European Federation of Neurological Societies/Peripheral Nerve Society Guideline* on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society

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

Research output: Contribution to journalReview article

Abstract

Background: Several diagnostic criteria for multifocal motor neuropathy (MMN) have been proposed in recent years, and a beneficial effect of intravenous immunoglobulin (IVIg) and various other immunomodulatory drugs has been suggested in several trials and uncontrolled studies. Objectives: The aim of this guideline was to prepare consensus guidelines on the definition, investigation, and treatment of MMN. Methods: Disease experts and a representative of patients considered references retrieved from MEDLINE and the Cochrane Library in July 2004 and prepared statements that were agreed in an iterative fashion. Recommendations: The Task Force agreed on good practice points to define clinical and electrophysiological diagnostic criteria for MMN and investigations to be considered. The principal recommendations and good practice points were as follows: (1) IVIg (2 g/kg given over 2-5 days) should be considered as the first line of treatment (level A recommendation) when disability is sufficiently severe to warrant treatment; (2) corticosteroids are not recommended (good practice point); (3) if initial treatment with IVIg is effective, repeated IVIg treatment should be considered (level C recommendation). The frequency of IVIg maintenance therapy should be guided by the individual response (good practice point). Typical treatment regimens are 1 g/kg every 2-4 weeks or 2 g/kg every 4-8 weeks (good practice point); (4) if IVIg is not (or not sufficiently) effective, then immunosuppressive treatment may be considered. Cyclophosphamide, cyclosporine, azathioprine, interferon-β1a, or rituximab are possible agents (good practice point); and (5) toxicity makes cyclophosphamide a less desirable option (good practice point).

Original languageEnglish (US)
Pages (from-to)1-8
Number of pages8
JournalJournal of the Peripheral Nervous System
Volume11
Issue number1
DOIs
StatePublished - Mar 1 2006
Externally publishedYes

Keywords

  • Definition
  • Diagnosis
  • Guidelines
  • Multifocal motor neuropathy
  • Treatment

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology

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