Lindsay F Smegal1, Alison J Sebold1, Adrienne M Hammill2, Csaba Juhász3, Warren D Lo4, Daniel K Miles5, Angus A Wilfong6, Alex V Levin7, Brian Fisher8, Karen L Ball8, Anna L Pinto9, Anne M Comi10. 1. Department of Neurology, Hugo Moser Kennedy Krieger Research Institute, Baltimore, Maryland. 2. Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio. 3. Departments of Pediatrics, Neurology, and Neurosurgery, Wayne State University School of Medicine, Children's Hospital of Michigan, Detroit, Michigan. 4. Neurology, Nationwide Children's Hospital, Columbus, Ohio. 5. Department of Neurology, Pediatric Epilepsy, New York University Langone Health, New York, New York. 6. Neurology, Barrow Neurological Institute, Phoenix Children's Hospital, Phoenix, Arizona. 7. Pediatric Ophthalmology and Ocular Genetics, Flaum Eye Institute, Golisano Children's Hospital, University of Rochester, Rochester, New York. 8. The Sturge-Weber Foundation, Houston, Texas. 9. Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts. 10. Department of Neurology, Hugo Moser Kennedy Krieger Research Institute, Baltimore, Maryland; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland; Department of Pediatrics, Johns Hopkins Hospital, Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address: comi@kennedykrieger.org.
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.
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.
Authors: Samuel Gittins; Dora Steel; Andreas Brunklaus; Imogen Newsom-Davis; Christina Hawkins; Sarah E Aylett Journal: Epilepsy Behav Date: 2018-09-06 Impact factor: 2.937
Authors: Laura A Hatfield; Nathan E Crone; Eric H Kossoff; Joshua B Ewen; Paula L Pyzik; Doris D M Lin; Thomas M Kelley; Anne M Comi Journal: Epilepsia Date: 2007-01 Impact factor: 5.864
Authors: Evan J Hess; Kirsten A Moody; Alexandra L Geffrey; Sarah F Pollack; Lauren A Skirvin; Patricia L Bruno; Jan L Paolini; Elizabeth A Thiele Journal: Epilepsia Date: 2016-10-03 Impact factor: 5.864
Authors: Alyssa M Day; Adrienne M Hammill; Csaba Juhász; Anna L Pinto; E Steve Roach; Charles E McCulloch; Anne M Comi Journal: Pediatr Neurol Date: 2018-11-24 Impact factor: 3.372
Authors: Andres M Kanner; Eric Ashman; David Gloss; Cynthia Harden; Blaise Bourgeois; Jocelyn F Bautista; Bassel Abou-Khalil; Evren Burakgazi-Dalkilic; Esmeralda Llanas Park; John Stern; Deborah Hirtz; Mark Nespeca; Barry Gidal; Edward Faught; Jacqueline French Journal: Neurology Date: 2018-06-13 Impact factor: 9.910
Authors: Alison J Sebold; Alyssa M Day; Joshua Ewen; Jack Adamek; Anna Byars; Bernard Cohen; Eric H Kossoff; Tomoyuki Mizuno; Matthew Ryan; Jacqueline Sievers; Lindsay Smegal; Stacy J Suskauer; Cameron Thomas; Alexander Vinks; T Andrew Zabel; Adrienne M Hammill; Anne M Comi Journal: Pediatr Neurol Date: 2020-11-02 Impact factor: 3.372
Authors: Joyce Y Wu; Monisha Goyal; Jurriaan M Peters; Darcy Krueger; Mustafa Sahin; Hope Northrup; Kit S Au; Sarah O'Kelley; Marian Williams; Deborah A Pearson; Ellen Hanson; Anna W Byars; Jessica Krefting; Mark Beasley; Gary Cutter; Nita Limdi; E Martina Bebin Journal: Epilepsia Date: 2019-11-05 Impact factor: 5.864