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Gammopathy with proximal motor axonopathy simulating motor neuron disease

Departments of Neurology and Neuropathology, University of California School of Medicine, San Francisco, CA.

We report a 38-year-old man with a pure motor syndrome and IgM gammopathy leading to flaccid quadriplegia. Improvement followed treatment with dexamethasone, cyclophosphamide, and plasmapheresis, but he died of pulmonary embolism. At autopsy, he had a proximal motor axonopathy with lymphocytic infiltration of ventral roots. Proximal motor neuropathy may masquerade as motor neuron disease. The association with gammopathy and response to treatment suggest that patients with motor neuron disease should be routinely screened for serum protein abnormalities.

Address correspondence and reprint requests to Dr. Parry, UCSF, Department of Neurology, M-794, San Francisco, CA 94143.

Dr. Ben-Zeev is co-director of the Apheresis and Oncology Unit, John Muir Memorial Hospital, Walnut Creek, CA.

Accepted for publication June 19, 1985.

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