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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.

Original publication

DOI

10.1177/0883073816685505

Type

Journal article

Journal

J Child Neurol

Publication Date

03/2017

Volume

32

Pages

418 - 428

Keywords

NMDA receptor antibody encephalitis, autoantibodies, autoimmune epilepsy, encephalopathy, voltage-gated potassium channel complex