获得性癫痫性失语综合征的临床特点分析

目的 研究中国人获得性癫痫性失语(Landau-Kleffner Syndrome,LKS)的临床、脑电图及治疗特点.方法 对中国大陆地区报道的文献中71例LKS患儿的临床资料进行回顾总结,并结合文献进行探讨.结果 获得性癫痫失语均发作于儿童期,临床均有失语及脑电图癫痫样放电,其中以混合性失语及腩电图棘-慢复合波为多见.多数临床有癫痫发作,听力均无异常,多数伴有精神行为异常.癫痫发作用抗癫痫药物控制良好,经皮质激素及免疫球蛋白的治疗,失语大多能改善.结论 中国儿童获得性癫痫性失语主要临床表现为获得性失语和癫痫发作,多伴有其它精神行为症状.EEG常见颞区的限局性放电,失语表现为听觉失认.早期诊断并采取包括抗癫痫药物和皮质激素应用的综合治疗,患者的预后总体良好.

An analysis of the clinical characteristics of Chinese acquired epileptic aphasia

Objective To investigate the clinical electroencephalographic(EEG)and therapeutic characteristics of Chinese acquired epileptic aphasia(also known as Landau-Kleffner syndrome-LKS).Methods The clinical data of 71 children with LKS in the literatures published in China mainland were retrospectively analyzed and discussed by combining published literatures.Results Seizures of LKS occured in childhood.All patients had acquired aphasia and EEG epilepti-form discharges,with mixed aphasia and EEG spike-slow wave for the more common.The majority of patients had seizures.There were no abnormalities of hearing and the majority of patients were accompanied by the psychological and behavioral abnormalities.Anti-epileptic drags(AEDs)showed favorable effectes on epileptic seizures.With glucocorticoid and immunoglobulin treatment,most apasia can be improved.Conclusions The two main clinical characteristics of acquired aphasia and epileptic seizures occurred in Chinese children with acquired epileptic aphasia.Psychological and behavioral abnormalities were very common in children.Aphasia was characterized by verbal auditory agnosia.Focal epileptic discharges were located in temporal area.Early diagnosis and comprehensive treatment ie AEDs combined with glucocorticoid can lead to a good prognosis.