Clinical and neurophysiological description of patients with POEMS syndrome
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a monoclonal gammopathy with polyneuropathy as a mandatory criterion. To describe potential associations between clinical expression and electrodiagnostic patterns in POEMS syndrome. Observational, ret...
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Published in | Gaceta médica de México Vol. 157; no. 5; pp. 466 - 472 |
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Main Authors | , , , , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
Mexico
2021
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Subjects | |
Online Access | Get full text |
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Summary: | POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a monoclonal gammopathy with polyneuropathy as a mandatory criterion.
To describe potential associations between clinical expression and electrodiagnostic patterns in POEMS syndrome.
Observational, retrospective, cross-sectional study of cases cared for in a referral center, diagnosed with POEMS syndrome from 2009 to 2019.
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.
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. |
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Bibliography: | ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 ObjectType-Undefined-3 |
ISSN: | 0016-3813 |
DOI: | 10.24875/GMM.M21000600 |