Application of the 2021 EAN/PNS criteria for chronic inflammatory demyelinating polyneuropathy

Abstract

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

Evaluation of diagnostic yield of the 2021 European Academy of Neurology/Peripheral Nerve Society diagnostic criteria for CIDP

“…There have so far been two studies that investigated the sensitivity and specificity of the EAN/PNS 2021 criteria and compared them with the EFNS/PNS 2010 criteria. 14,15 Of these, a multicenter study using an Italian CIDP database, reported that the EAN/PNS 2021 criteria showed lower sensitivity and higher specificity than the EFNS/ PNS 2010 criteria, 14 findings consistent with our results. A singlecenter study from the UK, which used a strict alternate reference standard (objective response to treatment) for a CIDP cohort similar to our study, reported equivalent sensitivity and specificity of both criteria and that the addition of the SCS criteria to the updated version showed no added diagnostic value.…”

Chronic Immune-Mediated Demyelinating Neuropathies

“…The best balance between sensitivity (88% for possible CIDP and 81% for CIDP) and specificity (86% for possible CIDP and 97% for CIDP) was achieved when at least four motor and four sensory nerves were tested on electrodiagnostic studies. 15,16 Generally speaking, a demyelinating pattern is present on electrodiagnostic testing with abnormalities noted in motor and sensory nerves.…”

“…Two 2022 validation studies of the EAN and Peripheral Nerve Society 2021 diagnostic criteria showed that these criteria are roughly equivalent to the European Federation of Neurological Societies and Peripheral Nerve Society criteria and tend to improve the diagnostic accuracy of CIDP variants. The best balance between sensitivity (88% for possible CIDP and 81% for CIDP) and specificity (86% for possible CIDP and 97% for CIDP) was achieved when at least four motor and four sensory nerves were tested on electrodiagnostic studies 15,16 . Generally speaking, a demyelinating pattern is present on electrodiagnostic testing with abnormalities noted in motor and sensory nerves.…”

Validation of the 2021 EAN/PNS diagnostic criteria for chronic inflammatory demyelinating polyneuropathy

“…Two JNNP studies reported the sensitivity and specificity of the 2021 EAN/PNS criteria, and the results are compared with those of the 2010 EFNS/PNS criteria 3 4. Doneddu et al described results of an Italian multicentre study based on Italian CIDP registry database (n=330),3 whereas Rajabally et al reported analyses on a CIDP cohort of a single centre in the UK (N=120) 4 Table 1. summarises the results of the two studies.…”

“… 3 4 Doneddu et al described results of an Italian multicentre study based on Italian CIDP registry database (n=330), 3 whereas Rajabally et al reported analyses on a CIDP cohort of a single centre in the UK (N=120). 4 Table 1 summarises the results of the two studies. Both studies show very high specificity, 98% and 94%, respectively.…”