4.4 Article

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

期刊

PEDIATRIC NEUROLOGY
卷 119, 期 -, 页码 3-10

出版社

ELSEVIER SCIENCE INC
DOI: 10.1016/j.pediatrneurol.2021.02.006

关键词

Sturge-Weber syndome; Seizures; Epilepsy; Antiseizure medication; Brain involvement; Neurosurgery; Neurocutaneous syndromes; Outcome

资金

  1. National Institutes of Health (NIH) [U54NS065705, R01NS041922]
  2. NIH Office of Rare Diseases Research at the National Center for Advancing Translational Science (NCATS) [U54NS065705]
  3. NIH Office of Rare Diseases Research at the National Institute of Neurological Disorders and Stroke (NINDS) [U54NS065705]
  4. Sturge-Weber Foundation
  5. Celebrate Hope Foundation (Comi)
  6. Foerderer Fund
  7. Robison D. Harley, MD Endowed Chair in Pediatric Ophthalmology and Ocular Genetics
  8. Adeline Lutz -Steven S.T. Ching, M.D. Distinguished Professorship in Ophthalmology
  9. Research to Prevent Blindness

向作者/读者索取更多资源

The study analyzed the use of neurological drug treatment in SWS patients across different demographic and SWS characteristic groups. Results showed that variables such as sex, ethnicity, and brain, skin, and eye involvement can impact medication use. The study also found that a history of neurosurgery, bilateral brain involvement, and family history may affect the number of antiseizure medications used by patients.
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 (4 4.8%), oxcarbazepine (39.9%), and phenobarbital (14.9%). Lamotrigine was more frequently used in adults than in children (P 1/4 0.001). History of neurosurgery was associated with no current antiseizure medication use (P 1/4 0.001), whereas bilateral brain involvement and family history of seizures were associated with using a higher number of antiseizure medications (P 1/4 0.002, P 1/4 0.027, respectively). Subjects with bilateral brain involvement and early seizure onset were associated with using a higher number of antiseizure medications (P 1/4 0.002) and phenobarbital use (0.003).

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