuba
BMD was studied in 34 children with spastic cerebral
palsy (CP group), 40 children undergoing anticonvulsant
therapy (epilepsy group), and 29 normal children (control
group). The CP group was divided into four groups according
to gross motor function. Dual energy X-ray absorptiometry
was performed to evaluate BMD of the whole body, of the
head, upper limbs, ribs, spine, pelvis, and lower limbs separate
from the whole body scan images, and of the lumber
spine separate from the lumber vertebrae images. In the
control group, BMDs increased with age. There was no
significant difference between the control group and epilepsy
group (P . 0:05). The CP group had BMD lower than the
control group for the whole body, upper limbs, ribs, spine,
pelvis, lower limbs, and lumber spine but not for the head
(P , 0:001). In the CP group, BMD Z scores for the pelvis,
lower limbs and lumber spine were remarkably lower than
for the head, upper limbs, ribs and spine. BMD of the upper
limbs and spine were particularly lower in the bed-ridden
group compared with the standing/walking group, whereas
BMD of the lower limbs were lower in the bed-ridden, rolling,
and crawling groups compared with the standing/walking
group. These results suggest that the high incidence of
femur fractures in CP is related not only to mechanical stress
overload on the lower legs but to the strongly diminished
BMD of the lower limbs and pelvis as well. And that BMD
of the lower limbs and pelvis are also associated with standing
posture and walking.
FP-T-020
The importance of rectal biopsy in the diagnosis of
neuronal intranuclear hyaline inclusion disease (NIHID)
R. Kulikova-Schupak a , K.G. Knupp a , J.M. Pascual a , S.S.
Chin a , R. Kairam b , M.C. Patterson a
a Division of Pediatric Neurology, Columbia University,
New York, NY, USA; b Bronx-Lebanon Hospital Center,
Bronx, NY, USA
NIHID is a rare neurodegenerative disorder of childhood
onset characterized by the presence of eosinophilic intranuclear
inclusions and neuronal loss throughout the nervous
system. Familial occurrence has been described. We identified
two patients, an 11-year old boy (P1) with new onset
bulbar weakness and Parkinsonism, and a 15-year old boy
(P2) with severe cognitive and motor deterioration of uncertain
etiology. P1 presented at 10 years of age with progressive
dysarthria, drooling, hand tremor and psychomotor slowing.
Key physical findings included saccadic pursuit, impaired
convergence, dysarthria, hypophonia, atrophy of tongue and
small hand muscles, increased tone, brisk reflexes, and electrophysiologic
evidence of chronic denervation. P2 was initiallyevaluated
at4yearsofage foragaitdisturbance andmotor
slowing. Examination revealed drooling, sparse speech,
dynamic equinus, titubation, hypertonia with hyperreflexia
and extensor plantar responses. Cognitive decline, loss of
head control, development of spasticity and myoclonic jerks
followed. At 15, the patient was unresponsive to voice, visual
or painful stimuli. He had absent gag and corneal reflexes,
diffusemuscleatrophyandspasticquadriplegia.Bothpatients
had a history of behavioral problems marked by frequent
temper tantrums. Both had non-diagnostic MRI imaging of
the head and metabolic panels. Rectal biopsy demonstrating
characteristic findings in the neurons of the myenteric plexus
was diagnostic 1 (P1) and 11 years (P2) after the initial evaluation.
Rectal biopsies should be considered in children with
otherwise unexplained multisystem degeneration, particularly
in the presence of both upper and lower motor neuron
signs accompanied by behavioral problems.
FP-T-021
Correlation between visual function and acuity in
children with severe mental retardation: a double blind
randomized cross-over controlled trial utilizing a visual
function checklist
K.K.T. Leung a , C.H. Ko b , C.Y. Ko c , L. Chia c , C.C.H. Lo b ,
P.W.T. Tse b
Tuen Mun Child Assessment Centre, a Department of Health,

Abstracts 643
Hong Kong; b Developmental Disabilities Unit and c Department
of Ophthalmology, Caritas Medical Centre, Hong
Kong, China
Introduction: Refractive errors is one of the major ocular
defects in children with severe mental retardation. While
their visual acuity may improve significantly after prescription
of corrective lenses, it is not clear whether this is associated
with prompt functional and behavioral improvement.
The latter may be assessed by the visual function checklist
(VFC), which is an innovative behavioral tool to assess the
child’s response to light perception, abilities of visual
exploration, fixation, following, distance viewing, grabbing,
orientation, and the presence of optokinetic nystagmus.
