Landau-Kleffner综合征六例  

Six cases reports for Landau-Kleffner syndrome

作　　者：李志梅[1] 崔韬[1] 

机构地区：[1]首都医科大学附属北京天坛医院神经内科,北京100050

出　　处：《脑与神经疾病杂志》2015年第3期184-187,共4页Journal of Brain and Nervous Diseases

基　　金：北京市优秀人才培养基金项目(2013D003034000045)

摘　　要：目的：研究Landau-Kleffner综合征（LKS）的临床、脑电图（EEG）特征、治疗反应及预后。方法对6例LKS患儿的临床及EEG资料进行分析，并对其治疗效果进行随访。结果该组患儿起病年龄3～8岁，男女比例2：1。起病前智力及语言发育正常。无癫痫家族史。均有获得性失语，表现为听觉性失认。均出现癫痫发作，表现为部分性发作、全面强直阵挛发作和不典型失神发作。听力检查正常。 EEG均异常，清醒期EEG显示单侧或双侧颞区为著高波幅棘慢波；睡眠期EEG显示癫痫样放电均较清醒期明显增多，并泛化至全导，其中2例表现为慢波睡眠期持续性棘慢复合波。头颅磁共振（ MRI）检查均正常。6例患儿经抗癫痫药物治疗其癫痫发作均完全控制。经皮质激素治疗，3例患儿失语完全恢复，2例部分恢复，1例未恢复。结论 Landau-Kleffner综合征是以获得性失语和癫痫发作为主要临床表现的儿童期癫痫综合征。 EEG表现为以颞区为著的癫痫样放电，睡眠期全导泛化。癫痫发作经抗癫痫药物治疗可以控制且转归良好。早期合理应用皮质激素可以改善失语状况，但仍有患儿遗留语言障碍。ObjectiveToinvestigatetheclinicalandelectroencephalographic（EEG）characteristics, therapeutic response and prognosis of Landau -Kleffner syndrome （LKS）.Metho ds We analyzed the clinical and EEG data of 6 children with LKS,and then followed up the therapeutic response and the long-term outcome.Rseults The age of onset was from 3 to 8 years old.Epileptic family histories were negative .All patients had acquired aphasia,characterized by verbal auditory agnosia.All patients had epileptic seizure, including partial seizure, generalized tonic-clonic seizure and atypical absence .There were no abnormalities of hearing test in all patients .All patients had EEG abnormalities , Repetitive, high-amplitude focal spike and waves of temporal lobe were observed during wakefulness , Epileptiform discharges were activated by sleep , Electrical status epilepticus during sleep were observed in 2 patients.MRI scan of brain were all normal.Anti-epileptic drugs showed favorable effects in all paitients . All patients were seizure free .All patients were treated with corticosteroid in 5 patients got languge improved and 3 patients fully recovered .Conclusion LKS is one of the children epileptic syndrome .Acquired aphasia and epileptic seizure were two main clinical characteristics .Focal epileptic discharges were often located in temporal lobe , and usually generalized and could be continuous during sleep .AEDs could control seizure .Early use of full dose corticosteroid could improve the language significantly but the language impairments remained in some cases .

关 键 词：LANDAU-KLEFFNER综合征 癫痫 失语 

分 类 号：R742.1[医药卫生—神经病学与精神病学]