Diagnostic and therapeutic scheme of autoimmune mediated encephalitis/encephalopathy

Abstract

Secondary encephalitis (encephalopathy) causally related with immune response induced by infection, etc., includes encephalitis mediated by innate immunity and adaptive immunity. In the latter, encephalitis mediated by antibodies to molecules at cell-surface of neuron seems to have relatively better outcome. In patients with encephalitis mediated by antibodies to NMDA-type glutamate receptors (NR), mean onset age is 26.5 years old, and the major initial symptoms are limbic symptoms including abnormal behavior, etc. The antibodies are causally related with internalization of NRs, resulting in antagonistic effect of NRs related with symptoms of encephalitis. In patients with encephalitis mediated by antibodies to voltage-gated potassium channel (VGKC), predominant affection of males are observed, and antibodies to leucine-rich glioma-inactivated 1 (LGI1) and contactinassociate protein (CASR) 2 are found as major epitopes. Patients with Hashimoto encephalitis mediated by antibodies to n-terminal of α-enolase (NAE) have broad spectra of clinical characteristics. Antibodies to TPO can be the marker for diagnosis, and thereafter, confirmation of antibodies to NAE is necessary for definitive diagnosis.