Proximal Motor Neuropathy, Dermato-Endocrine Syndrome, and IgGk Paraproteinemia

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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 λ 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.