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Master Thesis

Le syndrome de POEMS : caractérisation de la neuropathie et devenir post-traitement chez 36 patients

Abstract : Introduction: Polyneuropathy in POEMS syndrome (Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin change) has been rarely described in detail. Here, we describe the characteristics of neuropathy in a large series of patients, and try to identify prognostic factors. Methods: In this monocentric retrospective study, we analyzed the clinical and neurophysiological characteristics (at diagnosis, 6 months and 1 year follow-up) of patients with POEMS syndrome diagnosed in our University Hospital between 1980 and 2017. The primary end-point was the Modified Rankin Scale (mRS) score. Results: 36 patients (mean age at diagnosis: 54 years; 58% male) with POEMS syndrome were analyzed. Hematologic diagnosis was monoclonal gammapathy of clinical significance (MGCS) in 33,33% of cases, solitary plasmocytoma (36,11%), myeloma (11,11%), and multiple plasmocytoma (19,44%). Diagnosis was delayed up to 12 months after symptom onset in 25% of cases. The mean mRS at diagnosis was 2,67 +/- 1,10. The neuropathy was clinically and electrically sensorimotor, length-dependent and severe (56% of patients had mRS ≥ 3). Nerve conduction studies showed a mixed axonal and demyelinating pattern (46% of cases), a pure axonal pattern (26%), or pure demyelinating features (29%). 42% (n=15) of patients were treated with autologous stem-cell transplantation (ASCT) and 33% had radiotherapy. At 1-year follow-up, 47% of patients had improved, 34% were stabilized, and 19% worsened. Age over 65 years and weight loss were of poor prognosis (p=0,027 and p=0,040). Patients treated with ASCT improved in 82% of cases whereas patients treated with radiotherapy improved in45% of cases. ASCT was associated with good prognosis (OR: 15, IC95 :2,42-93,01, p=0,004). Conclusion: POEMS syndrome polyneuropathy is severe, rapidly progressing, length-dependent, with both axonal and demyelinating electrophysiological features in a majority of cases. Age over 65, weight loss are of poor prognosis whereas ASCT is associated with a good outcome.
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Submitted on : Tuesday, September 29, 2020 - 6:52:18 PM
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Nathalie Deschamps. Le syndrome de POEMS : caractérisation de la neuropathie et devenir post-traitement chez 36 patients. Neurosciences [q-bio.NC]. 2019. ⟨dumas-02953071⟩

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