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Landau—kleffner综合征(L—KS)又称伴有惊厥性疾病的获得性失语(Acquired aphasia with convulsive disorder)。1957年由Landau和kleffner首次报道。临床上较为罕见,到1990年文献报道仅有160例,国内有2例报道。1985年癫痫综合征国际分类将其定为一独立的类型,1989年的修正分类亦然。随着累积病例的增加,有关L—KS是一独立的临床综合征,还是儿童良性功能性部分性癫痫的一个特殊类型尚有争议,Landau本人也对其原命名提出异议。目前该病以临床表现和脑电图(EEG)改变做出诊断,尚无统一的诊断标准。现较为完善的定义是:获得性的高级皮层功能改变伴有睡眠期EEG异常活动,癫痫预后良好。
Landau-kleffner syndrome (L-KS), also known as Acquired aphasia with convulsive disorder. First reported by Landau and kleffner in 1957. Clinically rare, to the literature in 1990 reported only 160 cases, two cases were reported. The 1985 International Classification of Epilepsy made it an independent category, as amended in 1989. With the cumulative number of cases, there is debate as to whether L-KS is an independent clinical syndrome or a particular type of benign functional partial epilepsy in children, and Landau himself contested his original nomenclature. Currently the disease to clinical manifestations and changes in electroencephalogram (EEG) to make a diagnosis, there is no uniform diagnostic criteria. Now more perfect definition is: acquired high-grade cortical function changes associated with sleep abnormal EEG activity, epilepsy prognosis is good.