获得性癫癎失语综合征5例报告

A report on Landau-Kleffner syndrome in 5 cases

目的：研究获得性癫癎失语综合征（Landau-Kleffner syndrome，LKS）的临床、脑电图（EEG）特征、治疗反应和远期预后。方法：对5例LKS患儿的临床表现、EEG特点和临床疗效进行观察，并结合文献进行探讨。结果：起病年龄为4～12岁。5例患儿均有不同类型的癫癎发作，伴有进行性失语，其中1例智力明显低下。5例均有EEG异常，其中3例有颞区局限性棘慢波发放。癫癎发作均可用抗癫癎药物控制。经皮质激素治疗，失语有改善。随访癫癎发作控制均良好，但语言恢复较正常差，EEG癎样放电好转。结论：LKS是一种以获得性失语和癫癎发作两大症状为主要临床表现的儿童时期癫癎性脑病。失语表现为听觉失认，多伴有其他精神行为异常。EEG常见颞区的局限性癎样放电，睡眠期常泛化至全导联，并可呈持续发放。抗癫癎药物可控制癫癎发作，但对失语疗效不佳。早期应用足量皮质激素治疗可明显改善失语情况。EEG及癫癎发作转归良好，但常遗留语言障碍。

Objective：To investigate the clinical and eleetroencephalographic（EEG） characteristics, therapeutic response and long-term prognosis of Landau Kleffner syndrome （LKS）. Methods： In accordauce with published literature, the clinical manifestation , EEG and clinical aspects of the 5 children with LKS were observed and discussed. Results： The age of onset was from 4 to 12 years old. Five patients had different types of epilepsy associated with recognitiive disturbance and progressive aphasia. All the patients had EEG abnormality. Focal spike and slow waves of temporal lobe were recorded in 3 patients. Anti-epileptic drugs （AEDs） showed favorable effects on epileptic seizures. Aphasla of all the patients were improved after cortical hormone treatment. In the follow-up period, epileptic seizure was well controlled, V-EEG epileptic discharge was improved, while language ability partly recovered）Conclusion： LKS is one of the childhood epileptic encephalopathy with acquired aphasia and epileptic seizures. Aphasia is characterized by verbal auditory agnosia. Psychological and behavioral abnormalities are very com- mon in children with LKS. Focal epileptic discharges are often located in temporal area, or usually generalized ,and could be continuous during sleep, AEDs may control seizure well but have no effects on aphasia. Early use of full dose corticosteroids can improve the language function significantly. Long-term follow-ups show that language impairment often remains although EEG turns nomal and seizures are well controlled.