POEMS Syndrome With Peripheral and Central Nervous System Demyelination: Case Report

INTRODUCTION:POEMS syndrome is a rare, atypical plasma cell proliferative disorder. Predominantly motor chronic demyelinating polyneuropathy and monoclonal plasma cell disease are the major clinical features of POEMS syndrome. Demyelinating lesions of the central nervous system have been reported only in 2 patients with POEMS syndrome up to now. CASE REPORT:A 71-year-old man was investigated for numbness, burning sensation, and weakness of the lower extremities. He was found to have hypertrichosis, white nails, and multiple hemangiomas in the lower extremities. Neurological examination revealed decreased strength of the proximal and distal muscles of the lower extremities and absence of deep tendon reflexes. There was a marked impairment of temperature, position, and vibration senses in the lower extremities. Romberg test was positive. Nerve conduction was markedly reduced in all extremities and needle electromyography showed denervation pattern. Cerebrospinal fluid was clear, colorless, and had no oligoclonal band. IgM kappa monoclonal gammopathy was identified in serum immunofixation electrophoresis. In bone marrow aspirate plasma cells were counted as being