Atypical evolutions of benign localization-related epilepsies in children:Are they predictable?

Purpose: To emphasize that, in some patients, different atypical evolutions occur in the course of so-called benign focal epilepsies of childhood (BFE C) and to promote interest in finding clinical and/or electroencephalograph ic (EEG) clues to which patients might be prone to these risky evolutions. Methods: Twenty-six patients who started with the typical clinical and EEG features of benign childhood epilepsy with centrotemporal spikes (BCECTS) b ut who had reversible or persistent, serious epileptic events including sta tus epilepticus and language, cognitive, or behavioral impairments were fol lowed for less than or equal to 14 years. Repeated neurologic examinations, EEG records, and neuropsychological evaluations were done, and brain-imagi ng studies [computerized axial tomography (CAT) or magnetic resonance imagi ng (MRI)] were obtained in all patients. Results: The 26 patients were in four separate groups according to the natu re of their atypical evolution. Eleven children had atypical benign focal e pilepsy of childhood (ABFEC), three with Landau-Kleffner syndrome (LKS), se ven with status epilepticus of BCECTS, and five with mixed features of the other three groups. All the children whose BCECTS evolved into ABFEC have f inally recovered and are attending normal schools, although five have learn ing difficulties. Two of the three patients diagnosed with LKS recovered fr om aphasia, although some language difficulties persist in one. The seven w ho showed status epilepticus of BCECTS are now normal after 3-14 years of f ollow-up, and three of the five children showing mixed features fulfilled t he criteria for a diagnosis of epilepsy with continuous spikes and waves du ring slow sleep (CSWS). Conclusions: A small proportion of cases starting with BCECTS evolve into A BFEC, LKS, status of BCECTS, or epilepsy with CSWS. In such cases, BCECTS i s not always benign. Clinical and EEG markers should be sought to predict t hese atypical evolutions.