Methods: The subjects included ten children with severe
grade mental retardation and significant refractive errors.
They were randomly allocated to wear corrective and
plano lenses. They were then assessed by a developmental
paediatrician with the VFC. Subsequently, children from the
plano lens group crossed over to wear corrective lenses,
while those originally in the corrective lens group changed
onto plano lenses. The assessor, who was blinded to the
treatment allocation, would perform a second evaluation
with the VFC. The assessment was conducted in standardized
settings. The results were converted into a VQ, with a
range from 0 to 1. The mean VQ from both groups were
compared by paired samples t-test. As it was assumed the
visual function might improve or remain the same after
corrective lenses, the significance level was set at a one
tailed probability of ,0.05. Results: The mean VQ for the
corrective lens group and plano lens group were
0.829 ^ 0.304 and 0.789 ^ 0.313, respectively. There was
significant prompt improvement in the visual function of the
subjects after improvement in visual acuity (one tailed
P ¼ 0:045). Discussion: The correction of ocular defects
in children with severe mental retardation may be associated
with functional improvement. The VFC is complementary
to conventional acuity tests in monitoring the functional
improvement.
FP-T-22
Analysis and evaluation of visual-motor integrated
coordinating motion in children -a preliminary result
C. Chen a , S.-C. Chen a , C.-H. Yu a , J.-H. Yeh a , J.-H. Lai a , C.-
L. Chen b
a Department of Physical Medicine and Rehabilitation,
Taipei Medical University Hospital, Taipei, Chinese Taipei;
b Department of Physical Medicine and Rehabilitation,
Chang Gung Memorial and Children Hospital, Taoyuan,
Chinese Taipei
The pathophysiology of clumsy hand function in children
is strongly related with the development of visual-motor
integration. In 1967, Berry developed the Beery-Buktenica
developmental test of visual-motor integration (VMI) as an
assessment tool to evaluate the integration ability of visual
perception and motor coordination. Our study is designed to
analyze the motion of selected functional task during daily
life, e.g. spoon manipulation, which demands desirable eyehand
coordination for the children based on VMI test
results. Meanwhile, the comparison with normal children
will be carried out. We collected three children from 3 to
6 years old. They are examined with VMI test following two
additional tests (visual perception and motor coordination).
Furthermore, they are instructed to perform a selected functional
task in activity of daily living (ADL) by using a spoon
to transport objects in four directions (horizontal, vertical,
right downward oblique and left downward oblique). We
recorded the kinematic values including movement velocity,
trajectory and the displacement corresponding to the
straight line between target and initial positions. We found
the child with poor visual-motor integration made more
errors and the one who had pure motor coordination
performed the task with more slow velocity compared
with the normal child. However their movement trajectory
made no significant differences. This preliminary result
offered the clinical physician and therapists a guideline for
further training program in children with problems of visual
motor integration or motor coordination. Further recruitment
of subjects for more convinced conclusion was
required.
FP-T-023
How are fathers first informed about the disabilities of
their children and how do they accept the facts?
K. Miura a , M. Aoyama b , M. Shibata b , M. Yamamoto b ,O.
Nitta c , M. Miyao d , Y. Iwasaki e
a School of Occupational Therapy,
b School of Nursing,
c School of Physical Therapy Tokyo Metropolitan University
of Health Sciences;
d Division of Develop. Psychology
National Children’s Medical Care Center,
e Division of
Child Neurology, Tokyo Metropolitan Yotsugi Rehabilitation
Center, Japan
Purpose: This research was to investigate how fathers are
first informed about the disabilities of their children, and
how accept the facts. Subjects: Fifty-four fathers ranging
from 31 to 65 years (mean age: 45.6 years) whose children
have physical disabilities (CP, PMD, etc.) and are from 6 to
18 years old (mean age: 12.6 years) at a special school.
Method: Fathers answered a questionnaire developed from
a research project team at Tokyo Metropolitan University of
Health Sciences. Results: Most of the fathers were informed
of the disabilities of their children before the children were 1
year old, and the fathers were in the company of their wives
and doctors. Most of them responded like this: ‘I have no
actual feelings’, ‘it is a great shock’ and ‘I am greatly disappointed’.
One father felt that the ‘doctor did not think about
the parents well-being’. The period of acceptance ranged
from 10 months–11 years (mean period: 15 months). Half

644
Abstracts
of the fathers could accept the facts in a year, and for a
quarter of them, it took over 2 years. Conclusions: According
to another survey made by our project team for mothers
having children with physical disabilities, we found a similar
result concerning the period of acceptance. More social
support systems should be implemented for fathers and
mothers.
