Journal
JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM
Volume 15, Issue 3, Pages 185-195Publisher
WILEY
DOI: 10.1111/j.1529-8027.2010.00278.x
Keywords
demyelinating; guidelines; MAG; monoclonal gammopathy; neuropathy; paraprotein; POEMS; treatment
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Funding
- Grifols
- Biogen-Idec
- Baxter
- Laboratoire Francais du Biofractionnement (LFB)
- Octapharma
- Schering
- Bayer
- Sanquin blood supply foundation
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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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