title: [Efficacy, safety and tolerability of high-dose topiramate with rapid dose titration in symptomatic West syndrome].
abstract: OBJECTIVE
  We investigated the efficacy, safety, and tolerability of high-dose topiramate with rapid dose titration in 12 children with symptomatic West syndrome who suffered from severe motor and intellectual disabilities.
  
  
  METHODS
  Topiramate was introduced as add-on therapy at the daily dose of 1 mg/kg/day, followed by increments of 2 mg/kg at 3- or 4-day intervals, up to a maximum of 19 or 20 mg/kg/day. The ages at the start of topiramate therapy ranged from 5 to 22 months. Prior to the topiramate therapy, the patients had received 2 to 6 antiepileptic agents with (8 patients) or without ACTH (4 patients).
  
  
  RESULTS
  Topiramate appeared to be effective in 8 of the 12 patients (67%); four became seizure-free;three showed greater than 90% seizure reduction; one showed greater than 50% seizure reduction. The maintenance dose was 7 to 20 mg/kg/day (mean:17.9 +/- 3.9 mg/kg/day). In 4 of these 8 patients (50%), the spasms relapsed several months after complete cessation or diminution in the frequency of the spasms following treatment with topiramate. All of the 8 topiramate-responsive patients could continue the topiramate therapy throughout this study. The duration of topiramate therapy was 7 to 42 months (median: 12.5 months). There were no severe side effects that necessitated discontinuation of topiramate, including kidney stones.
  
  
  CONCLUSIONS
  High-dose topiramate with rapid dose titration was revealed to be effective, safe, and well-tolerated in children with symptomatic West syndrome.
authors.affiliation: Department of Pediatrics, Shiga Medical Center for Children, Moriyama, Shiga. tkumada@mccs.med.shiga-pref.jp
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Kumada
authors.forename: Tomoko
authors.initials: T
authors.lastname: Miyajima
authors.forename: Nozomi
authors.initials: N
authors.lastname: Oda
authors.forename: Hideki
authors.initials: H
authors.lastname: Shimomura
authors.forename: Keiko
authors.initials: K
authors.lastname: Saito
authors.forename: Ikuko
authors.initials: I
authors.lastname: Hiejima
authors.forename: Fumihito
authors.initials: F
authors.lastname: Nozaki
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Fujii
mesh_terms: D000927:Anticonvulsants / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D004305:Dose-Response Relationship, Drug; D005260:Female; D005632:Fructose / Q000008:administration & dosage / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008297:Male; D013036:Spasms, Infantile / Q000188:drug therapy*; D000077236:Topiramate; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants; D000077236:Topiramate; D005632:Fructose