使用氨己烯酸和 ACTH 治疗婴儿痉挛的临床特征和治疗-发展中国家的观点。
Clinical profile and treatment of infantile spasms using vigabatrin and ACTH--a developing country perspective.
机构信息
Medical College, The Aga Khan University, Stadium Road, Karachi 74800, Pakistan.
出版信息
BMC Pediatr. 2010 Jan 15;10:1. doi: 10.1186/1471-2431-10-1.
BACKGROUND
Infantile spasms represent a serious epileptic syndrome that occurs in the early infantile age. ACTH and Vigabatrin are actively investigated drugs in its treatment. This study describes the comparison of their efficacy in a large series of patients with infantile spasms from Pakistan.
METHODS
All patients with infantile spasms who presented to Aga Khan University Hospital, Karachi, Pakistan from January, 2006 to April, 2008 were included in this study. Inclusion criteria were clinical symptoms of infantile spasms, hypsarrythmia or modified hyparrythmia on electroencephalography, at least six months of follow-up period and receipt of any of the two drugs mentioned above. The type of drug distribution was random according to the availability, cost and ease of administration.
RESULTS
Fifty six cases fulfilled the inclusion criteria. 62.5% were males. Mean age at onset of seizures was 5 +/- 1.4 months. Fifty two (92.8%) patients demonstrated hypsarrythmia on electroencephalography. 64.3% cases were identified as symptomatic while 19.6% were cryptogenic and 16.1% were idiopathic. Eighteen patients received ACTH while 38 patients received Vigabatrin as first line therapy. Initial response to first line therapy was similar (50% for ACTH and 55.3% for Vigabatrin). Overall, the symptomatic and idiopathic groups responded better to Vigabatrin. The relapse rate was higher for ACTH as compared to Vigabatrin (55.5% vs. 33.3%) when considering the first line therapy. Four patients evolved to Lennox-Gastaut variant; all of these patients had initially received Vigabatrin and then ACTH.
CONCLUSION
Vigabatrin and ACTH showed no significant difference in the initial treatment of infantile spasms. However, patients receiving ACTH were 1.2 times more likely to relapse as compared to the patients receiving Vigabatrin when considering monotherapy. We suggest that Vigabatrin should be the initial drug of choice in patients presenting with infantile spasms. However, larger studies from developing countries are required to validate the therapeutic trends observed in this study.
背景
婴儿痉挛症是一种发生在婴儿早期的严重癫痫综合征。ACTH 和氨己烯酸是其治疗的研究热点药物。本研究描述了在巴基斯坦的一项大规模婴儿痉挛症患者系列研究中，这两种药物的疗效比较。
方法
本研究纳入了 2006 年 1 月至 2008 年 4 月期间在卡拉奇 Aga Khan 大学医院就诊的所有婴儿痉挛症患者。纳入标准为：婴儿痉挛症的临床症状、脑电图上出现高波幅失律或改良高波幅失律、至少 6 个月的随访期以及接受了上述两种药物中的任何一种。药物的分配类型根据药物的可获得性、成本和给药的便利性随机选择。
结果
共有 56 例患者符合纳入标准。男性占 62.5%。癫痫发作的平均发病年龄为 5 岁±1.4 岁。52 例（92.8%）患者脑电图上出现高波幅失律。64.3%的病例为症状性，19.6%为隐源性，16.1%为特发性。18 例患者接受 ACTH 治疗，38 例患者接受氨己烯酸作为一线治疗。一线治疗的初始反应相似（ACTH 为 50%，氨己烯酸为 55.3%）。总体而言，症状性和特发性组对氨己烯酸的反应更好。考虑一线治疗时，ACTH 的复发率高于氨己烯酸（55.5%比 33.3%）。4 例患者进展为 Lennox-Gastaut 变异型；这些患者最初均接受氨己烯酸治疗，然后接受 ACTH 治疗。
结论
在婴儿痉挛症的初始治疗中，ACTH 和氨己烯酸没有显著差异。然而，考虑到单药治疗，接受 ACTH 的患者复发的可能性是接受氨己烯酸的患者的 1.2 倍。我们建议在婴儿痉挛症患者中，氨己烯酸应作为一线药物。然而，需要来自发展中国家的更大规模的研究来验证本研究中观察到的治疗趋势。
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