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Original research
Application of the 2021 EAN/PNS criteria for chronic inflammatory demyelinating polyneuropathy
  1. Yusuf A Rajabally1,2,
  2. Saadia Afzal1,
  3. Lay Khoon Loo1,
  4. HS Goedee3
  1. 1Inflammatory Neuropathy Clinic, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  2. 2Aston Medical School, Aston University, Birmingham, UK
  3. 3Department of Neurology and Neurosurgery, University Medical Center Utrecht, Utrecht, The Netherlands
  1. Correspondence to Prof. Yusuf A Rajabally, Inflammatory Neuropathy Clinic, University Hospitals Birmingham NHS Foundation Trust, Birmingham, B15 2TH, UK; y.rajabally{at}aston.ac.uk

Abstract

Background The diagnostic value of new criteria of the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) for chronic inflammatory demyelinating polyneuropathy (CIDP) is unknown.

Methods We performed a retrospective study of fulfilment of EAN/PNS 2021 criteria on 120 consecutive patients with a clinical diagnosis of ‘suspected CIDP’ and objective treatment response, attending University Hospitals Birmingham, UK. Specificity was evaluated versus 100 consecutive controls.

Results The sensitivity of EAN/PNS criteria for ‘CIDP’ was 83.3%. The sensitivity for ‘CIDP’ or ‘possible CIDP’ was 93.3%. Specificity was of 94% for ‘CIDP’ and 79% for ‘CIDP’ or ‘possible CIDP’. No sensitivity/specificity differences were ascertained with previous versions (‘CIDP’: sensitivity: 83.3% vs 81.3%, p=0.74, specificity: 94% vs 96.1%, p=0.38, respectively; ‘CIDP’ or ‘possible CIDP’: sensitivity: 93.3% vs 96.7%, p=0.25 and specificity: 79% vs 69.2 %, p=0.09, respectively). F-wave prolongation, proximal and distal temporal dispersion were the most likely parameters to contribute to false positives, whereas distal motor latency was the least likely. No impact of sensory electrophysiology could be ascertained. ‘Typical CIDP’ represented 79% of the CIDP cohort. The largest component of the ‘variant CIDP’ group was represented by focal/multifocal forms (14%). With new criteria, 6.7% of the cohort did not meet requirements, among whom the majority (75%) had paranodopathy or chronic immune sensory polyradiculopathy (CISP).

Discussion The sensitivity and specificity of new EAN/PNS criteria for CIDP is equivalent to that of previous versions. The exclusion of paranodopathies and CISP from the CIDP spectrum impacts on management of a non-negligible proportion of treatment-responsive patients.

  • CLINICAL NEUROLOGY
  • NEUROPHYSIOL, CLINICAL
  • NEUROPATHY
  • EMG
  • NEUROMUSCULAR

Data availability statement

Data are available on reasonable request. Available on reasonable request.

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Data availability statement

Data are available on reasonable request. Available on reasonable request.

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Footnotes

  • Contributors YAR: data analysis, data interpretation, drafting of first version of manuscript. SA: data collection, data analysis, review of manuscript for intellectual content. LKL: data collection, review of manuscript for intellectual content HSG: data interpretation, drafting of first version of manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests LKL and SA have no disclosures.YAR has received speaker/consultancy honoraria from LFB, Polyneuron and Argenx and has received educational sponsorships from LFB and CSL Behring and has obtained research grants from LFB and CSL Behring. HSG has received research grants from Prinses Beatrix Spierfonds and travel grants speaker fee from Shire/Baxalta and Takeda.

  • Provenance and peer review Not commissioned; externally peer reviewed.