Can we improve the outcome of epileptic encephalopathies?

Abstract

The Canadian pediatric epilepsy network defines an epileptic encephalopathy (EE) as: “a brain dysfunction manifested by a progressive decline in cognitive and behavioral functions that is associated with frequent seizures and/or interictal epileptiform discharges in a child with medically refractory epilepsy.” Outcomes of these EEs are usually poor owing to the presence of continuous epileptic discharges and/or very frequent seizures, leading to excitotoxic cell death. In addition, early diagnosis is often delayed because of the subtle initial manifestations, and an inadequate choice of treatment not only worsens the seizures acutely but possibly alters long-term prognosis. Steroids, a ketogenic diet and vagal nerve stimulation are possible alternatives to improve the outcome of certain EEs, but I believe developing new therapeutic agents that target the mechanisms implicated in animal models should be favored. Possible targets include γ-aminobutyric acid B receptors, calcium channels or intracellular calcium stores.