会计学 1 格林巴利综合征及相关周围神经病 Guillain-Barré Introduction Guillain Barre 综合征的特点是四肢对称性 paresthesias generalized weakness areflexia Pathogenesis of GBS not yet fully understood and thinking is GBS may not be a single disease but a variety of acute neuropathies Pathogenesis of GBS not yet fully understood and thinking is with a number of related immune-mediated pathogenetic mechanisms Most common immunopathologic finding: endoneurial inflammation in spinal nerves roots, distal nerve segments, or around potential nerve entrapment sites. Pathogenesis of GBS not yet fully understood and thinking is with a number of related immune-mediated pathogenetic mechanisms B. Target antigens appear to be common to the axon myelin sheath or both. The exact antigens, mechanism of injury somewhat unclear. 病因 GBS是一种自身免疫性疾病 病人在发病前有感染史, 包括EB病毒,巨细胞病毒,支原体,弯曲杆菌等 GBS是一个脱髓鞘的过程 近年发现,空肠弯曲杆菌或痢疾之后的GBS能表现为轴突变性型 有时,外科手术急剧的诱发因子 脱髓鞘 流行病学 发病率: 1 to 2/100,000/year 性 别: 男性: 女性 = : 1 发 病: 双高峰, 儿童 & > 55 岁 遗传高危因子: FcγRIIa-H131 纯合性等位基因(vs R131) 较健康人群普遍 较其它基因型发病程度更严重 免受狼疮性肾炎的相同等位基因