Background: Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological, and hematological features. Objectives: The aim of this guideline was to prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein [paraproteinemic demyelinating neuropathy (PDN)]. Methods: Disease experts and a representative of patients considered references retrieved from MEDLINE and the Cochrane Library and prepared statements that were agreed in an iterative fashion. Recommendations: In the absence of adequate data, evidence-based recommendations were not possible, but the Task Force agreed on 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 M (IgM), 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 immunotherapies. 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; and (6) for POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with hemato-oncology advice.

European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society / R.D. Hadden, E. Nobile-Orazio, C. Sommer, A.F. Hahn, I. Illa, E. Morra, J.D. Pollard, R.A.C. Hughes, P. Bouche, D.R. Cornblath, R. Evers, C.L. Koski, J.M. Léger, P. Van den Bergh, P.A. van Doorn, I. van Schaik. - In: JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM. - ISSN 1085-9489. - 11:1(2006), pp. 9-19. [10.1111/j.1085-9489.2006.00059.x]

European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society

E. Nobile-Orazio
Secondo
;
2006

Abstract

Background: Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological, and hematological features. Objectives: The aim of this guideline was to prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein [paraproteinemic demyelinating neuropathy (PDN)]. Methods: Disease experts and a representative of patients considered references retrieved from MEDLINE and the Cochrane Library and prepared statements that were agreed in an iterative fashion. Recommendations: In the absence of adequate data, evidence-based recommendations were not possible, but the Task Force agreed on 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 M (IgM), 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 immunotherapies. 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; and (6) for POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with hemato-oncology advice.
Definition; Diagnosis; Guidelines; Paraproteinemic demyelinating neuropathy; Treatment
Settore MED/26 - Neurologia
2006
Article (author)
File in questo prodotto:
Non ci sono file associati a questo prodotto.
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/207802
Citazioni
  • ???jsp.display-item.citation.pmc??? 4
  • Scopus 51
  • ???jsp.display-item.citation.isi??? 39
social impact