Extremely low-dose vigabatrin for West syndrome with tuberous sclerosis

Naomi 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

Extremely low-dose vigabatrin for West syndrome with tuberous sclerosis

Naomi 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

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

1 Citation (Scopus)

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.

Original language	English
Pages (from-to)	255-258
Number of pages	4
Journal	Journal of Pediatric Epilepsy
Volume	2
Issue number	4
DOIs	https://doi.org/10.3233/PEP-14064
Publication status	Published - 2013

Keywords

Infantile spasms
Tuberous screlosis complex
Vigabatrin
West syndrome

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Clinical Neurology

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title = "Extremely low-dose vigabatrin for West syndrome with tuberous sclerosis",

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.",

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author = "Naomi Fukuyo and Yuko Sato and Yosuke Kakisaka and Wakaba Endo and Yuki Kubota and Atsuo Kikuchi and Tomoko Kobayashi and Kazuhiro Haginoya and Mitsugu Uematsu and Yurika Numata and Hiroshi Doi and Masato Mori and Hitoshi Osaka and Shigeo Kure",

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AU - Fukuyo, Naomi

AU - Sato, Yuko

AU - Kakisaka, Yosuke

AU - Endo, Wakaba

AU - Kubota, Yuki

AU - Kikuchi, Atsuo

AU - Kobayashi, Tomoko

AU - Haginoya, Kazuhiro

AU - Uematsu, Mitsugu

AU - Numata, Yurika

AU - Doi, Hiroshi

AU - Mori, Masato

AU - Osaka, Hitoshi

AU - Kure, Shigeo

PY - 2013

Y1 - 2013

N2 - 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.

AB - 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.

KW - Infantile spasms

KW - Tuberous screlosis complex

KW - Vigabatrin

KW - West syndrome

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Extremely low-dose vigabatrin for West syndrome with tuberous sclerosis

Abstract

Keywords

ASJC Scopus subject areas

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