Bickerstaff encephalitis: a comprehensive narrative review of pathophysiology, clinical features, and global health...

Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune neurological disorder first identified in 1951. It shares clinical and immunological features with Guillain-Barré syndrome (GBS) and Miller-Fisher syndrome (MFS). BBE, characterized by brainstem dysfunction, often after infection or vaccination, is characterized by immunopathogenesis involving microbial antigens and neural gangliosides, with anti-GQ1b IgG antibodies as key biomarkers.

A comprehensive narrative review was conducted by searching PubMed, Cochrane, Global Health Library, and Google Scholar for relevant literature up to 2025. Keywords included, "BBE syndrome," "Miller Fisher syndrome," "GBS syndrome," "anti-GQ1b antibody syndrome," and "autoimmune encephalitis." The Scale for the Assessment of Narrative Review Articles (SANRA) was utilized to ensure manuscript quality.

Most cases of BBE have positive anti-GQ1b IgG antibodies, and the condition usually manifests as ophthalmoplegia, ataxia, and reduced consciousness. Cerebrospinal fluid examination frequently shows higher protein levels, and MRI results may reveal midbrain lesions. BBE, GBS, and MFS all have a pathogenic spectrum, which points to a continuum of post-infectious autoimmunity. Intravenous immunoglobulin (IVIG) and plasmapheresis are the main treatment approaches; both have been shown to be effective in reducing symptoms and hastening recovery. The use of corticosteroids as an adjuvant treatment is possible. Pediatric patients tend to heal more quickly, and the prognosis is generally good, however severe instances may necessitate intensive care.

BBE is a rare disorder requiring early recognition and immunotherapy. Detection of anti-GQ1b IgG antibodies differentiates it from other neurological conditions. Further research is needed for standardization and improved outcomes.