Department of Neurology, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.
Adv Neurobiol. 2023;29:479-495. doi: 10.1007/978-3-031-12390-0_16.
Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) are acute immune-mediated neuropathies, often preceded by an infection. Anti-glycolipid antibodies are frequently detected in patients' sera in the acute-phase. In particular, IgG anti-GQ1b antibodies are positive in as high as 90% of MFS cases. Anti-glycolipid antibodies are useful for the diagnosis of GBS and MFS. In addition, those antibodies may be directly involved in the pathogenetic mechanisms by binding specifically to the regions where the target glycolipid antigen is densely localized. This was proven by the development of animal models of anti-glycolipid antibody-mediated neuropathies. The presence of antibodies that specifically recognize a new conformational epitope formed by two gangliosides (ganglioside complex) in the acute-phase sera of some GBS patients suggested existence of a carbohydrate-carbohydrate interaction between glycolipids. Further intensive research is needed to clarify this point.
格林-巴利综合征（GBS）和米勒费舍尔综合征（MFS）是急性免疫介导的神经病，常由感染引起。在急性期，患者血清中常检测到抗神经节苷脂抗体。特别是在高达 90%的 MFS 病例中，IgG 抗-GQ1b 抗体呈阳性。抗神经节苷脂抗体对 GBS 和 MFS 的诊断很有用。此外，这些抗体可能通过特异性结合靶神经节苷脂抗原密集定位的区域而直接参与致病机制。这一点通过抗神经节苷脂抗体介导的神经病动物模型的发展得到了证实。在一些 GBS 患者急性期血清中存在一种新型构象表位的抗体，该表位由两种神经节苷脂（神经节苷脂复合物）形成，这表明神经节苷脂之间存在糖-糖相互作用。需要进一步深入研究来阐明这一点。
