Autoimmune Epilepsies

Autoimmune epilepsies describe clinical syndromes wherein the immune system is suspected to be involved in the pathogenesis of seizures or as a mechanism for neuronal injury following seizures. These diseases typically affect otherwise healthy children and are characterized by explosive onset of focal seizures, encephalopathy, cognitive deterioration, or other focal neurological deficits, or all of these. Traditional neurological diagnostics lack sensitivity and specificity in the diagnosis of autoimmune epilepsies, and results must be considered in the clinical context. Consideration of an autoimmune etiology early in the clinical course is important to ensure timely initiation of immunotherapy, as appropriate, as conventional antiepileptic drugs alone are typically unable to control seizures and other neurological symptoms. This article discusses the autoimmune epilepsies of autoimmune encephalitis (including anti-N-methyl-D-aspartate receptor encephalitis), Rasmussen's encephalitis, and febrile infection-related epilepsy syndrome. Further research is needed to better understand pathogenic mechanisms, optimal immunotherapy, and the effect of treatment on prognosis.