TY - JOUR
T1 - Chronic inflammatory demyelinating polyradiculoneuropathy
T2 - Can we make a diagnosis in patients not fulfilling electrodiagnostic criteria?
AU - Liberatore, Giuseppe
AU - Manganelli, Fiore
AU - Doneddu, Pietro Emiliano
AU - Cocito, Dario
AU - Fazio, Raffaella
AU - Briani, Chiara
AU - Filosto, Massimiliano
AU - Benedetti, Luana
AU - Mazzeo, Anna
AU - Antonini, Giovanni
AU - Cosentino, Giuseppe
AU - Jann, Stefano
AU - Cortese, Andrea
AU - Marfia, Girolama Alessandra
AU - Clerici, Angelo Maurizio
AU - Siciliano, Gabriele
AU - Carpo, Marinella
AU - Luigetti, Marco
AU - Lauria, Giuseppe
AU - Rosso, Tiziana
AU - Cavaletti, Guido
AU - Santoro, Lucio
AU - Peci, Erdita
AU - Tronci, Stefano
AU - Ruiz, Marta
AU - Piccinelli, Stefano Cotti
AU - Schenone, Angelo
AU - Leonardi, Luca
AU - Toscano, Antonio
AU - Mataluni, Giorgia
AU - Spina, Emanuele
AU - Gentile, Luca
AU - Nobile-Orazio, Eduardo
N1 - This article is protected by copyright. All rights reserved.
PY - 2021/2
Y1 - 2021/2
N2 - 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.
AB - 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.
U2 - 10.1111/ene.14545
DO - 10.1111/ene.14545
M3 - Article
C2 - 32959475
VL - 28
SP - 620
EP - 629
JO - European Journal of Neurology
JF - European Journal of Neurology
SN - 1351-5101
IS - 2
ER -