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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

Research output: Contribution to journalArticle

Robert D. M. Hadden, Eduardo Nobile-Orazio, Claudia L. Sommer, Angelika F. Hahn, Isabel Illa, Enrica Morra, John D. Pollard, Michael P. T. Lunn, Pierre Bouche, David R. Cornblath, Eileen Evers, Carol Lee Koski, Jean-Marc Legar, Peter van den Bergh, Pieter A. van Doorn, Ivo N. van Schaik

Original languageEnglish
Pages (from-to)185 - 195
Number of pages11
Issue number3
PublishedSep 2010

King's Authors


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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