Article Text

Download PDFPDF
Original research
Comparison of the diagnostic accuracy of the 2021 EAN/PNS and 2010 EFNS/PNS diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy
  1. Pietro Emiliano Doneddu1,2,
  2. Alberto De Lorenzo1,
  3. Fiore Manganelli3,
  4. Dario Cocito4,
  5. Raffaella Fazio5,
  6. Chiara Briani6,
  7. Anna Mazzeo7,
  8. Massimiliano Filosto8,
  9. Giuseppe Cosentino9,10,
  10. Luana Benedetti11,
  11. Angelo Schenone11,12,
  12. Girolama Alessandra Marfia13,
  13. Giovanni Antonini14,
  14. Sabrina Matà15,
  15. Marco Luigetti16,17,
  16. Giuseppe Liberatore1,
  17. Emanuele Spina3,
  18. Erdita Peci18,
  19. Camilla Strano5,
  20. Mario Cacciavillani19,
  21. Luca Gentile7,
  22. Stefano Cotti Piccinelli8,
  23. Andrea Cortese9,10,
  24. Elisa Bianchi20,
  25. Eduardo Nobile-Orazio1,21
  1. 1Neuromuscular and Neuroimmunology Unit, Humanitas Clinical and Research Center—IRCCS, Milano, Italy
  2. 2Department of Biomedical Sciences, Humanitas University, Milan, Italy
  3. 3Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples Federico II, Napoli, Campania, Italy
  4. 4Divisione di Riabilitazione Neuromotoria, Istituti Clinici Scientifici Maugeri—Presidio Sanitario Major, Torino, Italy, Torino, Italy
  5. 5Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), Istituto di Ricovero e Cura a Carattere Scientifico Ospedale San Raffaele, Milano, Lombardia, Italy
  6. 6Neurology Unit, Department of Neuroscience, University of Padova, Padova, Italy
  7. 7Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, Sicilia, Italy
  8. 8Department of Clinical and Experimental Sciences, NeMO-Brescia Clinical Center for Neuromuscular Diseases, University of Brescia, Brescia, Italy
  9. 9IRCCS Mondino Foundation, Pavia, Italy
  10. 10Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy
  11. 11IRCCS Ospedale Policlinico San Martino, Genova, Italy
  12. 12Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Genova, Italy
  13. 13Dysimmune Neuropathies Unit, Department of Systems Medicine, University of Rome Tor Vergata, Roma, Italy
  14. 14Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, University of Rome La Sapienza, Rome, Italy
  15. 15Dipartimento Neuromuscoloscheletrico e degli organi di Senso, Neurology Unit, Careggi University Hospital, Firenze, Italy
  16. 16UOC Neurologia, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma, Italy
  17. 17Università Cattolica del Sacro Cuore, Roma, Italy
  18. 18Department of Neuroscience, University of Turin, Torino, Italy
  19. 19EMG Unit, Gruppo Data Medica, CEMES, Padova, Italy
  20. 20Laboratorio di Malattie Neurologiche, Istituto di ricerche farmacologiche Mario Negri IRCCS, Milano, Italy
  21. 21Department of Medical Biotechnology, Translational Medicine Milan University, Italy
  1. Correspondence to Professor Eduardo Nobile-Orazio, IRCCS Humanitas Research Hospital, Rozzano, Italy; eduardo.nobile{at}unimi.it

Abstract

Objectives To compare the sensitivity and specificity of the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with those of the 2010 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS).

Methods Sensitivity and specificity of the two sets of criteria were evaluated in 330 patients with CIDP and 166 axonal peripheral neuropathy controls. Comparison of the utility of nerve conduction studies with different number of nerves examined and of the sensitivity and specificity of the two criteria in typical CIDP and its variants were assessed.

Results EFNS/PNS criteria had a sensitivity of 92% for possible CIDP and 85% for probable/definite CIDP, while the EAN/PNS criteria had a sensitivity of 83% for possible CIDP and 74% for CIDP. Using supportive criteria, the sensitivity of the EAN/PNS criteria for possible CIDP increased to 85% and that of CIDP to 77%, remaining lower than that of the EFNS/PNS criteria. Specificity of the EFNS/PNS criteria was 68% for possible CIDP and 84% for probable/definite CIDP, while the EAN/PNS criteria had a specificity of 88% for possible CIDP and 98% for CIDP. More extended studies increased the sensitivity of both sets of criteria by 4%–7% but reduced their specificity by 2%–3%. The EFNS/PNS criteria were more sensitive for the diagnosis of typical CIDP while the EAN/PNS criteria were more specific for the diagnosis of distal and sensory CIDP.

Conclusions In our population, the EAN/PNS criteria were more specific but less sensitive than the EFNS/PNS criteria. With the EAN/PNS criteria, more extended nerve conduction studies are recommended to obtain an acceptable sensitivity while maintaining a high specificity.

  • neurophysiology
  • neuropathy
  • neuromuscular
  • EMG
  • neuroimmunology

Data availability statement

Data are available upon reasonable request. Anonymised data used for this study are available upon reasonable request from the corresponding author.

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Data availability statement

Data are available upon reasonable request. Anonymised data used for this study are available upon reasonable request from the corresponding author.

View Full Text

Footnotes

  • PED and ADL contributed equally.

