Chronic inflammatory demyelinating polyradiculoneuropathy: can a diagnosis be made in patients not fulfilling electrodiagnostic criteria?. (15th October 2020)

• Record Type: Journal Article
• Authors: Liberatore, G.; Manganelli, F.; Doneddu, P. E.; Cocito, D.; Fazio, R.; Briani, C.; Filosto, M.; Benedetti, L.; Mazzeo, A.; Antonini, G.; Cosentino, G.; Jann, S.; Cortese, A.; Marfia, G. A.; Clerici, A. M.; Siciliano, G.; Carpo, M.; Luigetti, M.; Lauria, G.; Rosso, T.; Cavaletti, G.; Santoro, L.; Peci, E.; Tronci, S.; Ruiz, M.; Cotti Piccinelli, S.; Schenone, A.; Leonardi, L.; Toscano, A.; Mataluni, G.; Spina, E.; Gentile, L.; Nobile‐Orazio, E.;
• Other Names: Doneddu Pietro Emiliano investigator.; Liberatore Giuseppe investigator.; Gallia Francesca investigator.; Nobile‐Orazio Eduardo investigator.; Peci Erdita investigator.; Cocito Dario investigator.; Tronci Stefano investigator.; Fazio Raffaella investigator.; Manganelli Fiore investigator.; Santoro Lucio investigator.; Spina Emanuele investigator.; Ruiz Marta investigator.; Briani Chiara investigator.; Cotti Piccinelli Stefano investigator.; Filosto Massimiliano investigator.; Beronio Alessandro investigator.; Benedetti Luana investigator.; Toscano Antonio investigator.; Gentile Luca investigator.; Mazzeo Anna investigator.; Mataluni Giorgia investigator.; Marfia Girolama Alessandra investigator.; Piccolo Laura investigator.; Cortese Andrea investigator.; Cosentino Giuseppe investigator.; Fierro Brigida investigator.; Verrengia Elena Pinuccia investigator.; Jann Stefano investigator.; Bianchi Elisa investigator.; Beghi Ettore investigator.; Clerici Angelo Maurizio investigator.; Scrascia Federica investigator.; Carpo Marinella investigator.; Garnero Martina investigator.; Schenone Angelo investigator.; Luigetti Marco investigator.; Sabatelli Mario investigator.; Dacci Patrizia investigator.; Lauria Giuseppe investigator.; Leonardi Luca investigator.; Antonini Giovanni investigator.; Rosso Tiziana investigator.; Schirinzi Erika investigator.; Siciliano Gabriele investigator.; Balducci Claudia investigator.; Cavaletti Guido investigator.;
• Is Part Of: European journal of neurology. Volume 28:Number 2(2021)
• Page Start: 620
• Page End: 629
• Publication Date: 2020-10-15
• Subjects: chronic inflammatory demyelinating polyradiculoneuropathy -- diagnostic criteria -- electrophysiology; Neurology -- Periodicals; Nervous system -- Diseases -- Periodicals; 616.8
• DOI: 10.1111/ene.14545
• Languages: English
• ISSNs: 1351-5101

Abstract

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. Abstract : In most reported CIDP series, there is a consistent proportion of patients who have the clinical features compatible with a diagnosis of CIDP but who do not fulfill the EFNS/PNS electrodiagnostic criteria and, therefore, might be denied the access to effective therapy. In our study, 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 supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non‐diagnostic nerve conduction studies.