Incomplete Presentations in Typical Chronic Inflammatory Demyelinating Polyneuropathy: A Single‐Center, Retrospective Study

Abstract

Introduction/Aims: Incomplete forms of typical chronic inflammatory demyelinating polyneuropathy (CIDP) have recently been described, but their frequency and clinical characteristics are uncertain. This study aimed to describe a cohort of patients with incomplete typical CIDP. Methods: We retrospectively analyzed 64 consecutive treatment-naïve patients with CIDP. Phenotypes were classified based on detailed motor examinations, and clinical, electrophysiological, and therapeutic characteristics were compared. Results: Nineteen (30%) subjects with typical CIDP presented with an incomplete phenotype; 12 (63.2%) exhibited a proximal arm-sparing pattern, 3 (15.8%) a distal arm-sparing pattern, 3 (15.8%) a pure paraparetic form, and 1 (5.2%) had a pure proximal form. In cases without full motor recovery, 11 (68.8%) maintained their original phenotype, while the rest transitioned to the complete (18.8%) or to another incomplete form (12.5%) due to involvement of previously unaffected segments. Subjects with incomplete typical CIDP had milder pre-treatment disability and weakness compared to those with the complete form, while other clinical and electrodiagnostic features were comparable. As opposed to the complete form, disability in incomplete typical CIDP at diagnosis showed no correlation with muscle strength. Discussion: Incomplete forms were observed in nearly one-third of subjects with typical CIDP. Incomplete typical CIDP represents a milder form of complete typical CIDP; however, its other disease characteristics, including treatment response, are similar, highlighting the importance of its proper prompt recognition as CIDP. Impairments beyond motor weakness, such as more diffuse proprioceptive loss, might play a role in the disability of patients with incomplete typical forms of CIDP.

Publication DOI: https://doi.org/10.1002/mus.28419
Divisions: College of Health & Life Sciences
Additional Information: Copyright © 2025 The Author(s). Muscle & Nerve published by Wiley Periodicals LLC. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Uncontrolled Keywords: chronic inflammatory demyelinating polyneuropathy,course,incomplete,phenotype,response,treatment,typical
Publication ISSN: 1097-4598
Last Modified: 01 May 2025 08:43
Date Deposited: 23 Apr 2025 08:58
Full Text Link:
Related URLs: https://onlinel ... .1002/mus.28419 (Publisher URL)
PURE Output Type: Article
Published Date: 2025-04-22
Published Online Date: 2025-04-22
Accepted Date: 2025-04-14
Authors: Min, Young Gi
Ahmed, Irad
Englezou, Christina
Rajabally, Yusuf

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License: Creative Commons Attribution


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