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
. 2025 Mar;30(1):e12682.
doi: 10.1111/jns.12682.

Distinguishing Chronic Inflammatory Demyelinating Polyneuropathy From Mimic Disorders: The Role of Statistical Modeling

Grace Swart  1 , Michael P Skolka  1 , Shahar Shelly  1   2 , Richard A Lewis  3 , Jeffrey A Allen  4 , Divyanshu Dubey  1   5 , Zhiyv Niu  3 , Judith Spies  6 , Ruple S Laughlin  1 , Smathorn Thakolwiboon  1 , Ashley R Santilli  1 , Hebatallah Rashed  1 , Igal Mirman  7 , Alexander Swart  8 , Sarah E Berini  1 , Kamal Shouman  1 , Marcus V Pinto  1 , Michelle L Mauermann  1 , John R Mills  5 , P James B Dyck  1 , William S Harmsen  9 , Jay Mandrekar  9 , Christopher J Klein  1   5
Affiliations

Distinguishing Chronic Inflammatory Demyelinating Polyneuropathy From Mimic Disorders: The Role of Statistical Modeling

Grace Swart et al. J Peripher Nerv Syst. 2025 Mar.

Abstract

Background and aims: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is difficult to distinguish from mimicking disorders, with misdiagnosis resulting in IVIG overutilization. We evaluate a clinical-electrophysiological model to facilitate CIDP versus mimic neuropathy prediction.

Methods: Using the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 CIDP guidelines we derived 26 clinical and 144 nerve conduction variables. The model was generated and validated utilizing total CIDP (n = 129) and mimics (n = 309); including (1) IgG4-nodopathies; (2) POEMS (polyneuropathy-organomegaly-endocrinopathy-monoclonal protein-skin changes); (3) anti-myelin-associated-glycoprotein; (4) paraneoplastic; (5) Waldenström B-cell lymphoma; (6) diabetic neuropathies; (7) amyloidosis; (8) Charcot-Marie-Tooth; (9) motor neuropathies/neuronopathies; and (10) idiopathic-inflammatory-myopathies.

Results: We analyzed 9282 clinical and 51 408 electrophysiological data points. Univariate analysis identified 11 of 26 clinical variables with significant odds ratios. A multivariate regression model using four clinical and two electrophysiologic variables achieved 93% area-under-curve (95% CI 91-95): progression over 8 weeks (OR 40.66, 95% CI 5.31-311.36), absent autonomic involvement (OR 17.82, 95% CI 2.93-108.24), absent muscle atrophy (OR 16.65, 95% CI 3.27-84.73), proximal weakness (OR 3.63, 95% CI 1.58-8.33), ulnar motor conduction velocity slowing < 35.7 m/s (OR 5.21, 95% CI 2.13-12.76), and ulnar motor conduction block (OR 13.37, 95% CI 2.47-72.40). A web-based probability calculator (https://news.mayocliniclabs.com/cidp-calculator/) was developed, with 100% sensitivity and 68% specificity at a 92% probability threshold. Specificity improved to 93% when considering "red flags," electrophysiologic criteria, and laboratory testing.

Interpretation: A probability calculator using clinical electrophysiological variables assists CIDP differentiation from mimics, with scores below 92% unlikely to have CIDP. The highest specificity is achieved by considering clinical "red flags," electrophysiologic demyelination, and laboratory testing.

Keywords: CIDP; EAN/PNS; European academy of neurology/peripheral nerve society; chronic inflammatory demyelinating polyradiculoneuropathy; diagnostic criteria.

PubMed Disclaimer

References

    1. L. Querol, M. Crabtree, M. Herepath, et al., “Systematic Literature Review of Burden of Illness in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP),” Journal of Neurology 268, no. 10 (2021): 3706–3716, https://doi.org/10.1007/s00415‐020‐09998‐8.
    1. J. A. Allen, L. Butler, T. Levine, and A. Haudrich, “A Global Survey of Disease Burden in Patients Who Carry a Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy,” Advances in Therapy 38, no. 1 (2021): 316–328, https://doi.org/10.1007/s12325‐020‐01540‐6.
    1. H. C. Lehmann, D. Burke, and S. Kuwabara, “Chronic Inflammatory Demyelinating Polyneuropathy: Update on Diagnosis, Immunopathogenesis and Treatment,” Journal of Neurology, Neurosurgery, and Psychiatry 90, no. 9 (2019): 981–987, https://doi.org/10.1136/jnnp‐2019‐320314.
    1. P. J. B. Dyck and J. A. Tracy, “History, Diagnosis, and Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy,” Mayo Clinic Proceedings 93, no. 6 (2018): 777–793, https://doi.org/10.1016/j.mayocp.2018.03.026.
    1. T. M. Burns, “Chronic Inflammatory Demyelinating Polyradiculoneuropathy,” Archives of Neurology 61, no. 6 (2004): 973–975, https://doi.org/10.1001/archneur.61.6.973.

Grants and funding

LinkOut - more resources