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儿童获得性癫痫性失语( Landau-Kleffner综合征)

Acquired epileptiform aphasia in children (Landau-Kleffner syndrome).

作者信息

Deonna T W

机构信息

Neuropediatric Unit, C.H.U.V., Lausanne, Switzerland.

出版信息

J Clin Neurophysiol. 1991 Jul;8(3):288-98. doi: 10.1097/00004691-199107010-00005.

Abstract

The association of a language disorder with epilepsy is frequent in children, but there is usually no causal relationship. In acquired epileptiform aphasia (AEA), the so-called Landau-Kleffner syndrome, there is increasing evidence that the language disorder is directly caused by epileptic discharges in critical language areas and must be viewed as a special kind of epileptic aphasia. This is based on a review of the published cases of AEA over the last 30 years and on the analogies that can be made between AEA and other epileptic syndromes, mainly benign partial epilepsy with centrotemporal spikes. AEA can start early in development and present as developmental dysphasia. It is only one among other cognitive or behavioral disturbances that can be epileptic manifestations of some particular epileptic syndromes, for example, epilepsy with continuous spike waves during slow sleep, which probably has the same pathophysiology as AEA. AEA must be seen, at least in some cases, as a particular form of resistant epilepsy. AEA is an important model because it suggests that isolated cognitive and behavioral disturbances can be epileptic manifestations in children.

摘要

在儿童中,语言障碍与癫痫的关联很常见,但通常不存在因果关系。在获得性癫痫性失语(AEA),即所谓的Landau-Kleffner综合征中,越来越多的证据表明,语言障碍是由关键语言区域的癫痫放电直接引起的,必须被视为一种特殊类型的癫痫性失语。这是基于对过去30年已发表的AEA病例的回顾,以及AEA与其他癫痫综合征(主要是伴有中央颞区棘波的良性部分性癫痫)之间的类比得出的。AEA可在发育早期起病,表现为发育性语言障碍。它只是一些特定癫痫综合征的癫痫表现所导致的其他认知或行为障碍之一,例如慢波睡眠期持续棘慢波癫痫,其病理生理机制可能与AEA相同(至少在某些情况下)。AEA必须被视为一种特殊形式的难治性癫痫。AEA是一个重要的模型,因为它表明孤立的认知和行为障碍可能是儿童癫痫的表现。

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