Benign pediatric localization- related epilepsies Part II. Syndromes in childhood

By definition, benign epilepsy syndromes occur in patients with no significant prenatal, perinatal, or postnatal complications, normal psychomotor development and negative laboratory and neuroimaging work-up, respond well to therapy, and remit without sequeale. The benign localizationrelated epilepsy syndromes of childhood include benign childhood epilepsy with centrotemporal spikes, Panayiotopoulos syndrome and Gastaut-type idiopathic childhood epilepsy with occipital paroxysms. Some patients initially presumed to have these or, for that matter, other benign syndromes in other age groups, follow a less typical course and continue to experience seizures or to exhibit neuropsychological deficits. Thus the diagnosis of a “possible” or “probable” benign epilepsy syndrome may need to be applied to patients initially suspected of having such syndromes until follow-up shows that they clearly follow a benign course. In Part I (Chahine and Mikati 2006) of our two-part review article, we discussed benign localization-related syndromes encountered in infancy. In this second part, we review the epidemiology, clinical manifestations, neuropsychological features, EEG findings, work-up and diagnostic criteria, differential diagnosis, genetics, management and prognosis of the three childhood-onset syndromes. In addition, we discuss their occasional overlap with or progression into other syndromes.