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
