Making the lame walk?

Abstract

The acronym POEMS, coined by Bardwick et al. in 1980,1 summarizes the features of a rare peripheral nerve syndrome characterized by polyneuropathy, organomegaly, endocrinopathies, monoclonal gammopathy, and skin lesions. Although there is debate about precise limits of the syndrome, most now agree that the defining features of POEMS are polyneuropathy and a monoclonal plasma cell dyscrasia, and that two or more other features (e.g., sclerotic bone lesions, hyperpigmentation or hypertrichosis, endocrine dysfunction) are necessary to confidently distinguish POEMS from related conditions such as monoclonal protein-associated (MGUS) neuropathy.2 Although rare, POEMS has features that are of interest to all neurologists. POEMS is fundamentally a paraneoplastic syndrome, but its pathophysiology is distinctive. Unlike most paraneoplastic neurologic syndromes (e.g., Lambert-Eaton myasthenic syndrome, sensory neuronopathy), which are accidental consequences of a host’s immune response to a neoplasm, the manifestations of POEMS appear to be caused by overproduction of a cytokine, vascular endothelial growth factor (VEGF), by the neoplastic plasma cells themselves.3 Most patients with POEMS …