• Pietro Emiliano Doneddu, Houseyin Akyil, Fiore Manganelli, Chiara Briani, Dario Cocito, Luana Benedetti, Anna Mazzeo, Raffaella Fazio, Massimiliano Filosto, Giuseppe Cosentino, Vincenzo Di Stefano, Giovanni Antonini, Girolama Alessandra Marfia, Maurizio Inghilleri, Gabriele Siciliano, Angelo Maurizio Clerici, Marinella Carpo, Angelo Schenone, Marco Luigetti, Giuseppe Lauria, Sabrina Matà, Tiziana Rosso, Giacomo Maria Minicuci, Marta Lucchetta, Guido Cavaletti, Giuseppe Liberatore, Emanuele Spina, Marta Campagnolo, Erdita Peci, Francesco Germano, Luca Gentile, Camilla Strano, Stefano Cotti Piccinelli, Elisa Vegezzi, Luca Leonardi, Giorgia Mataluni, Marco Ceccanti, Erika Schirinzi, Marina Romozzi, Eduardo Nobile-Orazio, and Italian CIDP Database study group.
• Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, via Manzoni 56, 20089 Rozzano, Milan, Italy.
• J. Neurol. Neurosurg. Psychiatr. 2023 Aug 1; 94 (8): 614621614-621.

BackgroundTo assess the ability of the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) clinical criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to include within their classification the whole spectrum of clinical heterogeneity of the disease and to define the clinical characteristics of the unclassifiable clinical forms.MethodsThe 2021 EAN/PNS clinical criteria for CIDP were applied to 329 patients fulfilling the electrodiagnostic (and in some cases also the supportive) criteria for the diagnosis of CIDP. Clinical characteristics were reviewed for each patient not strictly fulfilling the clinical criteria ('unclassifiable').ResultsAt study inclusion, 124 (37.5%) patients had an unclassifiable clinical presentation, including 110 (89%) with a typical CIDP-like clinical phenotype in whom some segments of the four limbs were unaffected by weakness ('incomplete typical CIDP'), 10 (8%) with a mild distal, symmetric, sensory or sensorimotor polyneuropathy confined to the lower limbs with cranial nerve involvement ('cranial nerve predominant CIDP') and 4 (1%) with a symmetric sensorimotor polyneuropathy limited to the proximal and distal areas of the lower limbs ('paraparetic CIDP'). Eighty-one (65%) patients maintained an unclassifiable presentation during the entire disease follow-up while 13 patients progressed to typical CIDP. Patients with the unclassifiable clinical forms compared with patients with typical CIDP had a milder form of CIDP, while there was no difference in the distribution patterns of demyelination.ConclusionsA proportion of patients with CIDP do not strictly fulfil the 2021 EAN/PNS clinical criteria for diagnosis. These unclassifiable clinical phenotypes may pose diagnostic challenges and thus deserve more attention in clinical practice and research.© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

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