CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1669821
This article is part of the Research TopicCase Reports in Autoimmune and Autoinflammatory Disorders: Volume IIView all 23 articles
Unilateral Ophthalmoplegia in Anti-GQ1b Antibody Syndrome: Case Report and Systematic Literature Review
Provisionally accepted
- Zhejiang Taizhou Hospital, Taizhou, China
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Abstract Introduction: Anti-GQ1b antibody syndrome encompasses immune-mediated neuropathies targeting ganglioside GQ1b, classically presenting as Miller Fisher syndrome (MFS) with the triad of ophthalmoplegia, ataxia, and areflexia. While bilateral ophthalmoplegia is typical, unilateral presentations represent a recognized variant phenotype. Methods: We report a case of a 17-year-old male with unilateral complete oculomotor nerve palsy, confirmed by positive serum anti-GQ1b IgG antibodies. We also conducted a literature review (PubMed, 2000-2025) identifying 17 additional cases of anti-GQ1b associated unilateral ophthalmoplegia, summarizing clinical features, investigations, management, and outcomes for all 18 patients. Results: Among the 18 patients, a male predominance was observed (13 males, 5 females), with a median age of 31 years (range 10-68). Most patients (17/18, 94.4%) reported a preceding illness. Unilateral external ophthalmoplegia was universal, most commonly affecting adduction (55.6%) and vertical gaze (55.6%). Internal ophthalmoplegia (IO) occurred in 6 cases (33.3%), including 4 unilaterally. Cerebrospinal fluid (CSF) protein was mildly elevated (23-97 mg/dL) in 7 of 18 cases. Treatments varied and prognosis was uniformly favorable, with most patients recovering within 3 months. Conclusion: Unilateral ophthalmoplegia, particularly when complicated by ipsilateral internal ophthalmoplegia, constitutes a distinct regional variant of anti-GQ1b antibody syndrome. Early serological testing for anti-GQ1b antibodies is key to diagnosis, especially in patients with antecedent infection.
Keywords: Unilateral ophthalmoplegia, Anti-GQ1b antibody syndrome, case report, Systematic Literature Review, Miller Fisher Syndrome
Received: 20 Jul 2025; Accepted: 25 Sep 2025.
Copyright: © 2025 Pan, Zheng, Yu, Jiang and Zhou. 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:
Huihua Jiang, jianghh@enzemed.com
Yuanlin Zhou, zyl@enzemed.com
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