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Extremely low-dose vigabatrin for West syndrome with tuberous sclerosis

Article type: Other

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

Affiliations: Department of Pediatrics, Tohoku University, School of Medicine, Sendai, Japan | Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai, Japan | Department of Ophthalmology, Tohoku University, School of Medicine, Sendai, Japan | Department of Pediatrics, Jichi Medical University, School of Medicine, Tochigi, Japan

Note: [] Corresponding author: Dr. Naomi Hino-Fukuyo, Department of Pediatrics, Tohoku University School of Medicine, Seiryo-Machi 1-1, Aoba-ku, Sendai 980-8574, Japan. Tel.: +81 22 717 7287; Fax: +81 22 717 7290; E-mail: naomi-h@zc4.so-net.ne.jp.

Keywords: West syndrome, infantile spasms, tuberous screlosis complex, vigabatrin

DOI: 10.3233/PEP-14064

Journal: Journal of Pediatric Epilepsy, vol. 2, no. 4, pp. 255-258, 2013

Received 30 July 2013
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Revision received 26 December 2013
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Accepted 3 January 2014
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Published: 2013
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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.