gq1b-ganglioside and Infections

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.

Topics: Animals; Autoimmune Diseases; Chromatography, Thin Layer; Gangliosides; Humans; Infections; Miller Fisher Syndrome; Oligosaccharides; Peripheral Nerves; Phenotype; Rats

The authors reviewed clinical profiles and laboratory findings for 100 cases of abducens nerve paresis without impairment of the other cranial nerves, limb weakness, and ataxia throughout the clinical course. Review of the medical records of 9300 patients referred to our neuoroimmunological laboratory for serum anti-ganglioside antibody testing. Information was obtained from each primary physician on symptoms of preceding infection; initial symptoms; neurological signs during the illness; the clinical course; treatment provided; and outcome. Isolated abducens nerve paresis was present in 100 patients and bilateral paresis in 29. Tentative diagnoses made by the primary physicians on request of anti-ganglioside antibody testing were abducens nerve palsy (n = 68), Fisher syndrome (n = 14), acute ophthalmoparesis without ataxia (n = 14). Symptoms of infection anteceded in 63. Tendon reflexes were absent or decreased in 27. Distal paresthesias were experienced by seven. Serum anti-GQ1b antibody was positive in 25. These findings suggest that some cases of isolated abducens nerve palsy can be categorized as a regional variant of Guillain-Barré syndrome or mild form of Fisher syndrome.

Topics: Abducens Nerve; Abducens Nerve Diseases; Adolescent; Adult; Aged; Aged, 80 and over; Autoantibodies; Child; Child, Preschool; Diagnosis, Differential; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Infant; Infections; Male; Middle Aged; Miller Fisher Syndrome; Plasmapheresis; Predictive Value of Tests; Reflex, Abnormal; Steroids

To describe two cases with bilateral abducens nerve paresis associated with serum anti-GQ1b IgG antibody.. Case reports.. Two boys, aged 12 and 10 years, experienced acute onset of diplopia after preceding symptoms and signs of infection. In both boys, examination showed only bilateral abducens nerve paresis. Although routine laboratory data and magnetic resonance imaging demonstrated no pathologic findings, titer of anti-GQ1b IgG antibody in the sera of both patients was increased. Diplopia and signs of bilateral abducens nerve paresis disappeared in 6 weeks without any specific treatment.. The anti-GQ1b IgG antibody in the sera of both patients probably contributed to the bilateral abducens nerve paresis.

Topics: Abducens Nerve Diseases; Antibodies; Child; Diplopia; Eye Movements; Gangliosides; Humans; Immunoglobulin G; Infections; Male; Remission, Spontaneous