2010 EFNS/PNS副蛋白血症性脱髓鞘神经病指南（第一次修订）

The aim of this guideline is to update the 2006 EFNS/PNS guidelineon management of patients with a demyelinating neuropathy and a paraprotein(paraproteinemic demyelinating neuropathy [PDN]) by review of evidence and expertconsensus. In the absence of adequate evidence, the panel agreed on good practicepoints:(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 causingthe neuropathy if it is immunoglobulin (Ig)M, anti-neural antibodies are present, andthe clinical phenotype is chronic distal sensory neuropathy;(3) patients with IgM PDNusually have predominantly distal sensory impairment, prolonged distal motor latencies,and often anti-myelin-associated glycoprotein antibodies;(4) IgM PDN may respond toimmunomodulatory therapies. Their potential benefit should be balanced against possibleside effects and the usually slow disease progression;(5) IgG and IgA PDN may beindistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy; and(6) Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skinchanges syndrome is a multi-system malignant PDN.