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 journalArticle

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
Pages (from-to)9-19
Number of pages11
JournalJournal of the Peripheral Nervous System
Volume11
Issue number1
DOIs
Publication statusPublished - Mar 2006

Keywords

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

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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    Hadden, R. D., Nobile-Orazio, E., Sommer, C., Hahn, A., Illa, I., Morra, E., Pollard, J. D., Hughes, R. A. C., Bouche, P., Cornblath, D. R., Evers, E., Koski, C. L., Léger, J. M., Van Den Bergh, P., Van Doorn, P. A., & Van Schaik, I. N. (2006). 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. Journal of the Peripheral Nervous System, 11(1), 9-19. https://doi.org/10.1111/j.1085-9489.2006.00059.x