Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy

Carina Bunschoten, Bart C. Jacobs, Peter Y.K. Van den Bergh, David R. Cornblath, Pieter A. van Doorn

Research output: Contribution to journalReview articlepeer-review

Abstract

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.

Original languageEnglish (US)
Pages (from-to)784-794
Number of pages11
JournalThe Lancet Neurology
Volume18
Issue number8
DOIs
StatePublished - Aug 2019

ASJC Scopus subject areas

  • Clinical Neurology

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