• Federica Cerri, Yuri Matteo Falzone, Nilo Riva, and Angelo Quattrini.
• Neuropathology Unit, Division of Neuroscience and Department of Neurology, Institute of Experimental Neurology, San Raffaele Scientific Institute, Via Olgettina 48, 20132, Milan, Italy.
• J. Neurol. 2019 Jan 1; 266 (1): 258-267.

AbstractPOEMS syndrome is a rare, chronic, disabling paraneoplastic disorder characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma cells disorder and skin changes. Diagnosis relies on the fulfillment of a set of clinical criteria of which polyneuropathy and a monoclonal plasma cell dyscrasia are early and essential features. Treatment may be either local or systemic and is aimed at the monoclonal plasma cell disorder. Our knowledge of the pathogenesis underlying the POEMS syndrome has advanced greatly over the past years, favoring an important progression in the recognition and management of this disorder. Here, we discuss the recent literature that has advanced our knowledge of the pathogenesis and clinical management of the polyneuropathy in POEMS syndrome.

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