Multi-Center Research Data of Epilepsy Management in Patients with Sturge-Weber Syndrome

Abstract Objective Epilepsy in the classic form of Sturge-Weber syndrome (SWS) is common and many important questions remain regarding treatment outcomes. We aimed to analyze a large multi-center database with focus on neurological drug treatment in different demographic and SWS characteristic groups. Study Design 268 patients with confirmed brain involvement and history of seizures were selected from a research data registry generated from a multi-center cross-sectional questionnaire. We examined associations between medication use and binary variables such as sex, ethnicity, and brain, skin, and eye involvement laterality. We analyzed group differences in mean number of anti-seizure medications (ASMs), age at diagnosis, enrollment, and seizure onset, and examined differences in median SWS neurological score subscales in groups of interest. Results The most frequently used medications were levetiracetam (48.1%), low–dose aspirin (44.8%), oxcarbazepine (39.9%), and phenobarbital (14.9%). Lamotrigine was more frequently used in adults compared to children (p=0.001). History of neurosurgery was associated with no current ASM use (p=0.001), while bilateral brain involvement and family history of seizures were associated with using a higher number of ASMs (p=0.002, p=0.027, respectively). Subjects with bilateral brain involvement and early seizure onset were associated with using a higher number of ASMs (p=0.003) and phenobarbital use (0.001). Conclusions Levetiracetam, low-dose aspirin, and oxcarbazepine were the most frequently used medications. More severely affected patients were frequently on a greater number of ASMs. Surgery for epilepsy was associated with ability to discontinue ASM. Longitudinal studies are needed to further investigate medication use in patients with SWS.