{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T15:21:18Z","timestamp":1778944878367,"version":"3.51.4"},"reference-count":22,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T00:00:00Z","timestamp":1760054400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>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 identifying 17 additional cases of anti-GQ1b associated unilateral ophthalmoplegia, summarizing clinical features, investigations, management, and outcomes for all 18 patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>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\u201397 mg\/dL) in 7 of 18 cases. Treatments varied and prognosis was uniformly favorable, with most patients recovering within 3 months.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2025.1669821","type":"journal-article","created":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T05:29:12Z","timestamp":1760074152000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Unilateral ophthalmoplegia in anti-GQ1b antibody syndrome: case report and systematic literature review"],"prefix":"10.3389","volume":"16","author":[{"given":"Juyuan","family":"Pan","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ningyu","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dan","family":"Yu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huihua","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuanlin","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,10,10]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2023.1250774","article-title":"Miller Fisher syndrome: an updated narrative review","volume":"14","author":"Noioso","year":"2023","journal-title":"Front Neurol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-8993(96)01123-7","article-title":"Ganglioside composition of the human cranial nerves, with special reference to pathophysiology of Miller Fisher syndrome","volume":"745","author":"Chiba","year":"1997","journal-title":"Brain Res"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000324266.95814.74","article-title":"Acute ophthalmoplegia (without ataxia) associated with anti-GQ1b antibody","volume":"71","author":"Lee","year":"2008","journal-title":"Neurology"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1159\/000326340","article-title":"Unilateral abducens nerve palsy as an early feature of multiple mononeuropathy associated with anti-GQ1b antibody","volume":"3","author":"Kinno","year":"2011","journal-title":"Case Rep Neurol"},{"key":"B5","doi-asserted-by":"publisher","first-page":"196","DOI":"10.1016\/S0161-6420(00)00420-6","article-title":"Acute ophthalmoparesis (without ataxia) associated with anti-GQ1b IgG antibody: clinical features","volume":"108","author":"Yuki","year":"2001","journal-title":"Ophthalmology"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000160399.08456.7C","article-title":"Antecedent infections in Fisher syndrome: a common pathogenesis of molecular mimicry","volume":"64","author":"Koga","year":"2005","journal-title":"Neurology"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1038\/cmi.2017.142","article-title":"Guillain-Barre syndrome, transverse myelitis and infectious diseases","volume":"15","author":"Rodriguez","year":"2018","journal-title":"Cell Mol Immunol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1186\/1471-2164-8-359","article-title":"Comparative genomic analysis of Campylobacter jejuni associated with Guillain-Barre and Miller Fisher syndromes: neuropathogenic and enteritis-associated isolates can share high levels of genomic similarity","volume":"8","author":"Taboada","year":"2007","journal-title":"BMC Genomics"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-018-9161-8","article-title":"Characteristics of single ocular motor nerve palsy associated with anti-GQ1b antibody","volume":"266","author":"Choi","year":"2019","journal-title":"J Neurol"},{"key":"B10","doi-asserted-by":"publisher","first-page":"3930","DOI":"10.3390\/jcm9123930","article-title":"Pediatric miller fisher syndrome; 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