Antiganglioside antibodies do not necessarily play a role in multifocal motor neuropathy.
Multifocal motor neuropathy (MMN) is a disorder with a highly characteristic clinical picture and one which is defined by a specific electrodiagnostic abnormality, namely, multifocal conduction block which is confined to motor axons. Sensory axons which traverse segments of severe or even complete motor conduction block conduct normally. A proportion of patients with MMN also have elevated levels of antibodies to GM1 ganglioside. However, about one half of MMN patients lack elevated levels of these antibodies and many others have only modest elevations, to a degree often seen in other neurological and even non-neurological disorders. Furthermore, clinical and electrophysiological improvement of MMN in response to treatment with high dose intravenous immunoglobulin is achieved in the absence of any change in antiglycolipid levels. Injection of serum from patients with MMN and elevated GM1 antibody levels produces demyelination in recipient rat nerves, suggesting a pathogenetic role for these antibodies in demyelination. However, sera of patients with identical antibody titers in other motor system diseases produced no demyelination, suggesting that the demyelinating factor resides in some other serum fraction. At present, there is insufficient evidence to support the contention that these antibodies play a critical pathogenetic role in MMN. Until more evidence is available it is important to define MMN on the basis of a characteristic clinical picture and a unique electrodiagnostic abnormality rather than on a pattern of serum antibodies.
Parry
G J
GJ
Department of Neurology, University of Minnesota, Minneapolis.
D000818:Animals; D000906:Antibodies / Q000032:analysis*; D001327:Autoimmune Diseases / Q000276:immunology; D005677:G(M1) Ganglioside / Q000276:immunology*; D006017:Glycolipids / Q000276:immunology; D006801:Humans; D016472:Motor Neuron Disease / Q000276:immunology; D009422:Nervous System Diseases / Q000276:immunology*; D051381:Rats
D000906:Antibodies; D006017:Glycolipids; D005677:G(M1) Ganglioside