Acquired epileptiform aphasia in children (Landau-Kleffner syndrome)

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.