ORIGINAL RESEARCH article
Front. Neurol.
Sec. Neuromuscular Disorders and Peripheral Neuropathies
Clinical features of Chronic Inflammatory Demyelinating Polyneuropathy: a single-center experience with 33 patients
Kumamoto University, Kumamoto, Japan
Select one of your emails
You have multiple emails registered with Frontiers:
Notify me on publication
Please enter your email address:
If you already have an account, please login
You don't have a Frontiers account ? You can register here
Abstract
Abstract Background. Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy with heterogeneous clinical presentations. Although several clinical subtypes, including typical and variants, have been described, evidence regarding their distribution and treatment outcomes remains limited, particularly for CIDP variants. Methods. We retrospectively analyzed 33 consecutive patients diagnosed with CIDP at Kumamoto University Hospital between January 2020 and March 2025. Clinical data included demographic characteristics, disease duration, subtype classification, electrophysiological and cerebrospinal fluid (CSF) findings, imaging findings, treatment details, and treatment responsiveness. Subtypes were classified according to the 2021 EAN/PNS diagnostic criteria. Treatment responsiveness was defined as objective improvement confirmed by at least two neurologists. Results. Among the 33 patients, 36.4% had typical CIDP and 63.6% had CIDP variants, including distal (39.4%), multifocal (15.2%), motor (3.0%), and sensory (6.0%) subtypes. Distal CIDP was the most frequent subtype. Patients with multifocal CIDP experienced the longest diagnostic delays (mean, 4.6 years) due to fewer demyelinating findings on electrophysiological studies. The MRC sum score was lowest in typical CIDP, suggesting greater disease severity. Nomura et al. 3 Intravenous immunoglobulin (IVIg) demonstrated high efficacy across all subtypes, including multifocal CIDP, in contrast to previous reports of lower responsiveness. During a mean follow-up period of 5.1 years, 78.8% of patients required maintenance therapy, most commonly IVIg and corticosteroids. No changes in clinical subtypes were observed during follow-up. Conclusions. In this single-center study, variants outnumbered typical CIDP, reflecting the case mix at a specialized tertiary referral center. Multifocal CIDP showed the longest diagnostic delays. IVIg demonstrated high efficacy across subtypes, including multifocal CIDP, contrasting with previous reports. These findings highlight the importance of improving diagnostic accuracy and establishing individualized long-term treatment strategies for CIDP.
Summary
Keywords
cidp, CIDP variants, efgartigimod, IVIg, Plasma Exchange, SCIG, steroid, typical CIDP
Received
11 November 2025
Accepted
16 February 2026
Copyright
© 2026 Nomura, Misumi and Ueda. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
*Correspondence: Toshiya Nomura
Disclaimer
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.