Neuropathies

Peripheral neuropathies encompass a spectrum of conditions that are clinically characterized by a sensory or motor neuropathy with acute or chronic onset. In 8–10% of patients with peripheral neuropathy, the underlying cause is considered an autoimmune condition. This can be a systemic autoimmune disease, for example, vasculitis or a primary autoimmune condition. In this chapter, the underlying pathologenesis of the most common chronic autoimmune neuropathies will be reviewed. Chronic immune-mediated demyelinating polyradiculopathy (CIDP) is the most common form and research over the last decades has identified crucial steps in its pathogenesis. These include aberrant T cell responses as well as autoantibodies against glycolipids and (para)nodal proteins. Autoantibodies against myelin-associated glycoprotein (MAG) are involved in the pathogenesis of anti-MAG neuropathy. Another rarer condition is multifocal motor neuropathy in which clinical and experimental evidence suggests a crucial role of antibodies against gangliosides in its pathogenesis.