FP-T-024
The modified paediatric coma scale as prognostic
indicator for immediate outcome in acute non-traumatic
encephalopathy
C.P. Wong a , J.A. Eyre b
a Department of Paediatrics, UMMC, University of Malaya,
Kuala Lumpur, Malaysia b Paediatric Neuroscience Group,
Sir James Spence Institute of Child Health, University of
Newcastle upon Tyne, Newcastle upon Tyne, United Kingdom
The study examined the prognostic significance of the
James’ modification of Glasgow Coma Scale in children
with acute non-traumatic encephalopathy. Children aged
below 5 years who were admitted into the non-traumatic
coma study in the North of England were recruited for this
study. The coma score on admission, the minimum coma
score and the duration of significant encephalopathy were
assessed for association with the outcome at 6 weeks after
the illness. Minimum coma score and duration of encephalopathy
correlated well with the outcome. Coma score on
admission was not found to be a significant prognostic
indicator. A low coma score on admission is however
specific but not sensitive in predicting death.
FP-T-025
Ischemic stroke in Thai children
A. Visudtibhan, S. Chiemchanya, D. Kitiviriyakul, A.
Chuansumrit, P. Visudhiphan
Division of Neurology, Department of Pediatrics, Faculty of
Medicine, Ramathibodi Hospital, Mahidol University,
Bangkok, Thailand
Background: The diagnosis for etiologies of ischemic
stroke in children has been improved with the development
of newer imaging and metabolic screening techniques.
Objective: To evaluate the etiologies, clinical courses and
outcomes of ischemic stroke in children. Method: A retrospective
study was conducted at Ramathibodi hospital.
Medical records of children aged below 15 years with
ischemic stroke during January 1st, 1992–December 31st,
2000 were reviewed. Children with trauma or central
nervous system infection were excluded. Results: Fortyone
children (19 girls, 22 boys) were included. Mean age
was 5.08 years (range 2 months–14 years). The most
common sites of stenosis or occlusion were the middle
cerebral artery or internal capsule area (73%). Stroke
subtypes were as follows: ten with Moyamoya disease,
five with heart disease with embolic phenomenon, 12
with undetermined causes. There were 14 children categorized
into inconclusive group who had abnormal coagulogram
or coagulating factors such as protein C, protein S,
antithrombin III. Among these children, five also had
cardiac anomaly. Owing to the lack of repeated blood
tests and none in this group had recurrent stroke, specific
diagnosis of hypercoagulation as the cause of stroke was
not possible. There were recurrences in three children (two
with Moyamoya disease, one without definite cause).
Conclusion: Ischemic stroke in Thai children was not
different from other studies. Abnormal coagulation might
be one of contributing factors of ischemic stroke in children.
Complete laboratory investigations in those without
definite cause of stroke should be performed to exclude
treatable cause of stroke in children.
FP-T-026
A rare cause of cerebral arterial stroke in childhood:
non-compaction of myocardium
S. Hascelik, D. Yalnizoglu, G. Kafali, A. Celiker, M. Topcu
Hacettepe University Children’s Hospital Departments of
Pediatric Neurology and Cardiology, Ankara, Turkey
Non-compaction is a developmental disorder of cardiogenesis
in fetal life characterized by prominent trabeculations
and recesses between them. Patients mostly present
with heart failure, arrythmias or thromboembolic events.
We report an 18 month-old girl with non-compaction of
the left ventricle presenting with thromboembolic stroke.
Her past medical history was remarkable for mild motor
delay and easy fatigue. Her parents were relatives, family
history was non-contributory. She was admitted to our
intensive care unit with right sided motor seizures, right
hemiparesis and altered mental status. CT scans and MR
imaging showed infarction in the territory of the left middle
cerebral artery (MCA). MR angiography showed occlusion
in the distal part of the left MCA. She was worked up
thorougly for the etiology of stroke. Two dimentional echocardiography
revealed trabeculations and deep recesses in
the left ventricle; a large thrombus was swabbing the whole
left atrium. Follow up cranial CT was obtained at 1 week
which showed stable radiologic findings and no hemorrhage.
The patient was then started on anticoagulant therapy,
and she died on the fourth day of treatment. No further
imaging studies were performed. We conclude that noncompaction
of myocardium is a rare disorder yet may
have severe and fatal complications. Echocardiography is
mandatory in all infants and children for evaluation of
stroke. Early initiation of anti-aggregant therapy may be
considered in children with non-compaction to prevent
future cardioembolic stroke.

