title: [Effectiveness of vigabatrin in west syndrome associated with tuberous sclerosis].
abstract: Vigabatrin (VGB) is one of the most effective anti-epileptic drugs for tonic spasms, those accompanied with tuberous sclerosis complex (TSC), but is not available in Japan. We treated 7 patients with West syndrome (WS) and TSC with VGB. In these patients, VGB treatment was started at 5-65 months of age. Six patients (86%) had complete cessation of tonic spasms. Of these, 3 patients had complete cessation within 24 hours after VGB treatment. The mean initial dosage of VGB was 36.2 mg x kg(-1) x day(-1), and the mean maintenance dosage was 38.4 mg x kg(-1) x day(-1). At the beginning of VGB treatment, 3 patients had hypsarrhythmia, 2 had focal discharge with generalization, and 2 had only focal discharge on electroencephalography. Hypsarrhythmia disappeared within 4-8 weeks after VGB treatment. Behavioral problems and sleep difficulty were observed in 6 patients. Visual field examination revealed no abnormalities in 3 patients. We hope that patients with WS and TSC can be treated with VGB as soon as possible in Japan.
authors.affiliation: Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi. nando@med.nagoya-cu.ac.jp
authors.forename: Naoki
authors.initials: N
authors.lastname: Ando
authors.forename: Shinji
authors.initials: S
authors.lastname: Fujimoto
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Ishikawa
authors.forename: Satoru
authors.initials: S
authors.lastname: Kobayashi
authors.forename: Ayako
authors.initials: A
authors.lastname: Hattori
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Ito
authors.forename: Hajime
authors.initials: H
authors.lastname: Togari
mesh_terms: D000927:Anticonvulsants / Q000008:administration & dosage / Q000627:therapeutic use*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D013036:Spasms, Infantile / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D014402:Tuberous Sclerosis / Q000150:complications*; D020888:Vigabatrin / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D000927:Anticonvulsants; D020888:Vigabatrin