Autoimmune encephalopathies and epilepsies in children and teenagers

Recognition of autoimmune encephalopathies and epilepsies in children and teenagers with acute or subacute onset of central nervous system dysfunction, through detection of the pertinent antibody on serum or cerebral spinal fluid, or through a response to immunotherapy may lead to an early diagnosis, and thus expedited implementation of immunotherapy and improved neurological outcome. The epidemiology of pediatric autoimmune encephalopathy and epilepsy is not well established, but advances in disease-specific biomarker discovery have lead to identification of disorders with either a cytotoxic T cell mediated pathogenesis or (more recently) possible autoantibody mediated disorders. This review summarizes the clinical presentations and recommended evaluations and treatment of pediatric epileptic encephalopathy suspected to be of autoimmune etiology.