Autoimmune channelopathies: new antibody-mediated disorders of the central nervous system

Abstract

Contrary to established wisdom, there now appear to be antibody-mediated central nervous system (CNS) disorders. Over the last few years, a number of patients have been defined with antibodies to voltage-gated (VGKC) or ligand-gated (NMDAR, GlyR) ion channels or ungated water (AQP4) channels. Some of the disorders improve spontaneously over time, others may be more chronic and relapsing-remitting, but immunotherapies reduce antibody levels and improve clinical outcomes. These are exciting developments that herald a new era of immunotherapy-responsive CNS diseases, and they raise interesting questions regarding the aetiological and pathogenic mechanisms mediating these conditions.