Abstracts

Rationale: The young age of onset and frequently intractable nature of epilepsy associated with Sturge-Weber syndrome (SWS) has been well-described. However, many clinicians familiar with this condition also anecdotally report prolonged seizure clusters separated by often months of seizure freedom. Methods: Over a 5-year period, 78 children and adults with SWS and at least one reported seizure were referred to and evaluated at the Hunter Nelson Sturge-Weber Center. Information regarding seizure type and frequency, EEG, and patient demographics were obtained at the initial clinic visit and analyzed. Results: 45 (58%) patients were male; the median age of seizure onset was 6 months (range: birth-26 years). Patients presented to our clinic for initial consultation at a median age of 5 years (range: 6 months-43 years). The leptomeningeal angioma involved the left hemisphere in 37 (48%), right in 29 (37%), and was bilateral in 12 (15%). Patients had tried a median of 2 anticonvulsants at presentation (range: 0-10). Eleven (14%) described a temporary period of atonic or absence seizures in the setting of a generalized EEG pattern. Clusters of seizures over at least 6 hours occurred in 35 (45%); they occurred infrequently (seizure-free periods of at least 6 months) in 55 (69%). A pattern of both clustering and infrequent seizures was noted in 30 (38%) and was slightly more common in females (53% vs. 37%, p=0.13) and less common in those with bilateral facial port-wine stains (17% vs. 31%, p=0.08) and focal EEG spikes (36% vs. 55%, p=0.08). Conclusions: Seizures in those with SWS occur at a young age, with more than one third having a clustering and sporadic seizure pattern. This seizure frequency was slightly more likely to occur in females and in those without focal EEG spikes or bilateral facial port-wine stains. This history of periodic, but severe seizures appears to be common with SWS and leads to difficult treatment decisions, especially in regards to the timing of potential surgical resection.