Chronic inflammatory demyelinating polyradiculoneuropathy: Can we make a diagnosis in patients not fulfilling electrodiagnostic criteria?

Giuseppe Liberatore, Fiore Manganelli, Pietro Emiliano Doneddu, Dario Cocito, Raffaella Fazio, Chiara Briani, Massimiliano Filosto, Luana Benedetti, Anna Mazzeo, Giovanni Antonini, Giuseppe Cosentino, Stefano Jann, Andrea Cortese, Girolama Alessandra Marfia, Angelo Maurizio Clerici, Gabriele Siciliano, Marinella Carpo, Marco Luigetti, Giuseppe Lauria, Tiziana RossoGuido Cavaletti, Lucio Santoro, Erdita Peci, Stefano Tronci, Marta Ruiz, Stefano Cotti Piccinelli, Angelo Schenone, Luca Leonardi, Antonio Toscano, Giorgia Mataluni, Emanuele Spina, Luca Gentile, Eduardo Nobile-Orazio

Research output: Contribution to journalArticlepeer-review

Abstract

OBJECTIVE: to identify the clinical and diagnostic investigations that may help supporting a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in patients not fulfilling the EFNS/PNS electrodiagnostic criteria.METHODS: we retrospectively reviewed the data from patients with a clinical diagnosis of CIDP included in a national database.RESULTS: we included 535 patients with a diagnosis of CIDP. This diagnosis fulfilled the EFNS/PNS criteria in 468 patients (87.2%) (definite in 430, probable in 33, possible in 3, while two had CISP). 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 raising to 54 (80.6%) if we also included a history of a relapsing course as a possible supportive criteria. 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% versus 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 supporting the diagnosis of CIDP in patients with a medical history and clinical signs compatible with this diagnosis but non-diagnostic nerve conduction studies.
Original languageEnglish
Pages (from-to)620-629
Number of pages10
JournalEur. J. Neurol.
Volume28
Issue number2
DOIs
Publication statusPublished - Feb 2021

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