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, 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.