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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.