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Peripheral neuropathy with IgM kappa monoclonal immunoglobulin directed against myelin-associated glycoprotein

Department of Neurology and Neurosurgery (Drs. Melmed, Duncan, Finlayson, and Stewart) and the Department of Biochemistry (Drs. Frail and Braun), McCill University, and the Division of Immunology (Dr. Danoff), Montreal General Hospital, Montreal. Quebec, Canada.

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, immunosup-pressive agents, and plasmapheresis produced no significant improvement.

Address correspondence and reprint requests to Dr. Melmed, Department of Neurology and Neurosurgery, McGill University, 1650 Cedar Avenue, Montreal, Quebec H3G 1A4, Canada.

Dr. Finlayson is now deceased.

Accepted for publication March 11, 1983

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