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Abstract Details

A Statistical Model to Facilitate Differentiation of Chronic Inflammatory Demyelinating Polyneuropathy Versus Mimics
Neuromuscular and Clinical Neurophysiology (EMG)
S16 - Updates on Nerve and Muscle Disorders (2:48 PM-3:00 PM)
010

CIDP misdiagnosis leads to inappropriate treatment with economic and patient burden. Consensus guidelines assist diagnosis but are underutilized based on complexity.

We sought to create a simple clinical-electrophysiological model facilitating CIDP versus mimic neuropathy prediction, accessible as a web-based probability calculator.

2021-EAN/PNS guidelines were used for CIDP diagnosis with 26 clinical and 144 nerve-conduction variables chosen based on these criteria and publications addressing mimics. 110 CIDP and 309 mimics (IgG4-nodopathies, paraneoplastic, POEMS, anti-MAG, diabetic radiculoplexus neuropathies (DRPN), MMN, inherited, eight others) underwent data extraction. Univariate and multivariate regression analysis identified the most informative variables, validated in a CIDP subset cohort.

We analysed 9,282 clinical and 51,408 electrophysiological data points. Univariate analysis identified 11/26 clinical variables with significant odds-ratios. A multivariate regression model revealed six clinical-electrophysiologic variables—progression over 8 weeks (OR 40.66, p=0.0004), absent autonomic involvement (OR 17.82, p=0.0018), absent muscle atrophy (OR 16.65, p=0.0007), proximal weakness (OR 3.63, p=0.0024), ulnar motor conduction velocity slowing <35.7 m/s (OR 5.21, p=0.0003), ulnar motor conduction block (OR 13.37, p=0.0026) —achieved 93%-area-under-curve (95%CI 91-95). EAN/PNS electrodiagnostic criteria did not enhance the model (92% area-under-curve, 95% CI 88.6-94.8). The calculator at 92% probability cut-off showed 100% sensitivity but 67% specificity. Specificity improved to 93% after considering clinical “red-flags”, 2021-EAN/PNS electrophysiologic demyelination, and laboratory testing where indicated for NF155/Contactin1-IgG4, paraneoplastic antibodies, VEGF-gammopathy, MAG-gammopathy and inherited neuropathy gene panel.

A CIDP calculator with limited clinical-nerve-conduction variables facilitates differentiation of CIDP versus mimics, with highest utility in ruling out CIDP mimics at scores under 92%. Specificity is enhanced by revisiting the clinical phenotype and considering individualized serum laboratory testing. Further validation studies of the model in external cohorts are warranted.
Authors/Disclosures
Grace Swart, MD
PRESENTER
Dr. Swart has received research support from National Health and Medical Research Council.
Michael P. Skolka, MD Dr. Skolka has nothing to disclose.
Shahar Shelly, MD (Rambam Medical Center) Dr. Shelly has stock in Remepy.
Richard A. Lewis, MD, FAAN (Cedars-Sinai Medical Center) Dr. Lewis has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Lewis has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CSL Behring. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Grifols. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Takeda. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Nuvig. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Dianthus. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Janssen. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Medscape. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Annexon. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Immunovant. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BioCryst. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NervoSave. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Seismic. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Annexon. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Boehringer Ingelheim. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alnylam. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Intellia. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Shernoff et al. Dr. Lewis has received publishing royalties from a publication relating to health care.
Jeffrey A. Allen, MD (University of Minnesota) Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for csl behring. Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Takeda. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Grifols. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alnylam. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astra Zeneca. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Johnson and Johnson. Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Annexon. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Immunovant. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CSL Behring. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Annexon. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Octapharma. Dr. Allen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Takeda. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Allen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for CSL behring. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Takeda. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Alnyla,m. Dr. Allen has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Alexion.
Judith M. Spies, MBBS, PhD (Royal Prince Alfred Hospital) Dr. Spies has nothing to disclose.
Divyanshu Dubey, MD, FAAN (Mayo Clinic) The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.
Ruple S. Laughlin, MD, FAAN (Mayo Clinic Rochester) Dr. Laughlin has nothing to disclose.
Smathorn Thakolwiboon, MD (Mayo Clinic Health System) Dr. Thakolwiboon has nothing to disclose.
Ashley Santilli, MD (Mayo Clinic) Dr. Santilli has nothing to disclose.
Hebatallah Rashed (Mayo Clinic) No disclosure on file
Igal Mirman, MD Dr. Mirman has nothing to disclose.
Alexander Swart, MD Dr. Swart has nothing to disclose.
Zhiyv Niu, PhD, FACMG (Mayo Clinic) Zhiyv Niu has nothing to disclose.
Sarah E. Berini, MD (Mayo Clinic) Dr. Berini has nothing to disclose.
Kamal Shouman, MD (Mayo Clinic) Dr. Shouman has nothing to disclose.
Marcus Vinicius R. Pinto, MD (Mayo Clinic) Dr. Pinto has nothing to disclose.
Michelle L. Mauermann, MD, FAAN (Mayo Clinic) The institution of Dr. Mauermann has received research support from IONIS. The institution of Dr. Mauermann has received research support from Alnylam. Dr. Mauermann has received publishing royalties from a publication relating to health care.
P. James B. Dyck, MD, FAAN (Mayo Clinic) Dr. Dyck has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Akcea/Ionis.
John R. Mills, MD, PhD (Mayo Clinic) The institution of Dr. Mills has received research support from Werfen Diagnostics. Dr. Mills has received intellectual property interests from a discovery or technology relating to health care.
William Harmsen (Mayo Clinic) William Harmsen has nothing to disclose.
Jayawant N. Mandrekar, PhD Dr. Mandrekar has nothing to disclose.
Christopher J. Klein, MD, FAAN (Mayo Clinic) Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda. Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sangamo Therapeutics . Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NMD Pharma.