Effectiveness of vigabatrin in West syndrome associated with tuberous sclerosis

Naoki Ando; Shinji Fujimoto; Tatsuya Ishikawa; Satoru Kobayashi; Ayako Hattori; Tetsuya Ito; Hajime Togari

Effectiveness of vigabatrin in West syndrome associated with tuberous sclerosis

Naoki Ando, Shinji Fujimoto, Tatsuya Ishikawa, Satoru Kobayashi, Ayako Hattori, Tetsuya Ito, Hajime Togari

Research output: Contribution to journal › Article › peer-review

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·kg^-1·day^-1, and the mean maintenance dosage was 38.4 mg·kg^-1·day ^-1. At the beginning of VGB treatment, 3 patients had hypsarrhythmia, 2 had focal discharge with generahzation, 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.

Original language	English
Pages (from-to)	444-448
Number of pages	5
Journal	NO TO HATTATSU
Volume	42
Issue number	6
Publication status	Published - 2010
Externally published	Yes

All Science Journal Classification (ASJC) codes

Pediatrics, Perinatology, and Child Health
Clinical Neurology

Cite this

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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·kg-1·day-1, and the mean maintenance dosage was 38.4 mg·kg-1·day -1. At the beginning of VGB treatment, 3 patients had hypsarrhythmia, 2 had focal discharge with generahzation, 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.",

author = "Naoki Ando and Shinji Fujimoto and Tatsuya Ishikawa and Satoru Kobayashi and Ayako Hattori and Tetsuya Ito and Hajime Togari",

year = "2010",

language = "English",

volume = "42",

pages = "444--448",

journal = "NO TO HATTATSU",

issn = "0029-0831",

publisher = "Japanese Society of Child Neurology",

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TY - JOUR

T1 - Effectiveness of vigabatrin in West syndrome associated with tuberous sclerosis

AU - Ando, Naoki

AU - Fujimoto, Shinji

AU - Ishikawa, Tatsuya

AU - Kobayashi, Satoru

AU - Hattori, Ayako

AU - Ito, Tetsuya

AU - Togari, Hajime

PY - 2010

Y1 - 2010

N2 - 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·kg-1·day-1, and the mean maintenance dosage was 38.4 mg·kg-1·day -1. At the beginning of VGB treatment, 3 patients had hypsarrhythmia, 2 had focal discharge with generahzation, 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.

AB - 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·kg-1·day-1, and the mean maintenance dosage was 38.4 mg·kg-1·day -1. At the beginning of VGB treatment, 3 patients had hypsarrhythmia, 2 had focal discharge with generahzation, 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.

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AN - SCOPUS:78649430308

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JO - NO TO HATTATSU

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Effectiveness of vigabatrin in West syndrome associated with tuberous sclerosis

Abstract

All Science Journal Classification (ASJC) codes

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