Hattori Ayako, Ando Naoki, Hamaguchi Kiyo, Hussein Mohamed Hamed, Fujimoto Shinji, Ishikawa Tatsuya, Togari Hajime
Department of Pediatrics, Neonatology and Congenital Disorders, Nagoya City University, Graduate School of Medical Sciences, Kawasumi, Nagoya, Japan.
Pediatr Neurol. 2006 Dec;35(6):415-8. doi: 10.1016/j.pediatrneurol.2006.07.002.
In this study, seven patients with cryptogenic West syndrome were subjected to short-duration adrenocorticotrophic hormone (ACTH) therapy. ACTH was administered daily for 7 to 12 days (mean 10 days), and then was withdrawn. Daily single dose was 0.022 to 0.027 mg/kg/day (0.024 mg/kg/day, 0.96 IU/kg), total dose was 0.17 to 0.28 mg/kg (0.23 mg/kg, 9.0 IU/kg), and treatment lag was 12 to 105 days (median 14 days). ACTH therapy controlled tonic spasms with no serious side effects in all patients, but two patients still had electroencephalographic abnormality. The intelligence quotients or developmental quotients of six patients with short treatment lag were 79 to 110 at the age of 2 to 6 years; only one patient with long treatment lag had a developmental quotient of 60. This new short-duration ACTH therapy could yield better cognitive outcomes for cryptogenic West syndrome.
在本研究中，7例隐源性韦斯特综合征患者接受了短期促肾上腺皮质激素（ACTH）治疗。ACTH每日给药7至12天（平均10天），然后停药。每日单剂量为0.022至0.027毫克/千克/天（0.024毫克/千克/天，0.96国际单位/千克），总剂量为0.17至0.28毫克/千克（0.23毫克/千克，9.0国际单位/千克），治疗延迟为12至105天（中位数14天）。ACTH治疗控制了所有患者的强直性痉挛，且无严重副作用，但有2例患者仍有脑电图异常。6例治疗延迟短的患者在2至6岁时的智商或发育商为79至110；只有1例治疗延迟长的患者发育商为60。这种新的短期ACTH治疗可为隐源性韦斯特综合征带来更好的认知结果。
