Abstracts

Rationale: To review surgical management of epilepsy in children with Rasmussen Syndrome at a tertiary referral centre. Methods: Data was collected prospectively and retrospectively on 21 children (13 female and 8 male) who came to surgery between 1987 -2010.Results: Clinical progress: Median age at first symptom was 6.5 years (range was 3.0 12.6). 17/21 presented with seizure, 1/21 with progressive weakness and 3/21 with behavioural and/or cognitive decline. Median age at first surgery was 10.4 years (range 4.2-16.1), median time from diagnosis to surgery 3.7 years (range 0.3-7.7) and median duration of follow-up 7.8 years (range 0.1-18.0). 11 of 21 had right hemisphere disease, being dominant in 2 /11 ( in one of these two, handedness had changed prior to surgery). The left hemisphere was affected in 10 out of 21 and was dominant in 7/10 (with change in handedness in 3/7 prior to surgery). Condition at time of surgery: All children had drug resistant epilepsy, 13/21 had epilepsia partialis continua and 5/21 had convulsive status epilepticus needing PICU admission. 20/21 had mild to moderate hemiparesis, and 18/21 behavioural and/or cognitive difficulties. 14/21 children had undergone neuropsychological evaluation and all had evident cognitive deterioration from onset of illness. 10/14 children had significant cognitive impairment (FSIQ<69), 3/14 had mild cognitive impairment (FSIQ>