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Samuel F. Berkovic, MD, FRACP; John D. Scarlett, MB, BS, FRACP, FRCPA; Graeme R. Symington, MD, FRACP; Xenia Dennett, PhD; Roger K. Woodruff, MB, BS, FRACP

Arch Neurol. 1986;43(8):845-848.

Abstract
• The association of monoclonal paraproteinemia, neuropathy, and dermato-endocrine disturbances is well recognized in Japan, and it also occurs in white patients. Neuropathy in such patients is classically distal and sensorimotor, and the paraprotein almost always contains light chains. A 58-year-old white man presented with severe progressive proximal motor neuropathy, dermatoendocrine changes, and an IgGk paraprotein. Over a 21/2-year period, treatment with melphalan and prednisolone produced improvement in the neuropathy and resolution of dermato-endocrine features with a corresponding decline in the serum paraprotein concentration. Subsequent reappearance of the paraprotein, despite treatment, was associated with clinical relapse.

Author Affiliations
From the Departments of Neurology (Drs Berkovic and Symington) and Haematology (Drs Scarlett and Woodruff), Austin Hospital, Heidelberg, Victoria, Australia; and the Muscle Unit, Department of Pathology, Royal Children's Hospital, Parkville, Victoria, Australia (Dr Dennett). Dr Berkovic is now with the Montreal Neurological Institute.

Footnotes
Accepted for publication Jan 9, 1986.

Reprints not available.

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