ABSTRACT
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.
Subject(s)
Gangliosides/immunology , Guillain-Barre Syndrome/immunology , Miller Fisher Syndrome/immunology , Ophthalmoplegia/immunology , Diagnosis, Differential , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/physiopathology , Guillain-Barre Syndrome/therapy , Humans , Immunotherapy , Magnetic Resonance Imaging , Miller Fisher Syndrome/diagnosis , Miller Fisher Syndrome/physiopathology , Miller Fisher Syndrome/therapy , Ophthalmoplegia/diagnosis , Ophthalmoplegia/physiopathology , Ophthalmoplegia/therapy , Peripheral Nerves/physiopathologyABSTRACT
A fair portion of the diagnostic practice of the neurologist and ophthalmologist involves the deciphering of visual symptoms in patients known to have migraine, and many who at the time of their initial presentation do not. The most common visual disorder in this category, migraine with aura, has been studied extensively, but no conclusive evidence has been proven linking the visual aura, believed to result from a spreading depression, to the subsequent development of the cephalgia. Vasospasm remains controversial in the pathophysiology of migraine, but may play a role in patients who suffer more permanent or complicated neurovisual dysfunction. This paper reviews some of the current thoughts on the subject, and hopefully will stimulate the reader to explore the complex issue of this common malady.
