Abstract:Objective: There is no report on Bickerstaff's brainstem encephalitis (BBE) patients in China. We here report the first case of BBE in China. Methods: Clinical features, results of electromyography, electroencephalography (EEG), magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) examination were studied to clarify the characteristics of this syndrome. Results: A 44-year-old man presented himself at our inpatient department with somnolence and dizziness as his initial symptoms. He developed multiple cranial nerves paralysis especially internal and external ophthalmoplegia, ataxia and tetraparesis within 1 week. His condition rapidly deteriorated, and he experienced coma. Electromyography showed indications of peripheral nerve dysfunction, electroencephalography revealed loss of basic rhythm, MRI demonstrated high-intensity abnormalities on T 2 -weighted images of medulla oblongata, and CSF albuminocytological dissociation was defined abnormally as high protein. Ten months later, he almost completely recovered. Conclusion: BBE, fisher syndrome (FS) and Guillain-Barré syndrome (GBS) are similar clinically; BBE and FS were proposed to be the variant of GBS.