Abstracts 645
FP-T-027
Simple reaction time in children with migraine
R. Matijevic, D. Filipovic, J. Mihaljev-Martinov, K.
Boskovic, V. Ivetic, I. Tanackov, S. Bogdanovic
Medicinski fakultet – Novi Sad, Katedra za fiziologiju, Novi
Sad, Yugaoslavia
In this study we assessed simple reaction time data in
children. Sample consisted of 90 children, 30 of whom
had migraine with aura, 30 with migraine without aura
and 30 healthy children aged 10–18 years. All children
were right handed. In the first group of children there
were 19 (63%) girls and 11 (37%) boys. In the second
group there were 23 girls (77%) and seven boys (23%). In
control group there were 16 girls (57%) and 14 boys (43%).
We tested reaction times with right and left hand on audio
and visual stimulus. In all three groups responses with right
and left hand where shorter on audio stimulus than on visual
stimulus and reaction of right hand is shorter than reaction
with left hand on same stimulus. Control group had significantly
shorter all four reaction times (visual stimulus – right
hand, visual stimulus – left hand, audio stimulus – right
hand, audio stimulus – left hand) than both groups with
migraine. Differentiation on base of average values of RT
between groups with migraine was possible only with some
of RT.
FP-T-028
Brain tumors in children. A review of 84 Brazilian cases
A.A. Espíndola, H. Matushita, A. Diament, U.C. Reed
Department of Neurology of the School of Medicine of São
Paulo University, São Paulo, Brazil
From 1970 the incidence of primary brain tumors among
children has increased and environmental factors as well as
an earlier diagnosis by improvement of detection methods
have been considered for explaining it. Along this period,
continuous therapeutic advances have optimized treatment
planning. For evaluating outcome data, we reviewed histological,
clinical and therapeutic aspects in 84 consecutive
children (0–16 years) with primary central nervous system
tumors, attended and followed-up from January/1997 to
May/2001. Tumor was supratentorial in 63.0%, infratentorial
in 30.9%, spinal intramedullary in 3.5% and spinal extradural
in 2.3%. Astrocytic tumors occurred in 34.5%,
followed by medulloblastoma (11.9%), craniopharygioma
(10.7%) and ependymoma (10.7%). The duration of the
symptoms before diagnosis ranged between 1 day and 8
years (mean 9.3 months). Clinically, increased intracranial
pressure manifested in 41.6%, seizures in 17.8%, focal deficits
in 22.6%, visual abnormalities in 20.2%, ataxia in
13.0% and endocrinological changes in 3.5%. Total removal
was achieved in 54.2%, and partial removal in 39.7%. In
respectively 27.3, 38.0 and 20.2%, chemotherapy, radiotherapy
or both were used. Follow-up ranged between 1
month and 4 years and 4 months (mean: 1 year and 6
months). Tumor recurred in 18 patients. At follow-up,
4.7% have died, 50.0% have no tumor and normal neurological
examination; 8.3% have no tumor but neurological
changes; 23.8% have residual tumor but normal neurological
examination and 13.0% have residual tumor and neurological
changes. We concluded that overall outcome has
improved in several childhood brain tumors and the extent
of removal has demonstrated to influence prognosis.
FP-T-029
Asymptomatic cerebrovascular lesions in children with
sickle cell disease
F.N. Arita, S. Rosemberg
Santa Casa School of Medicine, Department of Pediatrics,
Neuropediatrics Division., São Paulo, Brazil
Symptomatic ischemic cerebrovascular disorder is a
severe complication in children with sickle cell disease
(SCD), affecting 6 to 8% of patients within the first 20
years of life. In order to detect clinically silent ischemic
lesions and to determine their prognostic value, we
submitted 28 children with SCD between 5 and 15 years
of age with normal development and without neurological
disease to a unique transaxial T2-weighted MRI. Ischemic
lesions were found in 14 (50%) of the asymptomatic
patients. These were characterized by hyperintense, isolated
or multiple, uni or bilateral punctate or small plaques
images. Five patients presented isolated punctate lesions,
four presented unilateral multiple punctate or small plaques
and five had multiple bilateral lesions. The topographic
distribution of these lesions corresponded to the deep
watershed vascular territories in cerebral white matter.
Two patients with bilateral extensive lesions presented
strokes in the following few years, which recurred in one
of them despite hypertransfusion therapy. A third patient
with extensive lesions presented migraine-like episodes 3
years after the exam. Albeit the control MRI was similar, the
angio-MRI disclosed arterial stenosis and a ’moyamoya’
pattern, and transcranial doppler detected high velocities.
