Emma H Kaplan1, Eric H Kossoff2, Catherine D Bachur1, Milton Gholston1, Jihoon Hahn1, Matthew Widlus1, Anne M Comi3. 1. Department of Neurology, Kennedy Krieger Institute, Baltimore, Maryland. 2. Department of Pediatrics, Johns Hopkins Hospital, Baltimore, Maryland; Department of Neurology, Johns Hopkins Hospital, Baltimore, Maryland. 3. Department of Neurology, Kennedy Krieger Institute, Baltimore, Maryland; Department of Pediatrics, Johns Hopkins Hospital, Baltimore, Maryland; Department of Neurology, Johns Hopkins Hospital, Baltimore, Maryland. Electronic address: comi@kennedykrieger.org.
Abstract
OBJECTIVE: We analyzed individuals with epilepsy due to Sturge-Weber syndrome to determine which anticonvulsants provided optimal seizure control and which resulted in the fewest side effects. METHODS: One-hundred-eight records from a single center were retrospectively analyzed for Sturge-Weber syndrome brain involvement, epilepsy, Sturge-Weber syndrome neuroscores, and currently used anticonvulsants. RESULTS: Of the fourteen anticonvulsants that had been employed, the most often used agents were oxcarbazepine or carbamazepine, and levetiracetam. Individuals whose seizures at the most recent visit were fully controlled (seizure-free) for 6 months or longer were more likely to have ever tried, or currently used, oxcarbazepine or carbamazepine than those with uncontrolled seizures. Thirty-nine of 69 individuals (56.5%) were seizure-free with oxcarbazepine or carbamazepine history versus 11 of 35 individuals (31.4%) who had not taken these agents (P < 0.05); 38 of 62 patients (61.3%) were seizure-free while currently taking these anticonvulsants versus 12 of 42 (28.6%) not taking them (P < 0.01). Patients with seizure control for 6 months or longer were less likely to have ever tried, or to currently be taking, levetiracetam than those without control. Sixteen of 56 individuals (28.6%) were seizure-free with levetiracetam history versus 34 of 48 (70.8%) without it (P < 0.001); 14 of 43 individuals (32.6%) were seizure-free and currently taking levetiracetam versus 36 of 61 (59.0%) not taking it (P < 0.01). When topiramate was added as second-line medication, five of nine patients (55.6%) experienced decreased seizure severity, and worsening of glaucoma was not reported. CONCLUSIONS: Carbamazepine and oxcarbazepine were associated with better seizure control than levetiracetam in this Sturge-Weber syndrome cohort and so may be preferred as the initial therapy. When used as adjunctive therapy, topiramate was effective in this limited analysis without a clear increased incidence of glaucoma.
OBJECTIVE: We analyzed individuals with epilepsy due to Sturge-Weber syndrome to determine which anticonvulsants provided optimal seizure control and which resulted in the fewest side effects. METHODS: One-hundred-eight records from a single center were retrospectively analyzed for Sturge-Weber syndrome brain involvement, epilepsy, Sturge-Weber syndrome neuroscores, and currently used anticonvulsants. RESULTS: Of the fourteen anticonvulsants that had been employed, the most often used agents were oxcarbazepine or carbamazepine, and levetiracetam. Individuals whose seizures at the most recent visit were fully controlled (seizure-free) for 6 months or longer were more likely to have ever tried, or currently used, oxcarbazepine or carbamazepine than those with uncontrolled seizures. Thirty-nine of 69 individuals (56.5%) were seizure-free with oxcarbazepine or carbamazepine history versus 11 of 35 individuals (31.4%) who had not taken these agents (P < 0.05); 38 of 62 patients (61.3%) were seizure-free while currently taking these anticonvulsants versus 12 of 42 (28.6%) not taking them (P < 0.01). Patients with seizure control for 6 months or longer were less likely to have ever tried, or to currently be taking, levetiracetam than those without control. Sixteen of 56 individuals (28.6%) were seizure-free with levetiracetam history versus 34 of 48 (70.8%) without it (P < 0.001); 14 of 43 individuals (32.6%) were seizure-free and currently taking levetiracetam versus 36 of 61 (59.0%) not taking it (P < 0.01). When topiramate was added as second-line medication, five of nine patients (55.6%) experienced decreased seizure severity, and worsening of glaucoma was not reported. CONCLUSIONS:Carbamazepine and oxcarbazepine were associated with better seizure control than levetiracetam in this Sturge-Weber syndrome cohort and so may be preferred as the initial therapy. When used as adjunctive therapy, topiramate was effective in this limited analysis without a clear increased incidence of glaucoma.
Authors: Matthew D Shirley; Hao Tang; Carol J Gallione; Joseph D Baugher; Laurence P Frelin; Bernard Cohen; Paula E North; Douglas A Marchuk; Anne M Comi; Jonathan Pevsner Journal: N Engl J Med Date: 2013-05-08 Impact factor: 91.245
Authors: Alejandro J De la Torre; Aimee F Luat; Csaba Juhász; Mai Lan Ho; Davis P Argersinger; Kara M Cavuoto; Mabel Enriquez-Algeciras; Stephanie Tikkanen; Paula North; Craig N Burkhart; Harry T Chugani; Karen L Ball; Anna Lecticia Pinto; Jeffrey A Loeb Journal: Pediatr Neurol Date: 2018-04-18 Impact factor: 3.372
Authors: Negar Golchin; Hannah Johnson; Paul M Bakaki; Neal Dawson; Elia M Pestana Knight; Sharon B Meropol; Rujia Liu; James A Feinstein; Shari D Bolen; Lawrence C Kleinman; Alexis Horace Journal: Drugs Ther Perspect Date: 2019-07-12
Authors: Lindsay F Smegal; Alison J Sebold; Adrienne M Hammill; Csaba Juhász; Warren D Lo; Daniel K Miles; Angus A Wilfong; Alex V Levin; Brian Fisher; Karen L Ball; Anna L Pinto; Anne M Comi Journal: Pediatr Neurol Date: 2021-03-05 Impact factor: 4.210