TY - JOUR
T1 - European Federation of Neurological Societies/Peripheral Nerve Society Guideline* onmanagement of paraproteinemic demyelinating neuropathies. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society
AU - Hadden, Robert D.
AU - Nobile-Orazio, Eduardo
AU - Sommer, Claudia
AU - Hahn, Angelika
AU - Illa, Isabel
AU - Morra, Enrica
AU - Pollard, John D.
AU - Hughes, Richard A.C.
AU - Bouche, Pierre
AU - Cornblath, David R.
AU - Evers, Eileen
AU - Koski, Carol L.
AU - Léger, Jean Marc
AU - Van Den Bergh, Peter
AU - Van Doorn, Pieter A.
AU - Van Schaik, Ivo N.
PY - 2006/3
Y1 - 2006/3
N2 - 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.
AB - 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.
KW - Definition
KW - Diagnosis
KW - Guidelines
KW - Paraproteinemic demyelinating neuropathy
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=33644787369&partnerID=8YFLogxK
U2 - https://doi.org/10.1111/j.1085-9489.2006.00059.x
DO - https://doi.org/10.1111/j.1085-9489.2006.00059.x
M3 - Review article
C2 - 16519778
SN - 1085-9489
VL - 11
SP - 9
EP - 19
JO - Journal of the peripheral nervous system
JF - Journal of the peripheral nervous system
IS - 1
ER -