Clinical and pathophysiological implications of autoantibodies in autoimmune neuropathies

Abstract

Autoimmune neuropathies are a heterogeneous group of rare and disabling diseases in which the immune system targets peripheral nervous system antigens and that respond to immune therapies. This review focuses on Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, polyneuropathy associated with IgM monoclonal gammopathy, and autoimmune nodopathies. Autoantibodies targeting gangliosides, proteins in the node of Ranvier, and myelin-associated glycoprotein have been described in these disorders, defining subgroups of patients with similar clinical features and response to therapy. This topical review describes the role of these autoantibodies in the pathogenesis of autoimmune neuropathies and their clinical and therapeutic importance.

Keywords: Autoantibodies; Autoimmune neuropathies; CIDP; Caspr; Contactin; Guillain-Barré syndrome; IGM-MGUSP; Multifocal motor neuropathy; Neurofascin.