Prevalence and patterns of cranial nerve involvement in CIDP, autoimmune nodopathy, MMN, and anti-MAG neuropathy: a multicenter Korea/UK study of 582 patients
Min, Young Gi Kim, Hyunjin Han, Hee Jo Yoon, Byeol-A Kim, Jong Kuk Ju, Woohee Choi, Seok-Jin Kim, Sung-Min Kim, Ki Hoon Kwon, Young Nam
Min, Young Gi
Kim, Hyunjin
Han, Hee Jo
Yoon, Byeol-A
Kim, Jong Kuk
Ju, Woohee
Choi, Seok-Jin
Kim, Sung-Min
Kim, Ki Hoon
Kwon, Young Nam
Abstract
Background: Cranial nerve involvement is a well-recognized feature in Guillain-Barré syndrome (GBS) but remains less well understood in chronic forms of autoimmune neuropathies. Earlier studies of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were conducted before updated diagnostic criteria and the recognition of autoimmune nodopathy (AN), which may limit the interpretation of their findings.
Methods: We retrospectively analyzed 582 patients with chronic autoimmune neuropathies-CIDP (n = 431), multifocal motor neuropathy (MMN) (n = 64), anti-myelin-associated glycoprotein (MAG) neuropathy (n = 54), and AN (n = 33)-from 4 Korean and 1 UK centers. Patients with cranial nerve involvement were identified and described. CIDP patients with cranial nerve involvement (cranial+ CIDP) were compared with those without (cranial- CIDP).
Results: Cranial nerve involvement was observed in 8.8% (38/431) of CIDP and 24.2% (8/33) of AN patients but was absent in MMN (0/64) and anti-MAG neuropathy (0/54). Facial palsy was overall the most common manifestation (CIDP: 45%, AN: 50%). Patients with AN more frequently exhibited bilateral optic neuropathy (50%) and facial diplegia (38%), while CIDP patients more often showed trigeminal neuropathy and oculomotor nerve palsy (both 32%). Compared with cranial- CIDP, cranial+ CIDP patients were more often younger, of variant subtypes (especially multifocal), presented (sub)acutely with preceding infection/vaccination, followed by relapsing-remitting rather than progressive courses, and achieved greater improvement despite greater pre-treatment disability.
Conclusions: Cranial nerve involvement serves as a diagnostic clue in chronic autoimmune neuropathies, particularly in identifying AN and CIDP. Cranial+ CIDP appears to represent a distinct subset with partial overlap to GBS, suggesting unique underlying mechanisms.
MIDER Authors
Affiliations
Seoul National University; Severance Hospital; Yonsei University; Asan Medical Center; University of Ulsan; Dong-A University Hospital; Dong-A University; Chung-Ang University Hospital; Chung-Ang University; Seoul National University Hospital; Aston University; University Hospitals Birmingham NHS Foundation Trust
Date
2026-02-09
Type
Article
Collections
Citation
Min YG, Kim H, Han HJ, Yoon BA, Kim JK, Ju W, Choi SJ, Kim SM, Kim KH, Kwon YN, Kim SW, Lee EJ, Lim YM, Nazeer KK, Rajabally YA, Shin HY, Sung JJ. Prevalence and Patterns of Cranial Nerve Involvement in CIDP, Autoimmune Nodopathy, MMN, and Anti-MAG Neuropathy: A Multicenter Korea/UK Study of 582 Patients. Eur J Neurol. 2026 Feb;33(2):e70512. doi: 10.1111/ene.70512.