The discovery of the association of the anti-GQ1b IgG antibody with the 
postinfectious clinical syndromes of ophthalmoplegia, ataxia, and areflexia 
helped house the phenotypes of the Miller Fisher syndrome (MFS), atypical MFS, 
Guillain-Barré syndrome with ophthalmoplegia, and Bickerstaff's brainstem 
encephalitis under one roof. The neuro-ophthalmologic signs classically 
predominate and may vary from case to case, but they maintain clinically 
recognizable patterns that assist with the diagnosis. The identification of a 
common lipopolysaccharide on the plasma membrane in human cranial and peripheral 
nerves at the GQ1b epitope and on infectious particles of bacteria and viruses 
(ie, Campylobacter jejuni) demonstrates molecular mimicry. The high frequency of 
oculomotor dysfunction is partially explained by the tissue ganglioside 
concentration and distribution and the attraction of antibody-stimulating 
complement activation. Current experimental treatment targets antibody removal 
and neutralization and prevents membrane attack complex formation through 
deactivation of complement. This article aims to bring together the historically 
disparate opinions on the origins of these syndromes as either a purely 
peripheral nervous system or central nervous system dysfunction, highlight the 
clinical neuro-ophthalmologic signs, discuss some of the biology of the 
anti-GQ1b antibody, and review imaging abnormalities and treatment of this 
fascinating disorder.