Background: Bickerstaff's brainstem encephalitis continues to pose a diagnostic and treatment challenge since the original descriptions by Bickerstaff and Miller-Fisher. The clinical syndrome overlaps with AIDP and MFS, but is accompanied by decreased level of consciousness not attributable to other causes, and the variable presence of long-tract signs. Methods: The methods in this study include the case presentation with autopsy findings of an elderly male presented with progressive weakness and impairment of consciousness and the literature review. Results: Examination revealed areflexia and loss of most brainstem reflexes. Some improvement occurred after several weeks in the ICU, prior to death from pulmonary embolism. Pathologic specimens were similar to others in the literature, with inflammatory changes in nerve roots and brainstem. Conclusions: The above findings led us to conclude that Bickerstaff's brainstem encephalitis remains a clinical diagnosis despite advances in electrophysiologic testing and neuroimaging. BBE likely represents part of a spectrum, overlapping with AIDP and MFS. Immunomodulation may be helpful in shortening the clinical course. © 2012 Springer Science+Business Media, LLC.