Affiliations: Comprehensive Pediatric Epilepsy Center, Florida Hospital for Children, Orlando, FL, USA | Department of Neuropsychology, Florida Hospital for Children, Orlando, FL, USA
Note:  Corresponding author: Dr. Christine M. Salinas, Florida Hospital for Children, Comprehensive Pediatric Epilepsy Center, 615 E. Princeton St., Suite 540, Orlando, FL, USA. Tel.: +1 4073038127; Fax: +1 4073038197; E-mail: [email protected]
Abstract: Epileptic encephalopathies are severe pediatric syndromes in which aggressive epileptiform discharges and seizures are thought to lead to profound cognitive impairments due to delay or failure of developmental skills to emerge, arrest of early normal development resulting in a cognitive plateau, and regression or deterioration of previously acquired cognitive skills. Children with epileptic encephalopathy usually have a combination of early seizure onset, sometimes as early as in infancy with severe pathology, chronicity, and multiple seizure types, including but not limited to status events or frequent abnormal interictal patterns, requiring antiepileptic drugs polypharmacy, making these some of the most challenging epilepsy cases with poor cognitive and behavioral prognosis. We describe prognostic indicators for cognitive and behavioral comorbidity in childhood epilepsy. The main focus of the paper is to provide an overview of several epileptic encephalopathies by summarizing their clinical features, providing updates on recent advances in imaging and genetics, and discussing neurodevelopmental outcomes with respect to various treatment modalities, including epilepsy surgery.
Keywords: Epilepsy, children, encephalopathy, neuropsychology, cognitive, development