European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of paraproteinaemic demyelinating neuropathies: Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society

R. D M Hadden, E. Nobile-Orazio, C. Sommer, A. Hahn, I. Ilia, E. Morra, J. Pollard, R. A C Hughes, P. Bouche, D. Cornblath, E. Evers, C. L. Koski, J. M. Léger, P. Van Den Bergh, P. Van Doorn, I. N. Van Schaik

Research output: Contribution to journalArticle

Abstract

Background. Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological and haematological features. Objectives. To prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein (paraproteinaemic demyelinating neuropathy, PDN). Methods. Search of MEDLINE and the Cochrane library, review of evidence and consensus agreement of an expert panel. Recommendations. In the absence of adequate data, evidence based recommendations were not possible but the panel agreed 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 (Ig)M, 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 immune therapies. 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. (6) For POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with haemato-oncology advice.

Original languageEnglish
Pages (from-to)809-818
Number of pages10
JournalEuropean Journal of Neurology
Volume13
Issue number8
DOIs
Publication statusPublished - Aug 2006

    Fingerprint

Keywords

  • Demyelinating
  • Guidelines
  • MAG
  • Monoclonal gammopathy
  • Neuropathy
  • Paraprotein
  • POEMS
  • Treatment

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Hadden, R. D. M., Nobile-Orazio, E., Sommer, C., Hahn, A., Ilia, I., Morra, E., Pollard, J., Hughes, R. A. C., Bouche, P., Cornblath, D., Evers, E., Koski, C. L., Léger, J. M., Van Den Bergh, P., Van Doorn, P., & Van Schaik, I. N. (2006). European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of paraproteinaemic demyelinating neuropathies: Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. European Journal of Neurology, 13(8), 809-818. https://doi.org/10.1111/j.1468-1331.2006.01467.x