A Case of POEMS Syndrome Presented as CIDP

J Korean Assoc EMG Electrodiagn Med 1999;1(2):212-217.
Published online May 9, 1999.

Abstract

POEMS syndrome is a rare multisystem disorder manifests as polyneuropathy, organomegaly, endocrinopathy, M-proteins and skin changes. We report here the case of 54-year-old woman with POEMS syndrome that was followed by a 1-year history of symptoms that mimicked a chronic inflammatory demyelinating polyradiculoneuropathy(CIDP). The laboratory examination revealed multiple osteosclerotic bone lesions and IgG λparaproteinemia later in the course of the disease. Initial treatment with IVIg showed marked improvement of generalized weakness from bedridden state to walking with minimal assistance. In spite of the substantial improvement of peripheral neuropathy with steroid treatment the patient died suddenly at home 5 months after discharge from hospital.

Key Words: POEMS syndrome, CIDP, Steroid