癫痫性痉挛149例临床特征分析

Clinical study of 149 patients with epileptic spasms

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。

Objective To summary the clinical characteristics of epileptic spasm（ES）that is the unique form ofseizures in childhood. Methods A total of 149 patients with ES who visited our hospital from May 2009 to June 2015 werecollected. The documents of clinical data, treatment and outcome were analyzed retrospectively. The statistical analysis ofsome 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 patientsmanifested 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, inwhich 69 patients were with a clear cause. The proportions of developmental delay in the cryptogenic and symptomaticpatients were 74.5% and 92.2% after the onset of the ES. The degree of developmental delay was linearly related to thecourse of ES. The hyperarrhythmia of electroencephalogram（EEG） background was seen in 122 patients（81.9%）, and therewas no significant difference in hyperarrhythmia between different etiology groups or different onset age groups. The short-term efficiency of topiramate evaluated within one month was 30.5% in 59 newly diagnosed patients. The short- termeffective rate of adrenocorticotropic hormone（ACTH） was 80.3% in total 76 patients, but single ACTH treatment wasassociated with an increased recurrent rate. Conclusion ES is a highly age dependent seizure type that mainly found inWS,which typically follows a EEG hyperarrhythmia and developmental delay. The treatment of ES is difficult. ACTHcombined with effective antiepileptic drugs（AEDS） is currently the most reasonable drug treatment program.