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 localization-related 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.

Duke Scholars

Author Mohamad Abdul Mikati Pediatrics, Neurology