Wang Shuang, Zhang Yue-hua, Bao Xin-hua, Wu Ye, Jiang Yu-wu, Liu Xiao-yan, Qin Jiong
Department of Pediatrics, First Hospital, Peking University, Beijing 100034, China.
Zhonghua Er Ke Za Zhi. 2006 Feb;44(2):105-9.
To investigate the clinical and electroencephalographic (EEG) characteristics, therapeutic response and long-term prognosis of Landau Kleffner syndrome (LKS).
The clinical and EEG data of 10 children with LKS were analyzed, and therapeutic response and long-term outcome were followed up.
The age of onset was from 2 to 10.5 years of age. All patients had acquired aphasia, characterized by verbal auditory agnosia. All patients had epileptic seizures. Partial motor seizures during sleep occurred in 8 patients, and other seizure type including atypical absence seizure and generalized tonic-clonic seizure were also observed. Psychological and behavioral abnormalities occurred in 9 patients. There were no abnormalities of hearing and neuro-imaging tests in all patients, and family histories were negative. All the patients had EEG abnormalities. Focal spike and waves of temporal lobe were recorded in 9 patients. Electrical status epilepticus during sleep (ESES) was observed on Video-EEG (VEEG) monitoring in 4 patients. Anti-epileptic drugs (AEDs) showed favorable effects on epileptic seizures, but no effects on aphasia. All patients responded to corticosteroid, and got language improved. Eight patients were followed up for long-term outcome. All patients were seizure free, while the level of language development was abnormal in 5 patients. The VEEG follow-up was conducted in 6 patients. Continuous epileptic discharges in slow sleep recurred in 2 patients after the discontinuation of steroid therapy.
LKS is one of the childhood epileptic encephalopathy, and acquired aphasia and epileptic seizures are two main clinical characteristics. Aphasia is characterized by verbal auditory agnosia. Psychological and behavioral abnormalities are very common in children with LKS. Focal epileptic discharges were often located in temporal area, and usually generalized, and could be continuous during sleep. AEDs could control seizure but had no effects on aphasia. Early use of full dose corticosteroids could improve the language significantly. Long-term follow up showed that language impairments often remained, but the outcome in terms of EEG and epileptic seizure was good.
探讨Landau Kleffner综合征（LKS）的临床及脑电图（EEG）特征、治疗反应及远期预后。
分析10例LKS患儿的临床及EEG资料，并对治疗反应及远期结局进行随访。
发病年龄为2至10.5岁。所有患者均有获得性失语，以言语听觉失认症为特征。所有患者均有癫痫发作。8例患者出现睡眠期部分运动性发作，还观察到其他发作类型，包括非典型失神发作和全身强直阵挛发作。9例患者出现心理和行为异常。所有患者听力及神经影像学检查均无异常，家族史均为阴性。所有患者EEG均有异常。9例患者记录到颞叶局灶性棘波和慢波。4例患者在视频脑电图（VEEG）监测中观察到睡眠期癫痫性电持续状态（ESES）。抗癫痫药物（AEDs）对癫痫发作有良好效果，但对失语无效。所有患者对皮质类固醇治疗均有反应，语言功能得到改善。8例患者进行了远期结局随访。所有患者癫痫发作均得到控制，但5例患者语言发育水平异常。6例患者进行了VEEG随访。2例患者在停用类固醇治疗后慢波睡眠期再次出现持续性癫痫放电。
LKS是儿童癫痫性脑病之一，获得性失语和癫痫发作是两个主要临床特征。失语以言语听觉失认症为特征。心理和行为异常在LKS患儿中非常常见。局灶性癫痫放电常位于颞区，通常为广泛性放电，且可在睡眠期持续。AEDs可控制癫痫发作，但对失语无效。早期使用足量皮质类固醇可显著改善语言功能。长期随访显示，语言障碍常持续存在，但EEG及癫痫发作方面的结局良好。
