title: European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of paraproteinaemic demyelinating neuropathies: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society.
abstract: BACKGROUND
  Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological and haematological features. Objectives. To prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein (paraproteinaemic demyelinating neuropathy, PDN).
  
  
  METHODS
  Search of MEDLINE and the Cochrane library, review of evidence and consensus agreement of an expert panel.
  
  
  CONCLUSIONS
  In the absence of adequate data, evidence based recommendations were not possible but the panel agreed the following good practice points: (1) Patients with PDN should be investigated for a malignant plasma cell dyscrasia. (2) The paraprotein is more likely to be causing the neuropathy if the paraprotein is immunoglobulin (Ig)M, antibodies are present in serum or on biopsy, or the clinical phenotype is chronic distal sensory neuropathy. (3) Patients with IgM PDN usually have predominantly distal and sensory impairment, with prolonged distal motor latencies, and often anti-myelin associated glycoprotein antibodies. (4) IgM PDN sometimes responds to immune therapies. Their potential benefit should be balanced against their possible side-effects and the usually slow disease progression. (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy, clinically, electrophysiologically, and in response to treatment. (6) For POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with haemato-oncology advice.
authors.affiliation: Department of Neurology, King's College Hospital, London, UK. rob.hadden@doctors.org.uk
authors.forename: R D M
authors.initials: RD
authors.lastname: Hadden
authors.forename: E
authors.initials: E
authors.lastname: Nobile-Orazio
authors.forename: C
authors.initials: C
authors.lastname: Sommer
authors.forename: A
authors.initials: A
authors.lastname: Hahn
authors.forename: I
authors.initials: I
authors.lastname: Illa
authors.forename: E
authors.initials: E
authors.lastname: Morra
authors.forename: J
authors.initials: J
authors.lastname: Pollard
authors.forename: R A C
authors.initials: RA
authors.lastname: Hughes
authors.forename: P
authors.initials: P
authors.lastname: Bouche
authors.forename: D
authors.initials: D
authors.lastname: Cornblath
authors.forename: E
authors.initials: E
authors.lastname: Evers
authors.forename: C L
authors.initials: CL
authors.lastname: Koski
authors.forename: J M
authors.initials: JM
authors.lastname: Léger
authors.forename: P
authors.initials: P
authors.lastname: Van den Bergh
authors.forename: P
authors.initials: P
authors.lastname: van Doorn
authors.forename: I N
authors.initials: IN
authors.lastname: van Schaik
mesh_terms: D026683:Advisory Committees; D003299:Cooperative Behavior; D003711:Demyelinating Diseases / Q000175:diagnosis / Q000628:therapy; D005060:Europe; D019317:Evidence-Based Medicine; D006801:Humans; D016239:MEDLINE / Q000706:statistics & numerical data; D009462:Neurology; D010265:Paraproteinemias / Q000175:diagnosis / Q000628:therapy; D010525:Peripheral Nerves; D017410:Practice Guidelines as Topic; D012955:Societies, Medical