Aicardi J
Institute of Child Health, University College, Londres, Gran Bretaña.
Rev Neurol. 1999;29(4):380-5.
The Landau-Kleffner syndrome consists of the association of paroxysmal EEG abnormalities, more marked during sleep, acquired aphasia usually of receptive type, and epileptic seizures in three quarters of cases. Additional features include behavioral disturbances that may even present with autistic features, cognitive regression of variable degree, and sometimes motor difficulties, indicating the pervasive nature of the disorder. The epileptic activity, as manifested by the EEG, seems responsible for the pervasive dysfunction. Treatment with antiepileptic drugs is often ineffective. ACTH and corticosteroids are currently regarded as the best therapy but surgical treatment by subpial transection is being actively studied.
Landau-Kleffner综合征包括阵发性脑电图异常（睡眠期间更为明显）、通常为感受性失语以及四分之三的病例伴有癫痫发作。其他特征包括甚至可能表现出自闭症特征的行为障碍、不同程度的认知衰退，有时还有运动困难，表明该疾病具有广泛性。脑电图显示的癫痫活动似乎是广泛性功能障碍的原因。抗癫痫药物治疗往往无效。促肾上腺皮质激素和皮质类固醇目前被认为是最佳治疗方法，但软膜下横切术的手术治疗正在积极研究中。
