The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease

Abstract

Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome is a rare paraneoplastic disorder. The polyneuropathy can be the presenting symptom and is typically a painful, motor-predominant polyradiculoneuropathy often mimicking chronic inflammatory demyelinating polyradiculoneuropathy. The presence of a lambda monoclonal protein, elevated vascular endothelial growth factor, systemic features, and treatment resistance are clues to the diagnosis. Castleman disease (CD) is seen in a subset of these patients, and when present the neuropathy is similar but less severe. In contrast, in those patients with purely CD, the neuropathy is often a mild, painless distal sensory neuropathy.

Keywords: Castleman disease; Castleman neuropathy; Monoclonal gammopathy; POEMS neuropathy; POEMS syndrome.