Abstracts

Rationale: Sturge-Weber syndrome (SWS) is a sporadic, neurocutaneous disorder associated with facial port-wine nevus, leptomeningeal angioma, glaucoma and cognitive impairment. Seizures are a common manifestation, often resistant to multiple anticonvulsants. We studied the characteristics and clinical course of seizures in SWS which may be helpful in making treatment decisions in poorly controlled seizures. Methods: We retrospectively reviewed the record of 19 SWS patients (M:F=5:14) with at least one reported seizure, treated at Asan Medical Center between June 1993 and March 2010. Information included age at seizure onset, pattern of clustering seizures (defined as multiple repetitive seizures over a 24 hour period) or status epilepticus, and clinical outcome after surgical resection in intractable epilepsy (defined as uncontrolled seizures despite use of more than two anticonvulsants). Surgical resection was done in patients with non-clustering, intractable epilepsy. Results: The age at seizure onset was 17 months (1-9 months) and the mean duration of follow-up was 7.2 years (0.2-17.5 years). Many showed simple partial seizures (8/19, 40%) followed by complex partial seizures (7/19, 35%) and generalized seizures (4/19, 21%). Four patients (20%) showed sporadic, clustering seizures. The mean number of seizures per cluster was 10 (1-24) over a mean duration of 3.8 days (1-7 days). The mean duration of seizure-free periods lasted 13.5 months (1-21 months) and the mean age of last cluster was 3.5 years (0.8-7.2 years). Two patients (2/4, 50%) manifested as status epilepticus during clusters. Three patients (M:F=1:2) received surgery, among 5 patients (26%) with intractable epilepsy. Mean age at surgery was 2.7 years (1-4 years). Type of surgery included hemispherectomy, corpus callosotomy and lesionectomy. Patients who received hemispherectomy and corpus callosotomy, each showed class 1 outcome (Engel s seizure classification) for 5 years and 2 years. The patient who received lesionectomy showed class 2 outcome. Conclusions: SWS patients may show sporadic, clustering seizures during infancy, which are difficult to control but are dramatically reduced after several years. Evaluating the efficacy of anticonvulsants and making decisions regarding the appropriate time of surgery may be difficult because of this characteristic seizure pattern. However, surgical treatment still remains effective in some patients with intractable epilepsy.