• Gac Med Mex · Jan 2021

    Observational Study

    Clinical and neurophysiological description of patients with POEMS syndrome.

    • Luis Lee-Chen, Ricardo Williams-de-Roux, Erwin Chiquete, José Jesús Aceves-Buendía, Eduardo Ruiz-Ruiz, Jennefer Portillo-Valle, Tatiana Bliskunova, Elizabeth Rodríguez-Perea, Liz Toapanta-Yanchapaxi, Guillermo García-Ramos, Carlos Cantú-Brito, and Bruno Estañol.
    • Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
    • Gac Med Mex. 2021 Jan 1; 157 (5): 466-472.

    IntroductionPOEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a monoclonal gammopathy with polyneuropathy as a mandatory criterion.ObjectiveTo describe potential associations between clinical expression and electrodiagnostic patterns in POEMS syndrome.MethodObservational, retrospective, cross-sectional study of cases cared for in a referral center, diagnosed with POEMS syndrome from 2009 to 2019.ResultsEleven patients (8 men) were analyzed. Median age at diagnosis was 40 years (range: 31-51; mean: 37.19 ± 15.67 years). Mean latency at diagnosis was 9.7 ± 8.37 months. In all subjects, initial clinical manifestation was polyneuropathy. Most patients had an axonal pattern (n = 5), followed by demyelinating (n = 4) and mixed patterns (n = 2). Monoclonal gammopathy was observed in all (6 l and 5 k cases; immunoglobulin [Ig] G: 72 %; IgA: 18 %; IgM: 9 %). Medical Research Council sum score was lower in the axonal pattern (median: 37.00 vs. 45.5; p = 0.024). There were no differences in systemic involvement between electrophysiological patterns.ConclusionElectrophysiological patterns are unlikely to have a clear extra-neurological clinical correspondence; however, this will have to be definitively proven with a larger sample size.Copyright: © 2021 Permanyer.

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