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

• DOI: 10.1111/jns.12682
• Publication Date: 2025-01-13T06:59:09Z
• Authors: Grace Swart; Michael P. Skolka; Shahar Shelly; Richard A. Lewis; Jeffrey A. Allen; Divyanshu Dubey; Zhiyv Niu; Judith Spies; Ruple S. Laughlin; Smathorn Thakolwi...; Ashley R. Santilli; Hebatallah Rashed; Igal Mirman; Alexander Swart; Sarah E. Berini; Kamal Shouman; Marcus V. Pinto; Michelle L. Mauer...; John R. Mills; P. James B. Dyck; William S. Harmsen; Jay Mandrekar; Christopher J. Klein

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is difficult to distinguish from mimicking disorders, with misdiagnosis resulting in IVIG overutilization. We evaluate a clinical-electrophysiological model facilitate CIDP versus mimic neuropathy prediction. Using the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 guidelines we derived 26 clinical and 144 nerve conduction variables. The was generated validated utilizing total (n = 129) mimics 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; (10) idiopathic-inflammatory-myopathies. analyzed 9282 51 408 electrophysiological data points. Univariate analysis identified 11 variables significant odds ratios. A multivariate regression using four two electrophysiologic achieved 93% area-under-curve (95% CI 91-95): progression over 8 weeks (OR 40.66, 95% 5.31-311.36), absent autonomic involvement 17.82, 2.93-108.24), muscle atrophy 16.65, 3.27-84.73), proximal weakness 3.63, 1.58-8.33), ulnar velocity slowing < 35.7 m/s 5.21, 2.13-12.76), block 13.37, 2.47-72.40). web-based probability calculator (https://news.mayocliniclabs.com/cidp-calculator/) developed, 100% sensitivity 68% specificity at 92% threshold. Specificity improved when considering "red flags," criteria, laboratory testing. assists differentiation mimics, scores below unlikely have CIDP. highest by demyelination,