To explore the diagnostic features of Bickerstaff brainstem encephalitis (BBE) with positive anti-GQ1b antibody.

The clinical data of 3 BBE cases with positive anti-GQ1b antibody diagnosed by the Neurology Department of Guangdong Sanjiu Brain Hospital from February 2016 to May 2019 were retrospectively analyzed. Diagnostic features of BBE with positive anti-GQ1b antibody were summarized, and relevant literatures were reviewed.

The 3 patients were all female, with the age of 16, 19, 49 years old respectively, and all of them had a history of infection before the onset. All 3 cases had onset of ataxia, 2 cases had limb weakness at the onset, and 1 case developed consciousness disorder and limb weakness after aggravation. On physical examination, 3 patients had ophthalmoplegia, and 2 patients had positive pyramidal tract signs. Auxiliary examination: 3 patients presented positive of anti-GQ1b antibodies, 3 patients showed no obvious abnormalities in brain magnetic resonance imaging (MRI) scan and enhancement, 2 patients had abnormal electromyogram evoked potentials, and 1 patient was normal.

BBE is a syndrome that mainly manifests as acute ophthalmoplegia, ataxia accompanied by disturbance of consciousness and/or positive pyramidal tract signs. Thorough understanding of associated clinical features and serum antibody, imaging and nerve electrophysiological examination are helpful in achieving at the correct diagnosis.