2018•touchREVIEWS in NeurologyOpen access

The Challenge of Distinguishing POEMS from Chronic Inflammatory Demyelinating Polyneuropathy—Importance of Early Recognition and Diagnosis of POEMS

Frances Elaine Chow, Leila Darki, Said R. Beydoun

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Abstract

POEMS is a rare syndrome characterized by the unique constellation of polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes. Correct diagnosis is often delayed in early stages of the syndrome when patients exhibit only isolated polyneuropathy due to the clinical and electrodiagnostic similarities with chronic inflammatory demyelinating polyneuropathy. We describe a case in which early suspicion for POEMS uncovered underlying malignancy, and we review the clinical, electrophysiological, pathological, and laboratory findings characteristic of POEMS. The importance of high clinical suspicion is key in the proper diagnosis and management of this complex syndrome.

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What this paper is about

POEMS is a rare syndrome characterized by the unique constellation of polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes. Correct diagnosis is often delayed in early stages of the syndrome when patients exhibit only isolated polyneuropathy due to the clinical and electrodiagnostic similarities with chronic inflammatory demyelinating polyneuropathy. We describe a case in which early suspicion for POEMS uncovered underlying malignancy, and we review the clinical, electrophysiological, pathological, and laboratory findings characteristic of POEMS. The importance of high clinical suspicion is key in the proper diagnosis and management of this complex syndrome.

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Available abstract

POEMS is a rare syndrome characterized by the unique constellation of polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes. Correct diagnosis is often delayed in early stages of the syndrome when patients exhibit only isolated polyneuropathy due to the clinical and electrodiagnostic similarities with chronic inflammatory demyelinating polyneuropathy. We describe a case in which early suspicion for POEMS uncovered underlying malignancy, and we review the clinical, electrophysiological, pathological, and laboratory findings characteristic of POEMS. The importance of high clinical suspicion is key in the proper diagnosis and management of this complex syndrome.

Key concepts: POEMS syndrome, Organomegaly, Polyneuropathy, Chronic inflammatory demyelinating polyneuropathy, Medicine, Pathological, Malignancy, Polyradiculoneuropathy

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The Challenge of Distinguishing POEMS from Chronic Inflammatory Demyelinating Polyneuropathy—Importance of Early Recognition and Diagnosis of POEMS — Research Paper | ScholarLens