POEMS syndrome with peripheral and central nervous system demyelination: case report.

BACKGROUND

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.

METHODS

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 <2%, with normal morphology. Brain magnetic resonance imaging revealed hyperintense lesions in the regions of periventricular and subcortical white matter and thalamus. Normal TSH, T4, and T3 levels and elevated levels of antitiroglobulin antibody and antithyroid peroxydase antibody were found in the laboratory tests. Our patient was diagnosed with POEMS syndrome and treated with intermittent courses of melphalan and prednisone.

CONCLUSIONS

POEMS syndrome should also be considered in differential diagnosis of patients with demyelinating brain lesions, accompanied by peripheral neuropathy. Diagnostic evaluation of patients with demyelinating polyneuropathy should include brain magnetic resonance imaging.