MULTIFOCAL MOTOR NEUROPATHY - A DISORDER OF THE PERIPHERAL NERVOUS-SYSTEM

Multifocal motor neuropathy is a peripheral nervous system disease des cribed among chronic inflammatory demyelinating polyneuropathies. It i s characterized according to both clinical criteria, including chronic asymetric and multifocal deficit which starts and remains prominent i n the upper limbs, and electrophysiological criteria, including persis tant multifocal motor conduction blocks in motor nerves. High titers o f serum antiganglioside GM1 antibodies are discovered in nearly 40% of cares. Steroids and plasma exchange are not efficient. High doses of intravenous immunoglobulins (IVIg) improved symptoms in the majority o f open and controlled published studies. The quality of the response t o IVIg may worsen in some patients after a variable number of infusion s, leading to immunosuppressive treatments mainly with oral or intrave nous cyclophosphamide. Its etiology is unknown but the frequent presen ce of anti-GM1 antibody high serum titers, the pathological findings i n some rare morphological studies, and the response to IVIg favor the hypothesis of an autoimmune disorder.