Peripheral neuropathy with IgM kappa monoclonal immunoglobulin directed against myelin-associated glycoprotein.
Three patients with a monoclonal IgM kappa paraproteinemia had a slowly progressive segmental demyelinating peripheral neuropathy. Sural nerve biopsies showed predominantly large fiber loss, with widening of the intraperiod line of myelin associated with active demyelination. Immunoblot analysis showed reactivity of IgM to myelin-associated glycoprotein (MAG) of human myelin. An enzyme-linked immunosorbent assay (ELISA) was developed to provide a simple quantitative technique for detection of antibodies to MAG. Treatment with corticosteroids, immunosuppressive agents, and plasmapheresis produced no significant improvement.
D000368:Aged; D000911:Antibodies, Monoclonal / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay; D006023:Glycoproteins / Q000276:immunology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology*; D007075:Immunoglobulin M / Q000276:immunology*; D007145:Immunoglobulin kappa-Chains / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009185:Myelin Proteins / Q000276:immunology*; D010523:Peripheral Nervous System Diseases / Q000276:immunology* / Q000473:pathology
D000911:Antibodies, Monoclonal; D006023:Glycoproteins; D007147:Immunoglobulin Light Chains; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D009185:Myelin Proteins