Impact of 2021 European Academy of Neurology/Peripheral Nerve Society diagnostic criteria on diagnosis and therapy of chronic inflammatory demyelinating polyradiculoneuropathy variants.
European Academy of Neurology
Lewis-Sumner syndrome
Peripheral Nerve Society
chronic inflammatory demyelinating polyradiculoneuropathy
chronic inflammatory demyelinating polyradiculoneuropathy variants
diagnostic criteria
intravenous immunoglobulin
steroids
Journal
European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311
Informations de publication
Date de publication:
02 Jan 2024
02 Jan 2024
Historique:
revised:
20
11
2023
received:
10
08
2023
accepted:
08
12
2023
medline:
2
1
2024
pubmed:
2
1
2024
entrez:
2
1
2024
Statut:
aheadofprint
Résumé
There are different criteria for the diagnosis of different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines provide specific clinical criteria for each CIDP variant even if their therapeutical impact has not been investigated. We applied the clinical criteria for CIDP variants of the 2021 EAN/PNS guidelines to 369 patients included in the Italian CIDP database who fulfilled the 2021 EAN/PNS electrodiagnostic criteria for CIDP. According to the 2021 EAN/PNS clinical criteria, 245 patients achieved a clinical diagnosis of typical CIDP or CIDP variant (66%). We identified 106 patients with typical CIDP (29%), 62 distal CIDP (17%), 28 multifocal or focal CIDP (7%), four sensory CIDP (1%), 27 sensory-predominant CIDP (7%), 10 motor CIDP (3%), and eight motor-predominant CIDP (2%). Patients with multifocal, distal, and sensory CIDP had milder impairment and symptoms. Patients with multifocal CIDP had less frequently reduced conduction velocity and prolonged F-wave latency and had lower levels of cerebrospinal fluid protein. Patients with distal CIDP more frequently had reduced distal compound muscle action potentials. Patients with motor CIDP did not improve after steroid therapy, whereas those with motor-predominant CIDP did. None of the patients with sensory CIDP responded to steroids, whereas most of those with sensory-predominant CIDP did. The 2021 EAN/PNS criteria for CIDP allow a better characterization of CIDP variants, permitting their distinction from typical CIDP and more appropriate treatment for patients.
Sections du résumé
BACKGROUND AND PURPOSE
OBJECTIVE
There are different criteria for the diagnosis of different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines provide specific clinical criteria for each CIDP variant even if their therapeutical impact has not been investigated.
METHODS
METHODS
We applied the clinical criteria for CIDP variants of the 2021 EAN/PNS guidelines to 369 patients included in the Italian CIDP database who fulfilled the 2021 EAN/PNS electrodiagnostic criteria for CIDP.
RESULTS
RESULTS
According to the 2021 EAN/PNS clinical criteria, 245 patients achieved a clinical diagnosis of typical CIDP or CIDP variant (66%). We identified 106 patients with typical CIDP (29%), 62 distal CIDP (17%), 28 multifocal or focal CIDP (7%), four sensory CIDP (1%), 27 sensory-predominant CIDP (7%), 10 motor CIDP (3%), and eight motor-predominant CIDP (2%). Patients with multifocal, distal, and sensory CIDP had milder impairment and symptoms. Patients with multifocal CIDP had less frequently reduced conduction velocity and prolonged F-wave latency and had lower levels of cerebrospinal fluid protein. Patients with distal CIDP more frequently had reduced distal compound muscle action potentials. Patients with motor CIDP did not improve after steroid therapy, whereas those with motor-predominant CIDP did. None of the patients with sensory CIDP responded to steroids, whereas most of those with sensory-predominant CIDP did.
CONCLUSIONS
CONCLUSIONS
The 2021 EAN/PNS criteria for CIDP allow a better characterization of CIDP variants, permitting their distinction from typical CIDP and more appropriate treatment for patients.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e16190Subventions
Organisme : GBS/CIDP Foundation International
Organisme : CSL Behring
Organisme : Humanitas Clinical and Research Institute (Milan, Italy)
Organisme : Ministero della Salute
ID : Ricerca Finalizzata (Progetto RF-2016-02361887)
Organisme : Kedrion Biopharma
Organisme : Regione Lombardia
Investigateurs
Pietro Emiliano Doneddu
(PE)
Alberto De Lorenzo
(A)
Giuseppe Liberatore
(G)
Eduardo Nobile -Orazio
(EN)
Dario Cocito
(D)
Fiore Manganelli
(F)
Emanuele Spina
(E)
Enrica Pisano
(E)
Lucio Santoro
(L)
Daniele Velardo
(D)
Camilla Strano
(C)
Raffaella Fazio
(R)
Marta Ruiz
(M)
Mario Cacciavillani
(M)
Francesca Castellani
(F)
Chiara Briani
(C)
Filomena Caria
(F)
Massimiliano Filosto
(M)
Elisa Bianchi
(E)
Ettore Beghi
(E)
Elena Pinuccia Verrengia
(EP)
Stefano Jann
(S)
Antonio Toscano
(A)
Luca Gentile
(L)
Massimo Russo
(M)
Anna Mazzeo
(A)
Luca Leonardi
(L)
Giovanni Antonini
(G)
Giuseppe Cosentino
(G)
Ilaria Callegari
(I)
Andrea Cortese
(A)
Giorgia Mataluni
(G)
Girolama Alessandra Marfia
(GA)
Angelo Maurizio Clerici
(AM)
Federica Scrascia
(F)
Marinella Carpo
(M)
Angelo Schenone
(A)
Luana Benedetti
(L)
Corrado Cabona
(C)
Alessandro Beronio
(A)
Erika Schirinzi
(E)
Gabriele Siciliano
(G)
Marco Luigetti
(M)
Patrizia Dacci
(P)
Giuseppe Lauria
(G)
Tiziana Rosso
(T)
Claudia Balducci
(C)
Guido Cavaletti
(G)
Mario Sabatelli
(M)
Erdita Peci
(E)
Informations de copyright
© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.
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