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April 1977

Amyloidosis With Plasma Cell Dyscrasia: An Overlooked Cause of Adult Onset Sensorimotor Neuropathy

Author Affiliations

From the Department of Neurology (Dr Trotter), Washington University Medical School, St Louis, the Medical Neurology Branch (Dr Engel), National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD, and the Rackham Arthritis Research Unit (Dr Ignaczak), University of Michigan Medical Center, Ann Arbor.

Arch Neurol. 1977;34(4):209-214. doi:10.1001/archneur.1977.00500160023003

• In ten previously undiagnosed patients, we have found erstwhile-"primary" nonhereditary amyloidosis as an overlooked cause of a predominately sensory, painful, and hyperesthetic distal neuropathy occurring in middle-aged and older patients. These symptoms, associated with orthostatic hypotension, diarrhea or constipation, cardiac abnormality, and male impotence are virtually diagnostic (in the absence of diabetes mellitus). Tissue diagnosis is quickly made by crystal-violet metachromasia of amyloid in fresh-frozen sections of a muscle biopsy specimen. Immunoglobulin and bone marrow evidence of plasma cell dyscrasia in eight of the ten patients suggests that the neuropathy in this form of amyloidosis is actually secondary to a plasma-cell-originating dysproteinemia. Therapy with melphalan and prednisone was not of benefit.

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