  • Collaborators The Italian CIDP database study group: Enrica Pisano and Lucio Santoro from the Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples ‘Federico II’, Naples, Italy; Daniele Velardo, Camilla Strano from the Division of Neuroscience, Department of Neurology, Institute of Experimental Neurology (INSPE), San Raffaele Scientific Institute, Milan, Italy; Marta Ruiz, Francesca Castellani from the Neurology Unit, Department of Neuroscience, University of Padua, Padua, Italy; Filomena Caria from the NeMO-Brescia Clinical Center for Neuromuscular Diseases, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy; Ettore Beghi from IRCCS-Istituto Mario Negri, Milan, Italy; Elena Pinuccia Verrengia and Stefano Jann from the Department of Neuroscience, Niguarda Ca’ Granda Hospital, Milan, Italy; Antonio Toscano, Massimo Russo from the Department of Clinical and Experimental Medicine, Unit of Neurology, University of Messina, Messina, Italy; Luca Leonardi from the Unit of Neuromuscular Diseases, Department of Neurology Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, ‘Sapienza’ University of Rome, Sant’Andrea Hospital, Rome, Italy; Ilaria Callegari, Elisa Vegezzi from Translational Neurophysiology Research Unit, IRCCS Mondino Foundation, Pavia, Italy and Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy; Giorgia Mataluni from the Disimmune Neuropathies Unit, Department of Systems Medicine, Tor Vergata University of Rome, Rome, Italy; Angelo Maurizio Clerici from the Neurology Unit, Circolo Federica Scrascia and Marinella Carpo from the ASST Bergamo Ovest-Ospedale Treviglio, Treviglio, Italy; Corrado Cabona from IRCCS Ospedale Policlinico San Martino, Genoa, Italy; Alessandro Beronio from Ospedale Sant’Andrea, La Spezia, Italy; Erika Schirinzi and Gabriele Siciliano from the Neurology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy; Patrizia Dacci and Giuseppe Lauria from the Unit of Neuroalgology, IRCCS Foundation 'Carlo Besta' Neurological Institute, Milan, Italy; Tiziana Rosso from the Azienda UL.SS. 8 Asolo, Castelfranco Veneto, Italy; Claudia Balducci and Guido Cavaletti from the School of Medicine and Surgery and Experimental Neurology Unit, University of Milano-Bicocca, Monza, Italy; Mario Sabatelli from Centro Clinico NEMO Adulti, Universita’ Cattolica del Sacro Cuore, Roma, Italy.

  • Contributors PED and ADL contributed to the conception of the research project, performed the statistical analysis, wrote the first draft of the report and reviewed the report. FM, DC, RF, CB, AM, MF, GC, LB, AS, GAM, GA, SM, ML, GL, ES, EP, CS, MC, LG, SCP, EB contributed to the conception, organisation and execution of the research project, reviewed and commented on the statistical analysis and the report. ENO conceived, organised and designed the study, reviewed and commented on the statistical analysis, reviewed the report. PED and ENO accept full responsibility for the work and the conduct of the study, had access to the data, and controlled the decision to publish.

  • Funding The study was supported by a Grant from Ministero della Salute, Ricerca Finalizzata (Progetto RF-2016-02361887). The study derives from a project initially supported by Regione Lombardia, Italy, (Rare Disease Project 2013 'A Database from Lombardia on CIDP') and by the GBS-CIDP Foundation International (USA). The study was also supported by unrestricted grants from Kedrion Biopharma (Italy), CSL Behring (Italy) and Humanitas Clinical and Research Institute (Milan, Italy). The funders had no role in study design, data collection and analysis, decision to publish or preparation of the manuscript.

  • Competing interests Pietro Emiliano Doneddu has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Fiore Manganelli reports personal fees for scientific events from CSL Behring and has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Dario Cocito has received honoraria for lecturing from Shire, CSL Behring, and Kedrion and travel grants to attend scientific meeting from Shire, Kedrion, and CSL Behring. Raffaella Fazio has served on scientific advisory boards for CSL Behring and has received travel grants from Kedrion and CSL Behring to attend scientific meeting. Chiara Briani has served on scientific advisory boards for Pfizer, Alnylam, and Akcea, and has received travel grants from Kedrion and CSL Behring to attend scientific meeting. Anna Mazzeo has received travel grants from Kedrion and CSL Behring to attend scientific meeting. Massimiliano Filosto has served on scientific advisory boards for CSL Behring, Sanofi and Amicus and has received travel grants from Sanofi, Biogen, Kedrion and CSL Behring to attend scientific meeting. Giuseppe Cosentino has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Marco Luigetti has received travel grants to attend scientific meetings from Kedrion. Girolama Alessandra Marfia has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Giuseppe Liberatore has received travel grants to attend scientific meetings from CSL Behring and Kedrion. Erdita Peci has received travel grants to attend scientific meetings from CSL Behring. Eduardo Nobile-Orazio reports personal fees for Advisory or Scientific Board from ArgenX—Belgium, Takeda—Italy and USA, CSL-Behring - Italy and USA, Janssen—USA, Kedrion—Italy, LFB—France, Roche—Switzerland, Sanofi - USA. The other authors declare no conflict of interest.

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

Linked Articles