Abstracts

To review seizure control and developmental outcome in children with SWS undergoing hemispherectomy or resective surgery for epilepsy. Case note review of all cases with SWS undergoing hemispherectomy or resective surgery at a centre for paediatric epilepsy surgery in the UK. 10 children age 10m-17 years (median 24 months) underwent surgical treatment for epilepsy refractory to medical management. Pre-surgical evaluation was carried out according to protocols, including neurodevelopmental, neuropsychological and neuropsychiatric assessment, MRI, videotelemtry and in addition SPECT studies in those evaluated for resective surgery. Four infants had experienced an autistic pattern of regression in the context of severe seizure episodes prior to surgery. All children undergoing hemispherectomy had a marked hemiplegia.
Surgical treatment was functional hemispherectomy (6), lobar resection (2) and multilobar resection(2).
Median follow up was three years (7 months-10 years). Seizure frequency per month following surgery: 0 (5), [lt]1/month (3) and [gt]1/month (2). Number of anti-epileptic drugs (AEDs) at follow up 0 (2), 1(4), 2(4). AEDs were reducing in three cases.
Following hemispherectomy there was an increase in motor deficit (6). For the whole group, cognitive ability is within the normal range (2); Eight have developmental delay- moderate (3) severe (5). Seven have autistic features or autistic spectrum disorder. At follow up, sociability was reported as improved (5) and less good (2). Surgical treatment of epilepsy in SWS can result in a marked improvement in seizure control. Early hemispherectomy may be important in reducing the likelihood of autistic regression in children. The timing of hemispherectomy may also influence the possibility of a neurological deficit emerging, especially in those without an established hemiplegia. (Supported by The Sturge-Weber Foundation (UK))