Willison H J, O'Hanlon G M
University Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, Scotland, UK.
J Neuroimmunol. 1999 Dec;100(1-2):3-12. doi: 10.1016/s0165-5728(99)00213-1.
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.
在过去十年中，我们对吉兰-巴雷综合征（GBS）的临床变体——米勒-费希尔综合征（MFS）的发病机制有了显著进展。MFS包括共济失调、无反射和眼肌麻痹三联征。超过90%的病例中，它与抗GQ1b和GT1a神经节苷脂的急性期IgG抗体相关，这些抗体具有高度的疾病特异性。与GBS一样，MFS是多种感染后的感染后综合征，但人们特别关注它与空肠弯曲菌肠炎的关联。从感染患者中分离出的空肠弯曲菌血清菌株在其脂多糖核心寡糖中带有神经节苷脂样表位，可引发体液免疫反应，表现出与GQ1b/GT1a神经节苷脂的分子模拟。这些抗体被认为是该综合征的主要病因，旨在证明这一点的生理学研究将运动神经末梢作为潜在的致病作用部位。本综述描述了这些发现，并根据我们目前的知识状态制定了发病机制模型。