The remaining two patients with bilateral extensive lesions
and the nine patients with less extensive lesions as well as
the 14 patients without lesions remained well after 6 years
of follow-up. We conclude that bilateral and extensive
lesions seem to be an important predictive risk factor for
the development of ulterior cerebrovascular accident in children
with SCD.
FP-T-030
The retrospective study of early diagnosis and EEG
analysis in 31 migraine children
H. Li, X.-Q. Liu, W. Zhou
Qingdao Municipal Hospital, Qingdao Shandong, China

646
Abstracts
Objective: To study the clinical characteristics and
analyse the EEG in children with migraine. Methods: We
reviewed the clinical manifestations and auxiliary examinations
of 31 cases diagnosed as children migraine and
analyzed the EEG abnormality of them. Results: Thirtyone
children were studied, 19 males, 12 females. Headache
occurred bilaterally in 18 cases, and in one side in five cases.
Three cases had auras (10%). Duration of headache varied
from half an hour to 5 h. Headache accompanied with
nausea in eight cases, vomiting in 11 cases, and abdominal
pain in six cases (totally occurred in 80.6% cases). The EEG
abnormalities during attacks and interictal period mostly
were focal and diffuse slow waves or paroxysmal high
amplitude slow waves. Conclusions: There are many
features in children migraine attacks. Clinical manifestations
were the most important aspects in diagnose of children
migraine. Abnormalities of EEG can only be
considered as reference.
FP-T-031
Alternating hemiplegia in childhood: a study of ten
patients
Y.-H. Zhang, W.-X. Sun, Y.-W. Jiang, J. Qin, X.-R. Wu
Department of Pediatric Neurology, Peking University First
Hospital, Beijing, China
Alternating hemiplegia in childhood (AHC) is a rare
disorder of unknown cause. We described the clinical
features of ten patients (nine males and one female). The
age of onset was from 2 days to 55 months (average: 13.2
months). The initial symptoms were abnormal eye movements
or dystonic posturing in eight cases, hemiplegia in
two cases. All patients had recurrent alternating hemiplegic
episodes. The hemiplegia attacks lasted from a few minutes
to 10 days. The occurrence of the attacks ranged from 8
times daily to one time every 2 months. Five patients also
had the episodes of quadriplegia that could be isolated manifestation
or occurred when hemiplegia was shifting from
one side to the other. In eight patients the abnormal eye
movements or dystonia posturing recurred intermittently
during the hemiplegia attack. Sleep could relieve both
weakness and associated paroxysmal symptoms. Mental
retardation was present in seven cases, seizures in two
cases, and dysarthria in three cases. Ataxia and choreoathetosis
presented in one case, respectively. EEG was abnormal
in two patients; other laboratory investigations (such as
brain MRI, cerebral angiography, plasma lactate and pyruvate
levels, et al.) were normal in all patients. Nine patients
received flunarizine therapy. Flunarizine reduced the severity,
duration, or frequency of hemiplegic attacks in six
patients. Our results suggested that AHC was characterized
by frequent episodes of alternating hemiplegia with extrapyramidal
symptoms and mental retardation; flunarizine
was effective in treating some AHC patients.
FP-T-032
The risk of stroke recurrence in childhood
V. Brankvic-Sreckovic, V. Milic-Rasic, N. Jovic, S.
Todorovic
Clinic for Child Neurology and Psychiatry, Belgrade, Yugoslavia
Background: Stroke in children, as an important cause
of morbidity, is challenging for further research. Although
the different aspects of childhood stroke are under investigation,
the recurrence rate is still unknown precisely. It
seems to be lower than previously reported, but highly
correlated with certain etiologies and underlying mechanisms.
Patient and methods: Thirty-five children (21 boys,
16 girls) with diagnosed ischemic infarction, all fulfilling
both clinical and radiographic criteria, were investigated.
Patients with neonatal stroke and SVT were excluded. The
mean follow-up period was 59 months and median age of
stroke onset was 8.4 ^ 4.4 years (1–16 years). Diagnostic
studies were proceeded on a case-by-case analysis to determine
the risk factors associated with stroke. The patients
were analysed according to the etiologic contribution to
initial and recurrent stroke event. Results: Cardioembolic
(12 children-34.3%) and arteriopathic processes (12–
34.3%) were identified as the most probable mechanisms
of arterial ischemic stroke. Prothrombotic abnormalities
were found in four (11.4%) children. Underlying pathology
remained unknown in seven (20%). Cardiac abnormality
prior to first stroke was detected in one child.
