Abstracts

Rationale: Sturge-Weber Syndrome (SWS) is rare and is commonly associated with epilepsy. The purpose of this survey was to further define the incidence of specific neurologic complications and evolving trends in therapy in the community. Methods: An on-line survey was sent through the World Wide Web to members of the Sturge-Weber Foundation. The survey gathered demographic and medical information regarding individuals with SWS. Results: 101 patient responses were received from the following countries: USA, UK, South Africa, Italy, Turkey, Australia, Ireland, Israel, and India. There were 43 males and 58 females. Port-wine birthmarks were present on the right face in 51, left face in 55 and involved the body in 16. Forty-eight patients have glaucoma, 30 have migraine headaches and 70 have epilepsy. Seventy-seven patients have taken or are currently taking antiepileptic drugs (AEDs). The three most common AEDs taken in the past are Phenobarbital in 30 (39%), Phenytoin in 27 (35%) and Carbamazepine in 23 (30%). The three most common AEDs that are currently being taken are Levetiracetam in 22 (29%), Carbamazepine in 21 (27%) and Oxcarbazepine in 17 (22%). Five patients have received vagus nerve stimulation and 12 have had resective epilepsy surgery. Conclusions: Epilepsy is common in SWS occurring in 70% of patients. There appears to be a trend in AED therapy toward using the newer medications with Levetiracetam, Carbamazepine and Oxcarbazepine being the most commonly used. AEDs selected for use in SWS are most commonly sodium-channel blocking agents, likely reflecting the localization-related nature of their epilepsy. Resective epilepsy surgery was performed in 12% of this patient population.