Impact of 2021 European Academy of Neurology/Peripheral Nerve Society Diagnostic Criteria on Diagnosis and Therapy of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Variants

Authors: Lorenzo, A de; Liberatore, G; Doneddu, PE; Manganelli, F; Cocito, D; Briani, Ch; Fazio, R; Mazzeo, A; Schenone, A; Di Stefano, V; Cosentino, G; Marfia, GA; Benedetti, L; Carpo, M; Filosto, Massimiliano; AG; Clerici, AM; Luigetti, Marco; MS; Rosso, T; Lucchetta, M; Siciliano, G; Lauria PG; Cavaletti, G; Inghilleri, M; Cantisani, T; Notturno, F; Ricciardi, D; Habetswallner, F; Spina, E; Peci, E; Salvalaggio, A; Falzone, Y; Strano, C; Gentile, L; Vegezzi, E; Mataluni, G; Cotti Piccinelli, S; Leonardi, L; Romano, A; Nobile-Orazio, E; Pisano, E; Santoro, L; Velardo, D; Ruiz, M; Cacciavillani, M; Castellani, F; Caria, F; Bianchi, E; Beghi, E; Verrengia, EP; Jann, S; Toscano, A; Russo, M; Callegari, I; Cortese, A; Scrascia, F; Cabona, C; Beronio, A; Schirinzi, E; Dacci, P; Lauria, G; Balducci, C; Sabatelli, M.

Affiliations: IRCCS, Neurol Inst C Besta, Neuromuscular Dis & Neuroimmunol Unit, Milan, Italy. Human Univ, Dept Biomed Sci, Milan, Italy. Feder II Univ Naples, Dept Neurosci, Reprod Sci & Odontostomatol, Naples, Italy. Univ Turin, Dept Neurosci, Turin, Italy. Univ Padua, Dept Neurosci, Neurol Unit, Padua, Italy. IRCCS San Raffaele Sci Inst, Div Neurosci, Inst Expt Neurol, Dept Neurol, Milan, Italy. Univ Messina, Dept Clin & Expt Med, Unit Cardiol, Messina, Italy. IRCCS Osped Policlin San Martino Genova, I-16132 Genoa, Italy. Univ Genoa, IRCCS AOU San Martino IST, Dept Neurosci Rehabil Ophthalmol Genet Maternal &, Genoa, Italy. Univ Palermo, Dept Biomed Neurosci & Adv Diagnost, Palermo, Italy. Univ Pavia, Dept Brain & Behav Sci, Pavia, Italy. IRCCS Mondino Fdn, Pavia, Italy. Univ Tor Vergata Rome, Dept Syst Med, Hematol Unit, Rome, Italy. ASST Bergamo Ovest Osped Treviglio Caravaggio, Treviglio, Italy. Univ Brescia, Ctr Neuromuscular Dis & Neuropathies, Neurol Unit, ASST Spedali Civili, Brescia, Italy. Sapienza Univ Rome, St Andrea Hosp, Unit Neuromuscular Dis,Fac Med & Psychol, Dept Neurol Mental Hlth & Sensory Organs NESMOS, Rome, Italy. Circolo Hosp & Macchi Fdn, Neurol Unit, Varese, Italy. Univ Cattolica Sacro Cuore, Fdn Policlin Univ Agostino Gemelli IRCCS, Urol Dept, Rome, Italy. Careggi Univ Hosp, Neurol Unit, Florence, Italy. Osped San Bortolo, UOC Neuroradiol, Vicenza, VI, Italy. Osped St Maria Misericordia, Dept Radiol, Perugia, Italy. Univ Pisa, Dept Clin & Expt Med, Neurol Unit, Pisa, Italy. IRCCS Fdn Carlo Besta Neurol Inst, Neuroalgol Unit, Milan, Italy. Univ Milan, Dept Med Biotechnol & Translat Med, Milan, Italy. Milano Bicocca, Sch Med & Surg, Expt Neurol Unit, Monza, Italy. Sapienza Univ Rome, Policlin Umberto I Univ Hosp, Head & Neck Dept, Ophthalmol Unit, I-00161 Rome, Italy. Ospedaliera Perugia, Cardiol, Perugia, Italy. Osped St i Filippo & Nicola, Unit Vasc Surg, Laquila, Italy. Univ Campania Luigi Vanvitelli, Dept Adv Med & Surg Sci, Div Neurol & Neurophysiopathol, Naples, Italy. Cardarelli Hosp, Clin Neurophysiol Unit, Naples, Italy. IRCCS Human Res Hosp, Otorhinolaryngol Unit, Via Manzoni 56, I-20089 Milan, Italy.

Publication: European Journal of Neurology. 2024

ABSTRACT: BACKGROUND AND PURPOSE 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 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 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 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.