Antibody testing in peripheral nerve disorders

The identification of autoantibodies associated with dysimmune neuropathies was a major contribution to the characterization of peripheral nerve disorders, the understanding of their pathophysiology, and the clinical diagnosis of neuropathies. Antibodies directed to GM1, GQ1b, and disyalilated gangliosides, and anti-MAG antibodies are very useful in the diagnosis of acute or chronic motor or sensory-motor neuropathies with or without monoclonal IgM. Anti-onconeural anti-Hu and anti-CV2/CRMP antibodies allow when they are detected the diagnosis of paraneoplastic neuropathies. This chapter focuses on the description of these antibodies as diagnostic markers and on their immunopathogenesis. We give a background overview on the origin of these antibodies, their detection, and review those studies, which clearly show that these antibodies are capable of binding to the target tissues in peripheral nerve and thereby can exert a variety of pathophysiological effects. The corresponding electrophysiological and histological changes observed both in human and animal models are exemplified in order to get a better understanding of the immune mechanisms of these antibody-mediated neuropathies.