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

Publication ,  Journal Article
Smegal, LF; Sebold, AJ; Hammill, AM; Juhász, C; Lo, WD; Miles, DK; Wilfong, AA; Levin, AV; Fisher, B; Ball, KL; Pinto, AL; Comi, AM ...
Published in: Pediatr Neurol
June 2021

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.

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Published In

Pediatr Neurol

DOI

EISSN

1873-5150

Publication Date

June 2021

Volume

119

Start / End Page

3 / 10

Location

United States

Related Subject Headings

  • Young Adult
  • Sturge-Weber Syndrome
  • Outcome Assessment, Health Care
  • Neurosurgical Procedures
  • Neurology & Neurosurgery
  • Male
  • Humans
  • Female
  • Epilepsy
  • Cross-Sectional Studies
 

Citation

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Smegal, L. F., Sebold, A. J., Hammill, A. M., Juhász, C., Lo, W. D., Miles, D. K., … National Institutes of Health Sponsor: Rare Disease Clinical Research Consortium (RDCRN) Brain Vascular Malformation Consortium (BVMC) SWS Investigator Group, . (2021). Multicenter Research Data of Epilepsy Management in Patients With Sturge-Weber Syndrome. Pediatr Neurol, 119, 3–10. https://doi.org/10.1016/j.pediatrneurol.2021.02.006
Smegal, Lindsay F., Alison J. Sebold, Adrienne M. Hammill, Csaba Juhász, Warren D. Lo, Daniel K. Miles, Angus A. Wilfong, et al. “Multicenter Research Data of Epilepsy Management in Patients With Sturge-Weber Syndrome.Pediatr Neurol 119 (June 2021): 3–10. https://doi.org/10.1016/j.pediatrneurol.2021.02.006.
Smegal LF, Sebold AJ, Hammill AM, Juhász C, Lo WD, Miles DK, et al. Multicenter Research Data of Epilepsy Management in Patients With Sturge-Weber Syndrome. Pediatr Neurol. 2021 Jun;119:3–10.
Smegal, Lindsay F., et al. “Multicenter Research Data of Epilepsy Management in Patients With Sturge-Weber Syndrome.Pediatr Neurol, vol. 119, June 2021, pp. 3–10. Pubmed, doi:10.1016/j.pediatrneurol.2021.02.006.
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. Multicenter Research Data of Epilepsy Management in Patients With Sturge-Weber Syndrome. Pediatr Neurol. 2021 Jun;119:3–10.
Journal cover image

Published In

Pediatr Neurol

DOI

EISSN

1873-5150

Publication Date

June 2021

Volume

119

Start / End Page

3 / 10

Location

United States

Related Subject Headings

  • Young Adult
  • Sturge-Weber Syndrome
  • Outcome Assessment, Health Care
  • Neurosurgical Procedures
  • Neurology & Neurosurgery
  • Male
  • Humans
  • Female
  • Epilepsy
  • Cross-Sectional Studies