Epileptic encephalopathy is a heterogeneous group of epilepsy syndromes associated with severe cognitive and behavioral disturbances characterized by spontaneous, recurrent seizures and neurodevelopmental impairment. Epileptic encephalopathy include eight syndromes: Ohtahara syndrome, Dravet syndrome, West syndrome, myoclonic status in nonprogressive encephalopathies, Landau-Kleffner syndrome, Lennox-Gastaut syndrome, and epilepsy with continuous spike waves during slow-wave sleep. These syndromes vary in their age of onset, seizure types, developmental outcome, etiologies, EEG patterns, neuropsychological deficits, and prognosis. EIEE32 is a subtype of epileptic encephalopathy caused due to mutations in the KCNA2 gene.
The disease prognosis is very poor, with most affected children either dying or being severely neurologically impaired.