Epilepsy surgery in patients with Sturge-Weber Syndrome

Abstract

Sturge-Weber Syndrome (SWS) is a neurovascular condition caused by a mutation in the GNAQ gene. The most common neurological manifestations of SWS are epilepsy, developmental delay, and stroke-like episodes. Seizures are often the first neurological symptom, and most patients have drug-resistant epilepsy. Predictors for unfavorable epilepsy outcomes and the need for ASM polytherapy included age of onset, bilateral brain involvement, extensive unilateral hemispheric disease, and a positive family history of epilepsy. The surgical approach to SWS is challenging due to the associated abnormal vasculature and potential complications. Hemispherotomy is associated with high seizure freedom rates and ASM discontinuation, but resective surgery may be an efficient alternative in well-selected patients. Complete seizure control is the ultimate goal of epilepsy surgery; however, reducing seizure severity and frequency may help improve cognitive outcome and quality of life.