Progressive arteriopathies were determined in 3 (8.6%)
and transient cerebral arteriophaty of unknown origin
was found in five (14.3%) children. In four (11.4%) children
recurrent stroke was observed in a period of 4 days–
18 months after the first stroke manifestation. In three of
them (75%), recurrence was due to cardiac or transcardiac
embolism. In the remaining case, the diagnosis of Moyamoya
disease was established (repeated stroke in the same
arterial territory). In one patient (migraine-related stoke)
TIA was noticed before the stroke episode. Clinically
silent cerebral infarcts (multiple lacunar infarcts limited
to one hemisphere) disclosed by MRI preceded the overt
stroke episode in two patients. Antiplatelet agent (aspirin)
has been given in three patients, but no valuable data of
effectiveness could be obtained. Conclusion: The risk
factors of stroke in children appear to be multiple and
overlapping, disabling the precise etiologic diagnosis in
certain circumstances. However, congenital and acquired
heart diseases were the most common cause of repeated
stroke in our study. Better recognition of the underlying
mechanisms and aetiology of childhood stroke is crucial
for both effective therapeutic approach and prevention of
recurrences.

Abstracts 647
FP-T-033
Socioeconomic background of the families with children
in special education
L.E. Åberg a , T. Autti b , M. Mannerkoski a , M. Hoikkala a ,K.
Kuismanen a , H. Heiskala a
a Departments of Child Neurology and b Radiology, Helsinki
University Central Hospital, Helsinki, Finland
Our aim was to determine the socioeconomic background
of the families with children in special education. This
epidemiological study was carried out during the years
1997–1998 in southern Finland. The information was gathered
from randomly chosen 19 school health care units,
representing evenly different districts. The study group
included 900 pupils, 64% boys and 36% girls, born in
years 1980–1993. Out of these 900 pupils, 23% participated
in education modified for specific neurological disabilities,
46% in adjusted education and 30% in training education.
Multinomial logistic regression was used to compare the
size of the families, position of the pupil in the sibling series,
the parental age and socioeconomic status in these different
teaching groups. Our preliminary results show that neither
the family size nor the position in the sibling series differed
in the three teaching groups. As regards the age of the
parents, the fathers of children in the training education
were older as compared to the fathers of children in the
other teaching groups. Also the socioeconomic status of
parents differed, the parental socioeconomic status in the
training education being higher and in the adjusted education
lower than expected. These results indicate that aged
fathers and highly educated parents may be in risk of having
children in the training education, whereas parents with low
socioeconomic status may be in risk of having children in
adjusted education. The present information will also be
compared to the socioeconomic background of the families
with children in normal education.
FP-T-034
Characteric of the neurological signs at the children with
obesity
R.A. Abedimova
Institute of Postgraduate Study, Almaty, Kazakstan
The aim of study was an investigation of neurological
signs at the children with obesity. We observed 197 children
with obesity (97 of them with exogenicconstitutional
obesity (ECO), 100 of them with hypothalamic obesity
(HO)). We used different methods: neurological assessment,
electrophysiological and neuroradiological. Results: We
found some neurological symptoms such us motor delay
which were determined of 86% of children with ECO and
90% of children with HO). There were neuroradiological
changes such us dilatation of subarachnoidal spaces, devastation
of ventriculis of the brain and sub atrophy of frontal
lobe (41% children with ECO; 54% with HO). Electrophysiological
sings (bilateral synchronized wares, hyper
synchronization) were observed at 51% of children with
ECO, and 63% of children with HO. Moreover, after clinical
and tools investigation we were detected neurological
syndromes such as: astenoneurotical syndrome (58%
ECO; 69% HO); hypertension-hydrocephalic syndrome
(43% ECO; 51% HO); syndrome with vegetodystonia
(49% ECO; 55% HO). Conclusion: Therefore, despite on
the clinical forms of obesity, all children had neurological
sings which characterized of diffuse affection of the brain
during perinatal period.
FP-T-035
Instenon administration in children with burn disease
encephalopathy
T.A. Djumabekov, B.K. Nurmagambetova, B.D.
Gurkabaeva, A.V. Zikeeva, S.R. Aitmagambetova
Children’s Hospital, No.1 Almaty, Kazakhstan
Burn trauma in children is an important medical and
social problem. It is the most frequent pediatric trauma
and leads to high level of mortality or handicap. Serous
water and electrolytes dysbalance leads to disturbances of
central nervous system functions, encephalopathy of
hypoxic and toxic genesis. Instenon, is a product of
Nicomed, and there are several active parts in it, which
activate reticular formation, preserves the functional abilities
of neuron complexes of cortex and subcorical structures.
