The natural history of benign childhood epilepsy with centrotemporal spikes (BCECTS), BCECTS Plus syndromes, Rolandic epilepsy-related syndromes, and idiopathic benign childhood epilepsy with occipital spikes (Panayiotopoulos syndrome) is reviewed by researchers at Maggiore Hospital, Bologna, Italy. These syndromes constitute a group of agedependent idiopathic epilepsy disorders with a characteristic EEG pattern and neuropsychological deficits related to ongoing paroxysmal activity. These syndromes forming a continuum or spectrum have a common underlying mechanism, modulated by the duration, localization, and age of onset of the spike-wave discharge. Some consider the syndromes to represent a type of “epileptic encephalopathy.” Continuous spike-waves during sleep syndrome (CSWS) is included as an epileptic encephalopathy, a condition in which the epileptiform abnormalities are believed to contribute to a global regression of cognitive function and behavior. The role of the genetic predisposition to BCECTS and related syndromes requires further elucidation. [1]

COMMENT. Recent studies have questioned the benign nature of BCECTS and related syndromes, and have stressed the associated neuropsychological and cognitive deficits. These include attention (ADHD), visual-motor coordination, dyspraxia, language and learning disabilities, mental impairment, and behavioral disorders, usually concomitant with activation of interictal EEG discharges. BCECTS occurs as 15% of childhood epilepsies, the most frequent epilepsy in school age children. The genetic predisposition is frequent with male predominance. Seizures and EEG discharges resolve before the age of puberty, but cognitive and behavioral deficits often persist.