Acute vestibular syndrome associated with anti-GQ1b antibody

Neurology. 2019 Sep 10;93(11):e1085-e1092. doi: 10.1212/WNL.0000000000008107. Epub 2019 Aug 9.


Objectives: To delineate the clinical features and ocular motor findings in acute vestibular syndrome (AVS) associated with anti-GQ1b antibodies.

Methods: We reviewed 90 patients with positive serum anti-GQ1b antibody in association with various neurological syndromes at Seoul National University Bundang Hospital from 2004 to 2018. The diagnoses included typical Miller Fisher syndrome (n = 31), acute ophthalmoplegia without ataxia (n = 27), Guillain-Barre syndrome with ophthalmoplegia (n = 18), AVS (n = 11), and Bickerstaff brainstem encephalitis (n = 3). Of them, the 11 patients with AVS formed the basis of this study. We also conducted a systematic review on AVS reported in association with anti-GQ1b antibody.

Results: Patients with AVS showed various ocular motor findings that included head-shaking nystagmus (n = 6), spontaneous nystagmus (n = 5), gaze-evoked nystagmus (n = 5), central positional nystagmus (n = 3), canal paresis (n = 2), and abnormal head-impulse tests (n = 1) without any internal or external ophthalmoplegia. Compared to those with other subtypes, patients with AVS mostly showed normal deep tendon reflexes (8 of 11 [73%], p = 0.002) and normal results on nerve conduction studies (4 of 4 [100%], p = 0.010). The clinical and laboratory findings resolved or improved markedly in all patients within 6 months of symptom onset. Systematic review further identified 7 patients with AVS and positive serum anti-GQ1b antibody who showed various ocular motor findings, including gaze-evoked nystagmus, saccadic dysmetria, central positional nystagmus, and ocular flutter or opsoclonus.

Conclusion: Anti-GQ1b antibody may cause acute vestibulopathy by involving either the central or peripheral vestibular structures. AVS may constitute a subtype of anti-GQ1b antibody syndrome.

Publication types

  • Research Support, Non-U.S. Gov't
  • Systematic Review

MeSH terms

  • Acute Disease
  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Autoantibodies / blood*
  • Biomarkers / blood
  • Child
  • Female
  • Follow-Up Studies
  • Gangliosides / blood*
  • Humans
  • Male
  • Middle Aged
  • Vestibular Diseases / blood*
  • Vestibular Diseases / diagnosis*
  • Young Adult


  • Autoantibodies
  • Biomarkers
  • Gangliosides
  • GQ1b ganglioside