Unclassified clinical presentations of chronic inflammatory demyelinating polyradiculoneuropathy.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Doneddu Pietro Emiliano, Akyil Houseyin, Manganelli Fiore, Briani Chiara, Cocito Dario, Benedetti Luana, Mazzeo Anna, Fazio Raffaella, Filosto Massimiliano, Cosentino Giuseppe, Di Stefano Vincenzo, Antonini Giovanni, Marfia Girolama Alessandra, Inghilleri Maurizio, Siciliano Gabriele, Clerici Angelo Maurizio, Carpo Marinella, Schenone Angelo, Luigetti Marco, Lauria Giuseppe, Matà Sabrina, Rosso Tiziana, Minicuci Giacomo Maria, Lucchetta Marta, Cavaletti Guido, Liberatore Giuseppe, Spina Emanuele, Campagnolo Marta, Peci Erdita, Germano Francesco, Gentile Luca, Strano Camilla, Cotti Piccinelli Stefano, Vegezzi Elisa, Leonardi Luca, Mataluni Giorgia, Ceccanti Marco, Schirinzi Erika, Romozzi Marina, Nobile-Orazio Eduardo, Italian CIDP Database study group
Аннотация
METHODS: The 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').
RESULTS: At 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.
CONCLUSIONS: A 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.