There is Improvement in oxygenation and
phosphorilisation in nerve cells, stabilisation of central
and peripheral haemodynamics, stimulate the vessel moving
center, the center of vegetative regulation and active metabolism
of myocard. This drug was used as part of a complex
therapy of 34 children aged from 6 months to 14 years of age
with burn trauma, complicated by shock and encephalopathy.
All children had had trauma by boiling water and were
hospitalized in 2 h from the trauma moment. The burn area
varied from 10 to 62%. The I degree of burn shock was
diagnosed in 16 children, the II degree in 12 ones, III in
six ones. Antishock therapy was induced immediately.
Central vein catheterization and surgical dress of wound
were held. Infusional therapy was calculated according to
area and daily physiological solutional need by Brooke
formula. All patients underwent treatment using special
aerofluid bed with temperature regulation. Signs of encephalopathy
were: somnolence and consciousness disturbances
in 26 children, coma of I–II degrees in four. All
children had wide and narrow range tremor in all extremitiles
and in chicken. Because of increasing cardiovascular
and respiratory insufficiency and brain hypoxia, four children
were put on artificial lunges ventilation. Instenon was
administrated in children younger then 1 year of age 1 ml in
glucosa solution 5% intravenously one time daily. For the
older patients Instenon was administrated in dose 2 ml in

648
Abstracts
glucose solution 5% intravenously. Treatment course varied
from 5 to 7 days. Outcomes showed positive dynamics of
clinical indexes. A day after treatment started there were
rapid restore of central nervous system functions, then
tendency to consciousness restore appeared, even going
out of coma in 2–3 days. Artificial lungs ventilation restore
breathe and blood circulating. These factors supported water
and electrolytes metabolism, biochemical indexes restore
and transporting blood functions improvement, what manifests
in positive changes of acid – alkaline blood status.
Conclusion: Instenon administration in children with burn
shock, complicated by toxemia, leads to rapid restoration of
consciousness, improves gases transport, prevents brain
hypoxia and hypoxia of other organs and tissues. Administration
of this drug promotes the outcome of patients from
critical status and decreased the time of hospital treatment
for 3 days.
FP-T-036
Ischemic stroke in child with hypoplasia of right
common and internal carotid artery
J.K. Abdulla, M.O. Al Ajmi, S. Slakovic
Paediatric Neurology Unit, Paediatric Department, Al
Sabah Hospital, Al Safat, Kuwait
Although cerebrovascular disorders occur less often in
children than in adults, recognition of stroke in children
has increased because of the widespread application of
non-invasive neuroimaging studies such as CT, MRI and
MRA. The recognized causes of cerebrovascular disorders
in children are numerous and the probability of identifying
the cause depends on the thoroughness of evaluation. We
are reporting a case with Hypoplasia of rt. common and
internal cartoid artery which is recognized as a rare cause
of stroke in childhood. A 3 years old female child admitted
with acute left sided weakness. On examination she had left
sided hemiparesis, left facial palsy, other systemic examination
was normal. Urgent CT head showed hypodense lesion
in the right caudate and right lentiform nucleus suggestive
of brain infarction. Investigations were done excluded
congenital and acquired heart disease, haematological disorders
and coagulopathy, inflammatory, autoimmune and
other systemic disorders. MRI of brain and neck showed
ischemic partially haemorrhagic infarction in rt. paraventricular
mostly basal ganglia due to occluded or undeveloped
rt. middle cerebral artery, rt. ant. cerebral artery. MRA
showed hypoplastic rt. common car. artery, in particular
int. car. artery with occlusion of middle and anterior cerebral
arteries. Accentuated both posterior cerebral arteries
with marked collaterals supplying territory of rt. middle
cerebral artery. During hospitalization, she remained
conscious and she started to show slow gradual improvement
with physiotherapy. Currently, she is having mild left
sided spastic hemiparesis with shortening of the left tendon
Achillis and shortening of left leg.
FP-T-037
Therapeutic effect of parenan and cyproheptadine on
childhood migraine
B.-X. Tang
Pediatrics Department of Central Hospital, Jinshan Shanghai,
China
Objective: To evaluate the effect and safety of Perenan in
treating childhood migraine. Methods: Perenan and Cyproheptadine
were given orally in the period of 1–3 months.
