Imai Yuki, Yoshinaga Harumi, Ishizaki Yumiko, Watanabe Yoshiaki, Ohtsuka Yoko
Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama.
No To Hattatsu. 2009 Nov;41(6):457-61.
Vitamin B6 (VB6) is used frequently as one of the first-choice drug for the treatment of West syndrome (WS) in Japan. We report 2 cases of symptomatic WS who had a good response to readministration of VB6. Case 1 was a 3-year-old girl diagnosed as having severe hydroencephalus. She developed WS at the age of 9 months. She was treated with ACTH, but relapsed when she was 1 year old. Despite treatment with various conventional drugs and second ACTH therapy, her seizures were not suppressed. We reviewed past treatment records in another hospital, and found that VB, treatment was stopped because her EEG did not improve within a week. We then retried VB6 therapy when she was 3 years and 6 months old, and as a result she became seizure-free and hypsarrhythmia disappeared on EEG within a month. Case 2 was a boy with severe cerebral palsy who was diagnosed as having WS at the age of 9 months. His MRI revealed bilateral subdural hematoma. Treatment was started with VB6, and he became seizure-free within several days. But 7 days after starting VB6, treatment was stopped because of the side effects. VPA was started instead, but his EEG showed gradual worsening. Then, we added a smaller dose of VB6 to VPA. His EEG improved dramatically, and his seizures have been controlled without any side effects. The present cases indicate the possible clinical usefulness of successful VB6 retrials even in older patients with severe organic lesions, by means of combined therapy with other drug, and alternative design of the dosing. However, we suspect there might be many cases in which the efficacy of VB6 therapy has not been properly assessed due to the short observation period and/or side effects.
在日本，维生素B6（VB6）常被用作治疗韦斯特综合征（WS）的首选药物之一。我们报告2例有症状的WS患者，再次使用VB6后反应良好。病例1是一名3岁女孩，诊断为重度脑积水。她9个月大时患上WS。曾接受促肾上腺皮质激素（ACTH）治疗，但1岁时复发。尽管使用了各种传统药物并再次进行ACTH治疗，她的癫痫发作仍未得到控制。我们查阅了她在另一家医院的既往治疗记录，发现由于脑电图在一周内没有改善，VB6治疗被停用。当她3岁6个月大时，我们再次尝试VB6治疗，结果她在一个月内癫痫发作停止，脑电图上的高峰节律紊乱消失。病例2是一名患有重度脑瘫的男孩，9个月大时被诊断为WS。他的磁共振成像（MRI）显示双侧硬膜下血肿。开始使用VB6治疗，几天后他的癫痫发作停止。但开始使用VB6 7天后，由于副作用而停药。转而开始使用丙戊酸（VPA），但他的脑电图显示逐渐恶化。然后，我们在VPA中添加了较小剂量的VB6。他的脑电图显著改善，癫痫发作得到控制且没有任何副作用。目前的病例表明，即使是患有严重器质性病变的老年患者，通过与其他药物联合治疗以及调整给药方案，成功再次使用VB6可能具有临床实用性。然而，我们怀疑由于观察期短和/或副作用，可能有许多病例中VB6治疗的疗效未得到恰当评估。
