PDF File - Mahidol University

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