Landau-Kleffner 综合征的临床特征和远期预后

目的研究Landau-Kleffner综合征（LKS）的临床、脑电图特征、治疗反应和远期预后。方法对10例LKS患儿的临床及脑电图资料进行分析,并随访治疗效果及远期预后。结果起病年龄2～10、5岁。10例患儿均有获得性失语,表现为听觉失认。均有癫癎发作,8例有睡眠中部分运动性发作,其他发作形式还有不典型失神发作和全身强直一阵挛发作。9例伴有精神行为异常。10例均无听力异常、神经影像学异常及家族史。10例均有脑电图异常,9例有颞区限局性棘慢波发放,8例进行录像脑电图（VEEG）监测,4例发现睡眠中癫疴性电持续状态。10例癫疴发作均可用抗癫疴药物控制。经皮质激素治疗,10例失语均有改善。随访8例,癫癎发作控制均良好,5例语言较正常差。6例随访VEEG,2例停用激素后再次出现睡眠中持续放电。结论LKS是一个以获得性失语和癫疴发作为主要临床表现的儿童时期癫癎脑病。失语表现为听觉失认,多伴有其他精神行为异常。EEG常见颞区的限局性放电,睡眠期常泛化全导,并可呈持续发放。抗癫疴药物可控制癫疴发作,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。

Clinical characteristics and long-term prognosis of Landau-Kleffner syndrome

Objective To investigate the clinical and electroencephalographic(EEG) characteristics,therapeutic response and long-term prognosis of Landau Kleffner syndrome(LKS).MethodsThe clinical and EEG data of 10 children with LKS were analyzed,and therapeutic response and long-term outcome were followed up.Results 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.Conclusions 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.