Results: There were 25 children in Perenan treatment group
and 27 in Cyproheptidine treatment group. The effective
rate were 92 and 70.3% in Perenan and Cyproheptidine
treatment, respectively. The difference between the two
groups was statistically significant (P , 0:05). The therapeutic
effect of Perenan was better than that of Cyproheptadine,
and no side effect was observed during the treatment.
Conclusion: Perenan was an effective and safe drug in dealing
with childhood migraine.
FP-T-038
Synthesis and properties of chitosan-metal complexes
S.Sh. Rashidova, N.L. Voropaeva, S. Pulatova, I.N. Ruban
Institute of Polymer Chemistry and Physics Uzbekistan
Academy of Sciences, Tashkent, Republic Uzbekistan
Introduction: Investigation of chitosans interaction with
ions of transition metals and obtaining of water-soluble
polymermetal complexes with the controlled content of
metal ions is an actual task. Its deciding will allow to
propose a new preparations for medicine, agriculture etc.
Results and discussion: Chitosan complex formation with
metal ions of a transitional series (Co, Ni, Cu, Mn) have
been investigated. Estimation of changes in the spectra of
chitosan and its metal-complexes testify that all main
changes take place in a region of stretching and deformation
vibrations of amide bond (Amide-I, -II and -III). It is
accounted well, because chitosan metal-complexes synthesis
was carried out in an acid media and chitosan aminogroup
is protonized. Therefore the most preferable interaction
is an amide bond of samples chitin residue. In this case
a sharp change of Amide-I and -II intensity is observed,
depending on metal ions content, that can be used in a future
for metal content estimation in the samples. Potentiometric
estimation of chitosan interaction with metal ions evidences
of significant complex-formation in a region of neutral pH,
when an initial polymer precipitates. It is likely, that at a
transition to neutral and weak-alkaline media (pH < 8) chitosane
amino groups also take part in the process of interaction
with metal ions. It is necessary a more detailed
investigation of this phenomenal. Some experiments on
chitosane carboxymethylation by monochloracetic acid
were carried out. The conditions of chitosane-metalcomplex
synthesis which allow to get a homogeneous

Abstracts 649
complexes with metal ions content from 0.8 to 8.4 were
selected. It was shown, that in the samples synthesized,
metal ions are distributed rather uniformly along a polymeric
chains. The composition, structure of the complexes
formed and the conditions of incliding in a coordination
process of polymer different functional groups, as well as
a dynamic of macromolecular tangle behaviour in the
process of metal ions binding are determined. It eventually
allows to predict the system chitosan metal-ion system
behavior in a real conditions when use. Laboratory and
field testing of regulating and fungicidal properties of chitosan
and its metal-complexes on rice and cotton seeds was
carried out. The data obtained show a high activity of
preparations tested. The conditions for rice and cotton
seeds treatment as a growth stimulator on the base of chitin,
chitosane and their derivatives are selected.
FP-T-039
Daytime sleepiness in children and adolescents as
assessed by the Epworth sleepiness scale
C.V. Harden, G.A. Fenton, C. Vemulapalli, J. Puetz, P.
Codden, T.J. Geller
St Louis University Medical Center, USA
Objective: To evaluate the incidence of sleep abnormalities,
including excessive daytime sleepiness and restless leg
symptoms in post chemo/radiation therapy survivors of
leukemia, comparing them to healthy sibling controls.
Method: Patients with a previous diagnosis of Leukemia,
who are disease-free following therapy, and non-affected
siblings were consented, and questioned using the Epworth
Sleepiness Scale (ESS). This questionnaire provides a
measurement of the subject’s general daytime drowsiness.
A target population of 80 subjects has not yet been reached.
Nonetheless, a high incidence of ESS scores .10 has been
noted, (a level which in adult studies has been associated
with moderate daytime sleepiness). Results: Mean age of
evaluated leukemia survivors is 13.7 years, SD 4.5: mean
age of sibling controls is 12.9 years, SD of 5.5. Total group
mean age is 13.4 years; SD of 4.75. Total group mean ESS
score (42 subjects) is 7.42, SD of 3.66, with a 30.9% incidence
of ESS scores .10. Conclusions: Our research
suggests that ESS scores are higher in pediatric and adolescent
populations compared to adult studies. In reviewing the
literature regarding measures of chronic sleepiness, a single
reliable tool has not been established for this age group.
Among adolescent drivers in New Zealand, the distribution
of ESS scores was lower than in older drivers, but our data
may suggest a higher incidence of moderate daytime sleepiness
in an US pediatric and adolescent population. Our
enrollment does not yet permit a comparison between leukemic
survivors and control siblings, regarding the incidence
of excessive daytime sleepiness.