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Acute ophthalmoplegia (without ataxia) associated with anti-GQ1b antibody

From the Department of Neurology (S.-H.L.), College of Medicine, Chonnam National University; Chosun University (G.-H.L.); Seoul National University (J.S.K.); Chonbuk National University (S.-Y.O.); Kosin University (J.K.K.); Dong-A University (J.-K.C.); Inha University (C.-H.Y.); University of Ulsan (J.K.K.); Keimyung University (H.L.); Hallym University (H.-K.S.); and Kyung Hee University (K.C.C.), Korea.

Address correspondence and reprint requests to Dr. Ji Soo Kim, Department of Neurology, College of Medicine, Seoul National University, Department of Neurology, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, Seongnam-si, Gyeonggi-do, 463-707, Korea jisookim{at}snu.ac.kr

Background: Anti-GQ1b antibody has been found in Miller Fisher syndrome (MFS), Guillain-Barré syndrome (GBS) with ophthalmoplegia, Bickerstaff brainstem encephalitis (BBE), and acute ophthalmoplegia without ataxia (AO). The aim of this study was to determine the clinical features of AO associated with anti-GQ1b antibody.

Methods: We retrospectively collected 34 patients with anti-GQ1b antibody syndrome. Of these patients, 31 patients had ophthalmoplegia. The patients with ophthalmoplegia were classified into MFS (n = 13), AO (n = 11), GBS with ophthalmoplegia (n = 6), and BBE (n = 1). We analyzed clinical features and patterns of external and internal ophthalmoplegia of AO, and neuro-ophthalmologic findings were compared with those of other anti-GQ1b syndromes with ophthalmoplegia.

Results: AO was observed in 11 (32.4%) of the 34 patients with anti-GQ1b antibody. External ophthalmoparesis was present in all the patients and included mixed horizontal-vertical (n = 7), pure horizontal (n = 3), and pure vertical gaze palsy (n = 1). Binocular involvement was common, but unilateral ophthalmoparesis was also observed in 27.3%. Other findings included ptosis (n = 5, 45.5%) and internal ophthalmoplegia (n = 6, 54.5%). Other anti-GQ1b antibody syndromes had prominent neurologic signs including ataxia, weakness, and facial palsy in addition to ophthalmoplegia. The patterns of neuro-ophthalmologic findings did not differ between AO and other anti-GQ1b antibody syndromes with ophthalmoplegia.

Conclusions: Acute ophthalmoplegia (AO) commonly occurs in anti-GQ1b antibody syndrome and manifests as various combinations of external and internal ophthalmoplegia. Internal ophthalmoplegia is fairly common and unilateral involvement may occur in AO.

Abbreviations: AO = acute ophthalmoplegia; BBE = Bickerstaff brainstem encephalitis; GBS = Guillain-Barré syndrome; MFS = Miller Fisher syndrome.

Ji Soo Kim was supported by the second stage Brain Korea 21 Project in 2006.

Disclosure: The authors report no disclosures.

Received March 17, 2008. Accepted in final form April 25, 2008.

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