Electro-Clinical Profile of POEMS Cases at Montpellier University Hospital

Abstract

Introduction : POEMS syndrome is a rare, multisystemic B lymphoid hematological disease, classified among paraneoplastic disorders. Objective : The main objective of our study was to investigate the electro-clinical aspects of POEMS cases. Methodology : This descriptive study evaluated five POEMS patients seen from 2014 to 2024 at the Gui de Chauliac functional explorations unit of Montpellier University Hospital. For diagnosis, the presence of two mandatory major criteria including polyneuropathie and monoclonal gammapathy, plus one of the other major criterion (elevated VEGF) were required. Results : The average age at diagnosis was 54.6 years, with a predominance of male patients (80%). Most patients had a follow-up duration between 8 and 10 years. The most frequently observed clinical signs included distal-predominant paresthesias of the lower limbs, sock-like hypoesthesia, and bilateral foot drop. The mean disability score (ONLS) was 4. The mean interval before performing the electroneuromyogram was 18 months. Elevated VEGF levels could reach up to 7000 pg/ml. All patients exhibited demyelinating neuropathy, which was generally intermediate and homogeneous in the upper limbs, with early, length-dependent axonal loss (4/5). Conclusion : POEMS manifests as an aggressive neuropathy in a young adult. Early axonal loss is found in the lower limbs and secondary demyelination in the upper limbs without conduction block.