Patients with Sturge-Weber disease with
epilepsy refractory to medical
therapy have been reported to develop slowly progressive neurological deficits and ultimately become moderately or severely disabled. We studied six patients with
Sturge-Weber syndrome including its incomplete form. Three out of six patients with
Sturge-Weber syndrome revealed evolution of calcified
angioma on computed tomography. All of the three cases developed medically intractable
seizures. Total and/or subtotal
hemispherectomy was performed for these three cases. The surgery was effective for controlling
seizures in all three cases except one with
infantile spasm with hypsarrythmia on electroencephalogram who is still on
anticonvulsant. Although the unremitting deterioration in
mental retardation and
hemiparesis was not effectively prevented by the surgery possibly because the timing of surgery was delayed in one case, the surgery not only stopped the frequent medically-intractable
seizures, but also dramatically prevented the psychomotor deterioration in the other case. Although the role of surgical treatment for the patients with
Sturge-Weber syndrome remains poorly defined, one can expect excellent results if the indications for surgery are carefully analyzed and
hemispherectomy is performed on an individual basis.