Clinical and neurophysiological description of patients with POEMS syndrome.
Autor: | Lee-Chen L; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Williams-de-Roux R; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Chiquete E; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Aceves-Buendía JJ; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Ruiz-Ruiz E; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Portillo-Valle J; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Bliskunova T; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Rodríguez-Perea E; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Toapanta-Yanchapaxi L; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., García-Ramos G; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Cantú-Brito C; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico., Estañol B; Neurology and Psychiatry Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico. |
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Jazyk: | angličtina |
Zdroj: | Gaceta medica de Mexico [Gac Med Mex] 2021; Vol. 157 (5), pp. 466-472. |
DOI: | 10.24875/GMM.M21000600 |
Abstrakt: | Introduction: POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a monoclonal gammopathy with polyneuropathy as a mandatory criterion. Objective: To describe potential associations between clinical expression and electrodiagnostic patterns in POEMS syndrome. Method: Observational, retrospective, cross-sectional study of cases cared for in a referral center, diagnosed with POEMS syndrome from 2009 to 2019. Results: Eleven 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. Conclusion: Electrophysiological 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.) |
Databáze: | MEDLINE |
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