When epilepsy interferes with word comprehension: findings in Landau-Kleffner syndrome

Landau-Kleffner syndrome is characterized by a regression in receptive language. The factors that affect the clinical expression of this syndrome remain unclear. This study presents neuroimaging findings in 2 patients showing different clinical evolutions. Linguistic regression persisted in 1 patient and evolved positively in the other. In patient A (with severe linguistic regression) there was an overlap between areas engaged during word recognition and those involved in generating the epileptiform activity; in patient B (with better linguistic evolution), receptive language was predominantly represented in the right hemisphere (unaffected). Patient A underwent multiple subpial transections. The 2-year follow-up indicated linguistic improvement, absence of epileptiform activity, and activation of the left temporal cortex during word comprehension. These results suggest that the resolution of the linguistic deficit in Landau-Kleffner syndrome may be modulated by the language-specific cortex freed from interfering epileptiform activity or by reorganization of the receptive language cortex triggered by the epileptic activity.