天津医药

天津医药 ›› 2017, Vol. 45 ›› Issue (1): 58-63.doi: 10.11958/20160777

• 临床研究 • 上一篇    下一篇

癫痫性痉挛 149 例临床特征分析

张培元， 许海泉， 吴波， 张玉琴   

  1. 天津市儿童医院神经内科 （邮编 300074）
  • 收稿日期:2016-08-05 修回日期:2016-11-02 出版日期:2017-01-15 发布日期:2017-01-15

epilepsy； adrenocorticotropic hormone; epileptic spasms； hyperarrhythmia

ZHANG Pei-yuan, XU Hai-quan, WU Bo, ZHANG Yu-qin   

  1. Department of Neurology, Tianjin Children’ s Hospital, Tianjin 300074, China
  • Received:2016-08-05 Revised:2016-11-02 Published:2017-01-15 Online:2017-01-15

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摘要： 摘要： 目的 总结癫痫性痉挛（ES）这一儿童期特有的癫痫发作形式的临床特征。方法 选取 2009 年 5 月— 2015 年 6 月我院就诊的 ES 患儿 149 例， 回顾性分析其临床资料、 治疗和转归情况， 对病例的某些临床特征进行统计 学分析。结果 149 例患儿中男 97 例 （65.1%）、 女 52 例 （34.9%）， ES 起病年龄 30 d～42 个月 （中位数 6 个月）， 高峰 年龄 4～7 个月， 超过 12 个月起病 17 例 （11.4%）。147 例表现成串的痉挛发作， 1 对双生子仅表现单次痉挛发作， 13 例 （8.7%） 在 ES 起病前存在其他癫痫发作类型， 42 例 （28.2%） 伴有其他类型发作。符合 West 综合征 （WS） 诊断 83 例 （55.7%）； 隐源性病例 46 例（30.9%）， 症状性 103 例（69.1%）， 其中 69 例病因明确。起病后， 隐源性与症状性病例发 育落后的比例分别为 74.5%、 92.2%， 发育落后的程度与 ES 病程呈线性相关。脑电图（EEG）背景显示高度失律 122 例 （81.9%）， 在不同病因及不同起病年龄的分布差异无统计学意义。初诊加用托吡酯 59 例， 治疗 1 个月内的短期有 效率 30.5%； 总计经促肾上腺皮质激素（ACTH）治疗 76 例， 短期有效率 80.3%， 单用 ACTH 复发比例高。结论 ES 是一种具有高度年龄依赖性的癫痫发作类型， 主要见于 WS， 多伴有高度失律脑电图及起病后精神运动发育倒退。 ES 治疗困难， ACTH 联合有效的抗癫痫药物 （AEDS） 是目前最合理的药物治疗方案。

关键词: 癫痫, 促肾上腺皮质激素, 癫痫性痉挛, 高度失律

Abstract: Abstract： Objective To summary the clinical characteristics of epileptic spasm（ES）that is the unique form of seizures in childhood. Methods A total of 149 patients with ES who visited our hospital from May 2009 to June 2015 were collected. The documents of clinical data, treatment and outcome were analyzed retrospectively. The statistical analysis of some clinical characteristics and prognosis of patients were performed. Results There were 97(65.1% ) male and 52 (34.9% ) female in the 149 patients． The onset age of ES was from 30 days to 42 months (median value was 6 months) including 17 patients more than 12 months．The peak age of onset was 4-7 months． One hundred and forty-seven patients manifested clustered spasm. One pair of twins showed single spasm．Other types of seizure were seen before ES onset in 13 (8.7%) patients, and after ES onset in 42 (28.2%) patients．Eighty-three patients (55.7%) were diagnosed as West syndrome (WS). There were 46 patients (30.9%) with cryptogenic cause, 103 patients (69.1%) diagnosed with symptomatic epilepsy, in which 69 patients were with a clear cause. The proportions of developmental delay in the cryptogenic and symptomatic patients were 74.5% and 92.2% after the onset of the ES. The degree of developmental delay was linearly related to the course of ES. The hyperarrhythmia of electroencephalogram (EEG) background was seen in 122 patients (81.9%), and there was no significant difference in hyperarrhythmia between different etiology groups or different onset age groups. The shortterm efficiency of topiramate evaluated within one month was 30.5% in 59 newly diagnosed patients. The short- term effective rate of adrenocorticotropic hormone (ACTH) was 80.3% in total 76 patients, but single ACTH treatment was associated with an increased recurrent rate. Conclusion ES is a highly age dependent seizure type that mainly found in WS， which typically follows a EEG hyperarrhythmia and developmental delay. The treatment of ES is difficult. ACTH combined with effective antiepileptic drugs (AEDS) is currently the most reasonable drug treatment program.

Key words: epilepsy； adrenocorticotropic hormone, epileptic spasms； hyperarrhythmia