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Multicenter Study
. 2021 Jun:119:3-10.
doi: 10.1016/j.pediatrneurol.2021.02.006. Epub 2021 Mar 5.

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

Lindsay F Smegal  1 , Alison J Sebold  1 , Adrienne M Hammill  2 , Csaba Juhász  3 , Warren D Lo  4 , Daniel K Miles  5 , Angus A Wilfong  6 , Alex V Levin  7 , Brian Fisher  8 , Karen L Ball  8 , Anna L Pinto  9 , Anne M Comi  10 ; National Institutes of Health Sponsor: Rare Disease Clinical Research Consortium (RDCRN) Brain Vascular Malformation Consortium (BVMC) SWS Investigator Group
Collaborators, Affiliations
Multicenter Study

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

Lindsay F Smegal et al. Pediatr Neurol. 2021 Jun.

Abstract

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.

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

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