[Antiganglioside antibodies in the pathogenesis of autoimmune neuropathies].
Antiganglioside antibodies are frequently detected in sera from patients with autoimmune neuropathies, such as Guillain-Barré syndrome, Miller Fisher syndrome, IgM paraproteinemic neuropathy, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. In the acute phase sera from GBS patients, antiganglioside antibodies are detected in 60-70%. Ganglioside antigens recognized by serum antibodies are varied from case to case. IgG antibody against GQ1b ganglioside is specifically raised in sera from patients with Miller Fisher syndrome and Guillain-Barré syndrome with ophthalmoplegia. That antibody may bind to the paranodal myelin of oculomotor, trochlear and abducens nerves, where GQ1b ganglioside is specifically localized, to cause ophthalmoplegia. IgM M-protein which recognizes the disialosyl residue of GD1b is specifically associated with sensory ataxic neuropathy. The IgM M-protein may bind to the primary sensory neurons, where GD1b ganglioside is localized, to cause sensory disturbance. After we confirmed the localization of GD1b in the rabbit primary sensory neurons, we sensitized rabbits with GD1b and induced sensory ataxic neuropathy in them. This is the first established animal model of autoimmune neuropathy induced by sensitization with ganglioside. Some antiganglioside antibodies may determine the clinical phenotype of neuropathy by binding specifically to the ganglioside antigens which have unique localization.
Kusunoki
S
S
Department of Neurology, School of Medicine, University of Tokyo.
D000818:Animals; D001323:Autoantibodies / Q000032:analysis*; D003711:Demyelinating Diseases / Q000209:etiology* / Q000276:immunology; D005732:Gangliosides / Q000276:immunology*; D006801:Humans; D007075:Immunoglobulin M; D019846:Miller Fisher Syndrome / Q000209:etiology* / Q000276:immunology; D016472:Motor Neuron Disease / Q000276:immunology; D010265:Paraproteinemias / Q000276:immunology; D011129:Polyradiculoneuropathy / Q000209:etiology* / Q000276:immunology; D011817:Rabbits
D001323:Autoantibodies; D005732:Gangliosides; D007075:Immunoglobulin M