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Journal of Child Neurology
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Current Treatment of West Syndrome in Japan

Takeshi Tsuji, MD

Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan, ttsuji{at}med.nagoya-u.ac.jp

Akihisa Okumura, MD

Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan

Hiroshi Ozawa, MD

Department of Pediatrics, Shimada Center for Rehabilitation and Neurodevelopment Intervention, Tama-city Japan

Masatoshi Ito, MD

Department of Pediatrics, Shiga Medical Center for Children, Moriyama, Japan

Kazuyoshi Watanabe, MD

Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan, Faculty of Medical Welfare, Aichi Shukutoku University, Nagoya, Japan

About 10 years have passed since a previous survey on the treatment of West syndrome in Japan. To elucidate current practice, a questionnaire was sent to 113 institutes. It included (1) the drugs used for the treatment, (2) their dosage, and (3) the dosage and the schedule of adrenocorticotropic hormone therapy. Response rate was 51.3%. Adrenocorticotropic hormone, valproic acid, vitamin B6, and zonisamide were frequently used. Vitamin B6 was used most frequently as the first-choice drug followed by valproic acid, zonisamide, and adrenocorticotropic hormone. The most frequently used dose of synthetic adrenocorticotropic hormone-Z was 0.0125 mg/kg/d. Adrenocorticotropic hormone was administered every day for 2 weeks and then tapered off in more than 80% of the institutes. Although therapeutic strategy and drug usage have not changed largely during these 10 years, 2 alterations were observed: an increased use of zonisamide and a shortened duration of adrenocorticotropic hormone therapy.

Key Words: West syndrome • adrenocorticotropic hormone

Journal of Child Neurology, Vol. 22, No. 5, 560-564 (2007)
DOI: 10.1177/0883073807302606


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