Extremely low-dose vigabatrin for West syndrome with tuberous sclerosis

Naomi Hino-Fukuyo; Yuko Sato; Yosuke Kakisaka; Wakaba Endo; Yuki Kubota; Atsuo Kikuchi; Tomoko Kobayashi; Kazuhiro Haginoya; Mitsugu Uematsu; Yurika Numata; Hiroshi Doi; Masato Mori; Hitoshi Osaka; Shigeo Kure

doi:10.3233/PEP-14064

Journal of Pediatric Epilepsy, Table of Contents

Journal of Pediatric Epilepsy 2013; 02(04): 255-258
DOI: 10.3233/PEP-14064

Case Report

Georg Thieme Verlag KG Stuttgart – New York

Extremely low-dose vigabatrin for West syndrome with tuberous sclerosis

Authors

Naomi Hino-Fukuyo

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Yuko Sato

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Yosuke Kakisaka

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Wakaba Endo

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Yuki Kubota

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Atsuo Kikuchi

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Tomoko Kobayashi

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Kazuhiro Haginoya

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan

^bDepartment of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai, Japan
Mitsugu Uematsu

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Yurika Numata

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
Hiroshi Doi

^cDepartment of Ophthalmology, Tohoku University, School of Medicine, Sendai, Japan
Masato Mori

^dDepartment of Pediatrics, Jichi Medical University, School of Medicine, Tochigi, Japan
Hitoshi Osaka

^dDepartment of Pediatrics, Jichi Medical University, School of Medicine, Tochigi, Japan
Shigeo Kure

^aDepartment of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan

Subject Editor:

Abstract

PDF Download

Abstract

Treatment of west syndrome in patients with tuberous sclerosis, the relevant effective period and doses of vigabatrin (VGB) to avoid serious side effects still needs further investigation. We report on a Japanese girl who showed good results with a very low dose of VGB. Tonic spasms appeared at 5 mo of age. Adrenocorticotropic hormone therapy resulted in incomplete seizure control. VGB at the lowest practical dose (30 mg/kg/d) showed complete control after 3 d. With reduction of the dose to 10 mg/kg/d, side effects such as hyperactivity, irritability, and sleep disturbances improved. She was seizure-free for the next 6 mo with an improved developmental quotient. Ophthalmological evaluation revealed no abnormality. The present case illustrates that low-dose VGB therapy (10 mg/kg/d) has fewer side effects and may bring prompt seizure control in west syndrome with tuberous sclerosis.

Keywords

West syndrome - infantile spasms - tuberous screlosis complex - vigabatrin

PDF (45 kb)