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Gaceta médica de México

On-line version ISSN 2696-1288Print version ISSN 0016-3813

Abstract

LEE-CHEN, Luis et al. Clinical and neurophysiological description of patients with POEMS syndrome. Gac. Méd. Méx [online]. 2021, vol.157, n.5, pp.484-490.  Epub Dec 13, 2021. ISSN 2696-1288.  https://doi.org/10.24875/gmm.21000029.

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, and cross-sectional study of cases cared in a referral center diagnosed with POEMS syndrome from 2009-2019.

Results:

Eleven cases (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 (n = 2). Monoclonal gammopathy was observed in all patients (6 λ, and 5 κ 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 the systemic involvement between electrophysiological patterns.

Conclusion:

Electrophysiological patterns are unlikely to have a clear extra-neurological clinical correspondence; however this will need to be definitively proven with a larger sample size.

Keywords : Axonal; Desmyelinating; POEMS; Polineuropathy; Syndrome.

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