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In 1956, Fisher reported three cases of acute ophthalmoplegia, ataxia, and areflexia subsequent to upper respiratory infectious symptoms and postulated that the disorder is a Guillain-Barré syndrome (GBS) variant because of CSF albuminocytological dissociation identified in one patient (N Engl J Med 1956; 255:57). Some patients with Fisher syndrome progress to GBS, supporting Fisher’s postulation. In this review of the literature, Lo aimed to summarize published information on all aspects of Fisher syndrome, including clinical and laboratory diagnostic criteria, epidemiology, immunology, and treatment. The author emphasizes overlapping features of Fisher syndrome, GBS, and Bickerstaff brainstem encephalitis.