Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous but treatable immune-mediated neuropathy. Nerve conduction studies are considered essential for a definite diagnosis, but poor performance and misinterpretation of the results frequently leads to misdiagnosi...

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Bibliographic Details
Published in:Lancet neurology Vol. 18; no. 8; pp. 784 - 794
Main Authors: Bunschoten, Carina, Jacobs, Bart C, Van den Bergh, Peter Y K, Cornblath, David R, van Doorn, Pieter A
Format: Journal Article
Language:English
Published: England Elsevier Ltd 01.08.2019
Elsevier Limited
Subjects:
Ataxia
Cell adhesion & migration
Corticosteroids
Demyelination
Diagnosis
Genotype & phenotype
Guillain-Barre syndrome
Immunoglobulin G4
Immunoglobulins
Inflammation
Intravenous administration
Magnetic resonance imaging
Medical diagnosis
Motor neurone disease
Nerve conduction
Neuropathy
Phenotypes
Proteins
Ultrasound
ISSN:1474-4422, 1474-4465, 1474-4465
Online Access:Get full text
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Summary:Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous but treatable immune-mediated neuropathy. Nerve conduction studies are considered essential for a definite diagnosis, but poor performance and misinterpretation of the results frequently leads to misdiagnosis. Nerve ultrasound and MRI could be helpful in diagnosis. Whereas typical CIDP is relatively easy to diagnose, atypical variants with distinct phenotypes can be a diagnostic challenge. Intravenous or subcutaneous immunoglobulin, corticosteroids, and plasma exchange are effective treatments, but maintenance treatments are often required for years, and treatment regimens require careful and regular adjustments to avoid undertreatment or overtreatment. Patients who do not improve, or insufficiently improve after treatment, might have specific characteristics related to a distinct disease mechanism caused by immunoglobulin G4 antibodies to nodal or paranodal proteins, and could require alternative treatments. Future studies should focus on curative and individualised treatment regimens to improve the patient's condition and to prevent further nerve damage.
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ISSN:1474-4422
1474-4465
1474-4465
DOI:10.1016/S1474-4422(19)30144-9