West综合征32例临床分析

Clinical analysis of 32 cases of West syndrome

目的探讨West综合征患儿的临床表现、脑电图特征、诊断以及治疗和预后,提高对该病的认识。方法回顾性研究32例诊断为West综合征患儿的临床资料。结果 32例West综合征患儿中,男、女之比为23∶9;年龄5个月至3岁;临床表现为典型的"三联征"患儿28例(87.5%);所有患儿脑电图均为异常(典型高峰失律24例,变异型高峰失律8例)。32例患儿中,有4例肌注ACTH及口服抗癫痫药,痉挛完全控制2例,发作次数明显减少1例,无效1例;余28例患儿仅口服抗癫痫药,17例痉挛发作完全控制,6例发作次数明显减少,5例无效。随访16例患儿,2个月内有11例患儿痉挛发作完全控制,3例有效,2例无效。结论 West综合征是婴儿时期特有的一种癫痫类型,且在智力发育时期发病,常伴有脑损伤后遗症。除典型的发作形式和精神运动障碍外,还有特异的脑电图改变。

Objective To explore the clinical manifestations, EEG findings, diagnosis, therapy and prognosis of West syndrome. Methods The clinical data of 32 children with West syndrome were retrospectively studied. Results Of the 32 patients, there were 23 boys and 9 girls, and aged from 5 months to 3 years old. The classic triad （ entasia, developental regression and hypasarrhythmia） was seen in 28 children. All children＇ s EEG results were abnormal （ classic hypasarrhythmia in 24 cases and atypical hypasarrhythmia in 8 cases）. Four children were treated with ACTH and antiepilepfic drugs （AEDs）. After treatment, 2 children became spasm-free, 1 had more than 50% spasm reduction, and 1 did not respond to the treatment. The other 28 children were treated only with AEDs, and of them 17 became spasm-free, 6 had more than 50% spasm-free, 5 did not respond to the treatment. 16 cases were followed up for 2 months, and 11 cases of them were spasm-free, 3 cases showed less than 50% erduction and 2 did not respond to the treatment. Conclusions West syndrome is a special epileptic syndrome in the infantile period, and the onset is in the intelligence development period. The patients usually have brain damage sequelae. In addition to typital attacks and spiritual movement disorders, there are specific EEG changes in these patients.