European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathies. Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society – first revision

Authors

  • Joint Task Force of the EFNS and the PNS

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    • Membership of Task Force: Robert D. M. Hadden, UK; Eduardo Nobile-Orazio, Italy; Claudia L. Sommer, Germany; Angelika F. Hahn, Canada; Isabel Illa, Spain; Enrica Morra, Italy; John D. Pollard, Australia; Michael P. T. Lunn, UK; Pierre Bouche, France; David R. Cornblath, USA; Eileen Evers, UK; Carol Lee Koski, USA; Jean-Marc Léger, France; Peter Van den Bergh, Belgium; Pieter A. van Doorn, The Netherlands; Ivo N. van Schaik, The Netherlands.


  • Anticipated date for updating this guideline: Not later than October 2013.

Dr. Robert Hadden, PhD, FRCP, Department of Neurology, King's College Hospital, Denmark Hill, London SE5 9RS, UK. Tel: +44-20-3299-8343; Fax: +44-20-3299-8358; E-mail: robert.hadden@nhs.net

Abstract

The aim of this guideline is to update the 2006 EFNS/PNS guideline on management of patients with a demyelinating neuropathy and a paraprotein (paraproteinemic demyelinating neuropathy [PDN]) by review of evidence and expert consensus. In the absence of adequate evidence, the panel agreed on good practice points: (1) patients with PDN should be investigated for a malignant plasma cell dyscrasia; (2) a monoclonal gammopathy of undetermined significance is more likely to be causing the neuropathy if it is immunoglobulin (Ig)M, anti-neural antibodies are present, and the clinical phenotype is chronic distal sensory neuropathy; (3) patients with IgM PDN usually have predominantly distal sensory impairment, prolonged distal motor latencies, and often anti-myelin-associated glycoprotein antibodies; (4) IgM PDN may respond to immunomodulatory therapies. Their potential benefit should be balanced against possible side effects and the usually slow disease progression; (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy; and (6) Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes syndrome is a multi-system malignant PDN.

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