Multicenter Research Data of Epilepsy Management in Patients With Sturge-Weber Syndrome.

Journal Article (Journal Article;Multicenter Study)

BACKGROUND: Epilepsy in typical Sturge-Weber syndrome (SWS) is common, and many questions remain regarding the treatment outcomes. We analyzed a large multicenter database with focus on neurological drug treatment in different demographic and SWS characteristic groups. METHODS: A total of 268 patients with brain involvement and a history of seizures were selected from a research data registry generated from a multicenter 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 antiseizure medications and age at diagnosis, enrollment, and seizure onset and examined differences in median SWS neurological scores 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 than in children (P = 0.001). History of neurosurgery was associated with no current antiseizure medication use (P = 0.001), whereas bilateral brain involvement and family history of seizures were associated with using a higher number of antiseizure medications (P = 0.002, P = 0.027, respectively). Subjects with bilateral brain involvement and early seizure onset were associated with using a higher number of antiseizure medications (P = 0.002) and phenobarbital use (0.003). CONCLUSIONS: Levetiracetam, low-dose aspirin, and oxcarbazepine were the most frequently used medications. More severely affected patients were frequently on a greater number of antiseizure medications. Surgery for epilepsy was associated with the ability to discontinue antiseizure medication. Longitudinal studies are needed to further investigate medication use in patients with SWS.

Full Text

Duke Authors

Cited Authors

  • Smegal, LF; Sebold, AJ; Hammill, AM; Juhász, C; Lo, WD; Miles, DK; Wilfong, AA; Levin, AV; Fisher, B; Ball, KL; Pinto, AL; Comi, AM; National Institutes of Health Sponsor: Rare Disease Clinical Research Consortium (RDCRN) Brain Vascular Malformation Consortium (BVMC) SWS Investigator Group,

Published Date

  • June 2021

Published In

Volume / Issue

  • 119 /

Start / End Page

  • 3 - 10

PubMed ID

  • 33813331

Pubmed Central ID

  • PMC8162684

Electronic International Standard Serial Number (EISSN)

  • 1873-5150

Digital Object Identifier (DOI)

  • 10.1016/j.pediatrneurol.2021.02.006


  • eng

Conference Location

  • United States