The immunopathogenesis of Miller Fisher syndrome.
Over the past decade, remarkable progress has been made in our understanding of the pathogenesis of Miller Fisher syndrome (MFS), a clinical variant of Guillain Barré syndrome (GBS). MFS comprises the clinical triad of ataxia, areflexia and ophthalmoplegia. It is associated with acute-phase IgG antibodies to GQ1b and GT1a gangliosides in over 90% of cases which are highly disease specific. Like GBS, MFS is a post-infectious syndrome following diverse infections, but particular attention has been paid to its association with Campylobacter jejuni enteritis. Serostrains of C. jejuni isolated from infected patients bear ganglioside-like epitopes in their lipopolysaccharide core oligosaccharides, which elicit humoral immune responses exhibiting molecular mimicry with GQ1b/GT1a gangliosides. These antibodies are believed to be the principal cause of the syndrome and physiological studies aimed at proving this have focused on the motor-nerve terminal as a potential site of pathogenic action. This review describes these findings and formulates a pathogenesis model based on our current state of knowledge.
Willison
H J
HJ
University Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, Scotland, UK. gora13@udcf.gla.ac.uk
O'Hanlon
G M
GM
D000818:Animals; D001327:Autoimmune Diseases / Q000097:blood; D002855:Chromatography, Thin Layer; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007239:Infections / Q000150:complications; D019846:Miller Fisher Syndrome / Q000097:blood / Q000276:immunology*; D009844:Oligosaccharides / Q000276:immunology; D010525:Peripheral Nerves / Q000276:immunology; D010641:Phenotype; D051381:Rats
D005732:Gangliosides; D009844:Oligosaccharides; C039129:GQ1b ganglioside