Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?
Year: 2020
Type: article
Study design: Observational Study
Abstract: BACKGROUND AND PURPOSE: The aim was to identify the clinical and diagnostic investigations that may help to support a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria. METHODS: The data from patients with a clinical diagnosis of CIDP included in a national database were retrospectively reviewed. RESULTS: In all, 535 patients with a diagnosis of CIDP were included. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in three, while two had chronic immune sensory polyradiculopathy). Sixty-seven patients had a medical history and clinical signs compatible with CIDP but electrodiagnostic studies did not fulfill the EFNS/PNS criteria for CIDP. These patients had similar clinical features and frequency of abnormal supportive criteria for the diagnosis of CIDP compared to patients fulfilling EFNS/PNS criteria. Two or more abnormal supportive criteria were present in 40 (61.2%) patients rising to 54 (80.6%) if a history of a relapsing course as a possible supportive criterion was also included. Increased cerebrospinal fluid proteins and response to immune therapy most frequently helped in supporting the diagnosis of CIDP. Response to therapy was similarly frequent in patients fulfilling or not EFNS/PNS criteria (87.3% vs. 85.9%). CONCLUSIONS: Patients with a clinical diagnosis of CIDP had similar clinical findings, frequency of abnormal supportive criteria and response to therapy compared to patients fulfilling EFNS/PNS criteria. The presence of abnormal supportive criteria may help in supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.
Source: European Journal of Neurology
DOI: 10.1111/ene.14545
Authors: G. Liberatore, F. Manganelli, P. E. Doneddu, D. Cocito, R. Fazio, C. Briani, M. Filosto, L. Benedetti, A. Mazzeo, G. Antonini, G. Cosentino, S. Jann, A. Cortese, G. A. Marfia, A. M. Clerici, G. Siciliano, M. Carpo, M. Luigetti, G. Lauria, T. Rosso, G. Cavaletti, L. Santoro, E. Peci, S. Tronci, M. Ruiz, S. Cotti Piccinelli, A. Schenone, L. Leonardi, A. Toscano, G. Mataluni, E. Spina, L. Gentile, E. Nobile‐Orazio, the Italian CIDP Database Study Group
Institutions: University of Naples Federico II, Istituti Clinici Scientifici Maugeri, IRCCS Ospedale San Raffaele, University of Padua, Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia, University of Brescia, Ospedale Policlinico San Martino, University of Genoa, University of Messina, Azienda Ospedaliera Sant'Andrea, Sapienza University of Rome, University of Pavia, Fondazione Istituto Neurologico Nazionale Casimiro Mondino, University of Palermo, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Azienda Socio Sanitaria Territoriale Grande Ospedale Metropolitano Niguarda, University College London, University of Rome Tor Vergata, University of Insubria, Ospedale di Circolo e Fondazione Macchi, University of Pisa, Azienda Ospedaliera Treviglio, Azienda Socio Sanitaria Territoriale di Bergamo Ovest, Università Cattolica del Sacro Cuore, Agostino Gemelli University Polyclinic, University of Milan, Fondazione IRCCS Istituto Neurologico Carlo Besta, AULSS 2 Marca Trevigiana, University of Milano-Bicocca
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W3087670201