Pediatric autoimmune epileptic encephalopathies are predominantly characterized by the presence of autoantibodies to the surface of neuronal proteins, for example, N-methyl-d-aspartate (NMDA) receptor antibodies, but also include diseases with non-cell surface antibodies (eg, anti-Hu, glutamic-acid decarboxylase antibodies). In some cases with distinct clinical and para-clinical features, an autoimmune epileptic encephalopathy can be diagnosed without the presence of an antibody and will also respond favorably to immunotherapy. In this review, we summarize the common presentations of pediatric autoimmune epileptic encephalopathies, treatments, and outcomes, and report recent findings in the field of epilepsy, encephalopathy, and the immune system.
Keywords: NMDA receptor antibody encephalitis; autoantibodies; autoimmune epilepsy; encephalopathy; voltage-gated potassium channel complex.