AOCCN2017

講演情報

Poster Presentation

[P1-1~141] Poster Presentation 1

2017年5月11日(木) 09:30 〜 16:00 Poster Room A (1F Navis A・B・C)

[P1-46] Long term epilepsy outcome in West syndrome

Jithangi Wanigasinghe (University of Colombo, Sri Lanka)

West syndrome is a devastating epilepsy syndrome that typically begins in infancy. It catastrophic nature is often described based on the refractoriness to treatment, associated developmental regression and other neurological co-morbidities. The literature on long term in these children is limited.
Children with West syndrome who were initially treated with hormonal therapies (oral prednisolone or intramuscular ACTH) were prospectively followed up at a tertiary care neurology clinic in Sri Lanka. They were followed up by a paediatric neurologist. Their epilepsy outcome at age of 4 years is described in this paper.
Out of the initial 82 children with West syndrome, only 51 were available for review at 4 years. There were 11 deaths and 20 were lost for follow up. Out of the balance 51 the male female ratio was 1.45: 1. Total number of children completely free of any type of epilepsy (epileptic spasms or any other type of epilepsy) for more than one year period was 22 (43%). There were four who were continuing to experienced spasms while five were experiencing epileptic spasms with other types of seizures concurrently. These five fulfilled criteria for Lennox Gastaut syndrome. There were another 20 who experienced a focal epilepsy only.
These findings outline the long term outcome of children with West syndrome with 43% being free of epilepsy. Spasms continued only in less than 20%.