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Brain, Vol. 106, No. 1, 169-195, 1983
© 1983 Oxford University Press

research-article

CHRONIC DEMYELINATING NEUROPATHY ASSOCIATED WITH BENIGN IGM PARAPROTEINAEMIA

I. S. SMITH, S. N. KAHN1, B. W. LACEY, R. H. M. KING, R. A. EAMES, D. J. WHYBREW and P. K. THOMAS

Institute of Neurology and National Hospital for Nervous Diseases, Queen Square, the Westminster Hospital Medical School and the Royal Free Hospital School of Medicine London

Twelve cases of chronic sensorimotor neuropathy associated with benign IgM paraproteinaemia are described. The onset was in the sixth or seventh decades and 9 cases were male. Tremor and ataxia were common features. The ESR was raised in 6 cases and the CSF protein content elevated in 10. There was severe reduction of motor nerve conduction velocity. Sera from all cases contained monoclonal IgM antibodies strongly reactive with human peripheral myelin, made up by the paraprotein. Histological studies on nerve biopsies taken from 8 cases all demonstrated a demyelinating neuropathy. Eight biopsies were examined by immunofluorescence. Monoclonal IgM was present on the myelin sheaths of surviving myelinated nerve fibres. The findings suggested that the neuropathy was of autoimmune origin and caused directly by the antimyelin antibody.

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Received May 12, 1982. Revised September 23, 1982.

1Present address: William Pepper Laboratory, University of Pennsylvania Hospital, 3400 Spruce St., Philadelphia PA 19104, USA.


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