Relevance of diagnostic investigations in chronic inflammatory demyelinating poliradiculoneuropathy: Data from the Italian CIDP database

Giuseppe Liberatore, Fiore Manganelli, Dario Cocito, Raffaella Fazio, Chiara Briani, Massimiliano Filosto, Luana Benedetti, Giovanni Antonini, Giuseppe Cosentino, Stefano Jann, Anna Mazzeo, Andrea Cortese, Girolama Alessandra Marfia, Angelo Maurizio Clerici, Gabriele Siciliano, Marinella Carpo, Mario Sabatelli, Giuseppe Lauria, Tiziana Rosso, Eduardo Nobile-OrazioPietro Emiliano Doneddu, Francesca Gallia, Eduardo Nobile-Orazio, Erdita Peci, Stefano Tronci, Lucio Santoro, Emanuele Spina, Marta Ruiz, Stefano Cotti Piccinelli, Alessandro Beronio, Antonio Toscano, Luca Gentile, Giorgia Mataluni, Laura Piccolo, Ilaria Callegari, Brigida Fierro, Verrengia Elena Pinuccia, Elisa Bianchi, Ettore Beghi, Federica Scrascia, Martina Garnero, Angelo Schenone, Marco Luigetti, Patrizia Dacci, Luca Leonardi, Erika Schirinzi, Claudia Balducci, Guido Cavaletti

Research output: Contribution to journalArticlepeer-review

Abstract

The objective of our work was to report the clinical features and the relevance of diagnostic investigations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We retrospectively reviewed data from patients with a clinical diagnosis of CIDP included in a national database. Among the 500 included patients with a clinical diagnosis of CIDP, 437 patients (87%) fulfilled the European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP (definite in 407, probable in 26, possible in four). In 352 patients (86%) motor nerve conduction abnormalities consistent with demyelination were sufficient for the diagnosis of definite CIDP. In 55 patients, this diagnosis required the addition of one or two (from probable or from possible CIDP, respectively) supportive tests, while in 20 cases they improved the diagnosis from possible to probable CIDP, seven patients did not change diagnosis. Considering these 85 patients, cerebrospinal fluid studies were performed in 79 cases (93%) upgrading the certainty of diagnosis in 59% of examined patients. Sensory nerve conduction studies (NCS) were performed in 85% of patients with an improvement of diagnosis in 32% of cases. Nerve biopsy and ultrasound and magnetic resonance imaging (US/MRI) exams resulted positive in about 40% of examined patients, but they were performed in few patients (7 patients and 16 patients, respectively). A response to the therapy was present in 84% of treated patients (n = 77), contributing to support the diagnosis in 40 patients in whom the other supportive criteria were not sufficient. In most patients with CIDP the diagnosis is possible solely with motor NCS while other investigations may help improving the diagnosis in a minority of patients.

Original languageEnglish
Pages (from-to)152-161
Number of pages10
JournalJournal of the Peripheral Nervous System
Volume25
Issue number2
DOIs
Publication statusPublished - Jun 1 2020

Keywords

  • chronic inflammatory demyelinating neuropathy
  • CIDP
  • diagnostic criteria
  • EMG
  • peripheral neuropathy

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology

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