Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Epileptic Disord. 2009 Dec;11(4):339-44. doi: 10.1684/epd.2009.0283. Epub 2010 Jan 4.
Infantile spasms are usually resistant to conventional antiepileptic drugs. Although adrenocorticotropic hormones or vigabatrin are regarded as standard agents for the treatment of infantile spasms, there are still limitations of their use. We determined the efficacy and tolerability of high-dose zonisamide in patients with recent-onset infantile spasms.
Seventeen patients with infantile spasms, who were admitted to our hospital between October 2005 and November 2007, were eligible for enrollment within two months of diagnosis. Zonisamide was administered at a starting dose of 2-8 mg/kg/day, increasing by 2-5 mg/kg/day every three to four days until the seizures disappeared or the dose reached 30 mg/kg/day. Complete response was defined as clinical cessation of infantile spasms over 28 consecutive days and disappearance of hypsarrhythmia by EEG analysis.
Of the 17 treated patients (nine who received initial monotherapy and eight add-on therapy), five of 12 (42.0%) cryptogenic patients and two of five (40.0%) symptomatic patients showed complete disappearance of spasms. The maximum daily dose was 10-28 mg/kg, and the effective daily dose was 10-22 mg/kg. Mean time period before disappearance of spasms and hypsarrhythmia was eight days. Seizure recurrence was observed in three of the seven patients who showed complete disappearance of spasms. Adverse effects included irritability in four patients and poor oral intake in two patients.
Although further study of zonisamide is warranted, high-dose zonisamide can be effective and safe in some infants with newly diagnosed West syndrome.
婴儿痉挛症通常对传统抗癫痫药物具有耐药性。虽然促肾上腺皮质激素或氨己烯酸被认为是治疗婴儿痉挛症的标准药物，但它们的使用仍存在局限性。我们确定了高剂量佐尼沙胺治疗新近诊断的婴儿痉挛症患者的疗效和耐受性。
2005 年 10 月至 2007 年 11 月期间，我院收治的 17 例婴儿痉挛症患者，在确诊后 2 个月内符合入组条件。佐尼沙胺起始剂量为 2-8mg/kg/天，每 3-4 天增加 2-5mg/kg/天，直至发作停止或剂量达到 30mg/kg/天。完全缓解定义为临床停止痉挛 28 天以上，脑电图分析显示高波幅失律消失。
17 例治疗患者（9 例初始单药治疗，8 例加用治疗）中，12 例隐源性患者中有 5 例（42.0%）和 5 例症状性患者中的 2 例（40.0%）痉挛完全消失。最大日剂量为 10-28mg/kg，有效日剂量为 10-22mg/kg。痉挛和高波幅失律消失前的平均时间为 8 天。7 例痉挛完全消失的患者中有 3 例出现发作复发。不良反应包括 4 例患者出现烦躁不安和 2 例患者出现进食不佳。
尽管需要进一步研究佐尼沙胺，但高剂量佐尼沙胺对一些新诊断的 West 综合征婴儿可能是有效且安全的。
