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Review
. 2025 Oct 1;38(5):478-484.
doi: 10.1097/WCO.0000000000001414. Epub 2025 Aug 1.

Controversies in the diagnosis of chronic inflammatory demyelinating polyneuropathy

Pietro Emiliano Doneddu  1   2 , Carla Fasano  1 , Claudia Lozi  1 , Gaia Marenna  3 , Eduardo Nobile-Orazio  1   4
Affiliations
Review

Controversies in the diagnosis of chronic inflammatory demyelinating polyneuropathy

Pietro Emiliano Doneddu et al. Curr Opin Neurol. .

Abstract

Purpose of review: Despite decades of clinical recognition, the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) remains fraught with uncertainty. This review examines major areas of ongoing controversy in the diagnostic evaluation of CIDP, focusing on recent changes to electrodiagnostic criteria, disease boundaries, and emerging concepts of axonal damage.

Recent findings: Recent literature highlights three key areas of diagnostic uncertainty: the evolution and limitations of electrodiagnostic criteria; the diagnostic boundary between CIDP and antimyelin-associated glycoprotein (anti-MAG0 antibody neuropathy; and the recognition of CIDP cases that do not fulfil electrodiagnostic criteria, raising interest in axonal variants and the potential role of biomarkers such as neurofilaments. Across these domains, discrepancies between empirical evidence and expert-based guidelines persist, contributing to misdiagnosis and treatment variability.

Summary: Current CIDP criteria, though improved, remain partly based on expert opinion rather than empirical validation. The clinical heterogeneity of CIDP and its overlap with mimicking disorders further complicate diagnosis. A broader, more flexible diagnostic framework - integrating electrophysiology, biomarkers, and treatment response - is essential to enhance diagnostic accuracy and guide therapy. Future research should focus on refining criteria to strengthen electrodiagnostic standards and better accommodate atypical and axonal presentations.

Keywords: antimyelin-associated glycoprotein neuropathy; axonal damage; chronic inflammatory demyelinating polyradiculoneuropathy; electrodiagnostic criteria; neurofilaments.

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Conflict of interest statement

Pietro Emiliano Doneddu reports personal fees for Advisory from ArgenX - Belgium and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Eduardo Nobile-Orazio reports personal fees for Advisory or Scientific Board from ArgenX − Belgium, CSL-Behring - Italy and USA, Dianthus − USA, Janssen – USA, Kedrion – Italy, LFB – France, Roche – Switzerland, Sanofi – USA, Takeda − Italy and USA,. The other authors declare no conflict of interest.

Figures

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References

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    2. Current guidelines for the diagnosis and treatment of CIDP. These represent a revision of the previous 2010 EFNS/PNS guidelines.

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