PEDIATRIC EPILEPSY | Cortical Dysplasia and Catastrophic Epilepsies of Childhood

Abstract

Catastrophic epilepsies of childhood (CEC) are a class of disorders distinguished not by a common etiology or seizure semiology, but by the early age of occurrence, the severity and frequency of the seizures themselves, and a frequent association with mental retardation. If seizures cannot be controlled, the ultimate impact of CEC on development is often devastating. Although a variety of etiologies contribute to CEC, malformations of cortical development, including cortical dysplasia, form the pathological substrate in a significant number of cases. For this reason, our laboratory has tried to better understand the relationship between cortical dysplasia and epilepsy in an animal model, in utero irradiation in rats. Although we have found a number of cortical abnormalities in this model, the most striking alterations involve impaired development of the cortical interneurons. We hypothesize that this impaired inhibition may be a critical link between abnormal cortical development and seizures in some types of human epilepsy.