Abstracts

Rationale: Anecdotal reports have described cortical malformations in epileptic patients with Sturge-Weber syndrome (SWS). No data are available regarding the prevalence or significance of this association.Methods: We reviewed retrospectively the clinical profile, pre-operative MRI studies and pathology reports of all patients with SWS and medically intractable epilepsy evaluated in our epilepsy surgery program between 1979 and 2006.Results: 12 patients (M/F=7/5) were identified. A port-wine stain was noted in 10 cases; none had glaucoma. Mean age at seizure onset was 11.1±16.7 months. Seizures occurred daily in 7 and weekly in 5 cases. Seven patients were mentally retarded and 8 patients were hemiparetic. Eight patients underwent excisional surgery (mean age 10.3±6.5y) including hemispherectomy (n=4) and focal cortical resection (n=4). Pre-operative MRI studies available for review (n=9) revealed evidence of cortical malformation, leptomeningeal angiomatosis and gyriform calcifications in all cases. Tissue was available in 6 of 8 operated cases and revealed polymicrogyria (n=3) and cortical dysplasia (n=3).Conclusions: Cortical malformations are consistently identified in patients with medically intractable epilepsy and Sturge-Weber-Syndrome and likely represents the underlying cause of epilepsy. Implications for surgical therapy will be discussed.