Ando Naoki, Fujimoto Shinji, Ishikawa Tatsuya, Kobayashi Satoru, Hattori Ayako, Ito Tetsuya, Togari Hajime
Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi.
No To Hattatsu. 2010 Nov;42(6):444-8.
Vigabatrin (VGB) is one of the most effective anti-epileptic drugs for tonic spasms, those accompanied with tuberous sclerosis complex (TSC), but is not available in Japan. We treated 7 patients with West syndrome (WS) and TSC with VGB. In these patients, VGB treatment was started at 5-65 months of age. Six patients (86%) had complete cessation of tonic spasms. Of these, 3 patients had complete cessation within 24 hours after VGB treatment. The mean initial dosage of VGB was 36.2 mg x kg(-1) x day(-1), and the mean maintenance dosage was 38.4 mg x kg(-1) x day(-1). At the beginning of VGB treatment, 3 patients had hypsarrhythmia, 2 had focal discharge with generalization, and 2 had only focal discharge on electroencephalography. Hypsarrhythmia disappeared within 4-8 weeks after VGB treatment. Behavioral problems and sleep difficulty were observed in 6 patients. Visual field examination revealed no abnormalities in 3 patients. We hope that patients with WS and TSC can be treated with VGB as soon as possible in Japan.
氨己烯酸（VGB）是治疗强直痉挛（包括伴有结节性硬化症（TSC）的强直痉挛）最有效的抗癫痫药物之一，但在日本无法获得。我们用VGB治疗了7例患有韦斯特综合征（WS）和TSC的患者。这些患者在5至65个月大时开始接受VGB治疗。6例患者（86%）的强直痉挛完全停止。其中，3例患者在VGB治疗后24小时内强直痉挛完全停止。VGB的平均初始剂量为36.2mg·kg⁻¹·d⁻¹，平均维持剂量为38.4mg·kg⁻¹·d⁻¹。在开始VGB治疗时，3例患者有高度节律失调，2例有局灶性放电伴泛化，2例脑电图仅有局灶性放电。高度节律失调在VGB治疗后4至8周内消失。6例患者出现行为问题和睡眠困难。视野检查显示3例患者无异常。我们希望在日本，患有WS和TSC的患者能够尽快接受VGB治疗。
