Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2025 Jan 1;17(1):e76749.
doi: 10.7759/cureus.76749. eCollection 2025 Jan.

Chronic Inflammatory Demyelinating Polyneuropathy: A Narrative Review of a Systematic Diagnostic Approach to Avoid Misdiagnosis

Rodolfo M Roman-Guzman  1   2 , Adriana P Martinez-Mayorga  1   2 , Louis D Guzman-Martinez  1 , Ildefonso Rodriguez-Leyva  1   2
Affiliations
Review

Chronic Inflammatory Demyelinating Polyneuropathy: A Narrative Review of a Systematic Diagnostic Approach to Avoid Misdiagnosis

Rodolfo M Roman-Guzman et al. Cureus. .

Abstract

Chronic inflammatory demyelination polyradiculoneuropathy (CIDP) is a rare autoimmune neuropathy generated by cellular and humoral immune responses. Its course can be chronic, progressive, monophasic, or relapsing-remitting. Misdiagnosis and inappropriate therapy are common in CIDP. Given the scarcity of integrative information, we aimed to briefly summarize the epidemiology, pathophysiology, clinical phenotypes, diagnostic tools, and diagnostic criteria and provide a systematic diagnostic approach. We reviewed articles on Medline (PubMed) from 2018 to 2023, using Google Scholar to summarize the topics. The results are presented as a narrative review, in accordance with recommendations of the Scale for the Assessment of Narrative Review Articles (SANRA) guidelines. The included evidence showed that CIDP is a challenging neuropathy to diagnose and treat. Pathologic factors initiating typical CIDP and atypical CIDP are still clearly unknown. CIDP is diagnosed using the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria, which combine clinical features with electrophysiological evidence of demyelination. However, some patients need to fulfill the requirements. Another challenge is monitoring the disease progression and recognizing patients who do not respond to evidence-based first-line therapy to individualize their treatment. Based on the evidence, we conclude that 2021 EFNS/PNS guidelines allow for a more accurate diagnosis and treatment of CIDP and its variants. New diagnostic tools and molecular approaches are helpful in the diagnosis process but cannot replace clinical and electrodiagnostic criteria.

Keywords: atypical-cidp; chronic inflammatory demyelinating polyneuropathy (cidp); clinical phenotypes; electrophysiological criteria; inflammatory neuropathies; typical-cidp.

PubMed Disclaimer

Conflict of interest statement

Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Spectrum of CIDP based on the 2021 second revision of the European Academy of Neurology/Peripheral Nerve Society guidelines
CIDP, Chronic inflammatory demyelination polyradiculoneuropathy; DADS, Distal acquired demyelinating and symmetric neuropathy; MADSAM, Multifocal acquired demyelinating sensory and motor neuropathy; A-CIDP, acute-onset CIDP Figure credits: Rodolfo M. Roman-Guzman
Figure 2
Figure 2. Overview of clinical and electrodiagnostic criteria for typical CIDP and atypical CIDP variants
CIDP, Chronic inflammatory demyelination polyradiculoneuropathy; SC, Supportive criteria Figure credits: Rodolfo M. Roman-Guzman
See this image and copyright information in PMC

References

    1. Comprehensive approaches for diagnosis, monitoring and treatment of chronic inflammatory demyelinating polyneuropathy. Fisse AL, Motte J, Grüter T, Sgodzai M, Pitarokoili K, Gold R. https://doi.org/10.1186/s42466-020-00088-8 Neurol Res Pract. 2020;2 - PMC - PubMed
    1. Chronic inflammatory demyelinating polyneuropathy - a narrative review. (Article in Portuguese) Meireles ALF. https://doi.org/10.11606/issn.1679-9836.v100i1p57-61 Rev Med. 2021;100:57–61.
    1. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force - second revision. Van den Bergh PY, van Doorn PA, Hadden RD, et al. Eur J Neurol. 2021;28:3556–3583. - PubMed
    1. Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Bunschoten C, Jacobs BC, Van den Bergh PY, Cornblath DR, van Doorn PA. Lancet Neurol. 2019;18:784–794. - PubMed
    1. The misdiagnosis of CIDP: a review. Allen JA. https://doi.org/10.1007/s40120-020-00184-6. Neurol Ther. 2020;9:43–54. - PMC - PubMed

LinkOut - more resources