European Federation of Neurological Societies/Peripheral Nerve Society Guideline* onmanagement of paraproteinemic demyelinating neuropathies. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society

Robert D. Hadden, Eduardo Nobile-Orazio, Claudia Sommer, Angelika Hahn, Isabel Illa, Enrica Morra, John D. Pollard, Richard A.C. Hughes, Pierre Bouche, David R. Cornblath, Eileen Evers, Carol L. Koski, Jean Marc Léger, Peter Van Den Bergh, Pieter A. Van Doorn, Ivo N. Van Schaik

Research output: Contribution to journalReview articlepeer-review

Abstract

Background: Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological, and hematological features. Objectives: The aim of this guideline was to prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein [paraproteinemic demyelinating neuropathy (PDN)]. Methods: Disease experts and a representative of patients considered references retrieved from MEDLINE and the Cochrane Library and prepared statements that were agreed in an iterative fashion. Recommendations: In the absence of adequate data, evidence-based recommendations were not possible, but the Task Force agreed on the following good practice points: (1) patients with PDN should be investigated for a malignant plasma cell dyscrasia; (2) the paraprotein is more likely to be causing the neuropathy if the paraprotein is immunoglobulin M (IgM), antibodies are present in serum or on biopsy, or the clinical phenotype is chronic distal sensory neuropathy; (3) patients with IgM PDN usually have predominantly distal and sensory impairment, with prolonged distal motor latencies, and often anti-myelin-associated glycoprotein antibodies; (4) IgM PDN sometimes responds to immunotherapies. Their potential benefit should be balanced against their possible side effects and the usually slow disease progression; (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy clinically, electrophysiologically, and in response to treatment; and (6) for POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with hemato-oncology advice.

Original languageEnglish (US)
Pages (from-to)9-19
Number of pages11
JournalJournal of the Peripheral Nervous System
Volume11
Issue number1
DOIs
StatePublished - Mar 2006
Externally publishedYes

Keywords

  • Definition
  • Diagnosis
  • Guidelines
  • Paraproteinemic demyelinating neuropathy
  • Treatment

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology

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