Acquired aphasic epilepsy (Landau-Kleffner syndrome). Report of 10 cases

Objective: The association of a language disorder with epilepsy is observed in some circumstance, with or without a causal relationship. In Landau-Kleffner syndrome (LKS), it is estimated that the aphasia is directly caused by epileptic discharges in language areas.

Methods: Ten children with LKS are studied. The clinical and electroencephalographic characteristics in these ten cases were analyzed.

Results: Aphasia was present between 3 years and 6 years 5 months of age (X: 4 years 8 months) in a progressive form in 8 cases and abruptly in 2 cases. The epileptic seizures present in nine children began between 22 months and 7 years 3 months of age (X: 4 years 1 month). Focal, multifocal and/or generalized discharges, unstable and variable, were frequently noted during awake state EEG records and on EEGs during the sleep state in four children continuous spike waves during 75-80% of slow sleep were observed.

Conclusions: We discuss the importance of the different clinical and EEG findings in the evolution of aphasia which condition the longterm prognosis, emphasizing the value of the discharges on the sleep EEGs. This suggests that LKS could be the severe form of a more widespread age-dependent epileptic syndrome that also includes the CSWS (epilepsy with continuous spike and waves during slow sleep) and the atypical benign partial epilepsy. Three syndromes have cognitive and behavioral manifestations and continuous spike waves during slow sleep.