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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

Naomi Hino-Fukuyo
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Yuko Sato
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Yosuke Kakisaka
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Wakaba Endo
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Yuki Kubota
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Atsuo Kikuchi
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Tomoko Kobayashi
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Kazuhiro Haginoya
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
b   Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai, Japan
,
Mitsugu Uematsu
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Yurika Numata
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
,
Hiroshi Doi
c   Department of Ophthalmology, Tohoku University, School of Medicine, Sendai, Japan
,
Masato Mori
d   Department of Pediatrics, Jichi Medical University, School of Medicine, Tochigi, Japan
,
Hitoshi Osaka
d   Department of Pediatrics, Jichi Medical University, School of Medicine, Tochigi, Japan
,
Shigeo Kure
a   Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan
› Author Affiliations
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Publication History

30 July 2013

03 January 2014

Publication Date:
18 July 2015 (online)

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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