0516-研究生课程-GBS指南解读.pptVIP

武汉大学人民医院 湖北省人民医院 卢 祖 能 lzn196480@126.com ;炎症性神经病的分类;Winer JB. GBS: clinical variants and their pathogenesis. J Neuroimmunol, 2011, 231(1-2): 70-2 ; ;AIDP的免疫机制 从巨噬细胞到T细胞所呈现的细菌蛋白抗原表位。T细胞被激活，渗透到内皮，识别交叉反应性抗原，并且在较低的区段释放细胞因子，细胞因子可激活神经内膜的巨噬细胞。这些过程可释放酶和毒性一氧化氮（NO）自由基，并最终侵入致密的髓鞘。在较高的区段，活化T细胞可释放细胞因子，这有助于B细胞产生抗体；所产生的抗体可穿过受损的血神经屏障，并在远离轴突的雪旺细胞表面与未识别的交叉反应性抗原表位相连接，固定补体，损害雪旺细胞，由此产生髓磷脂的水泡样分解;AMAN、AMSAN和Fisher综合征的免疫机制 细菌的神经节苷脂样抗原表位刺激B细胞诱导抗体，该抗体具有调理交叉反应性轴膜抗原的作用，可固定补体并使得巨噬细胞靶向性侵入轴周间隙，并且阻断传导或引起轴突变性。在Fisher综合征，运动神经末端、突触周围的雪旺细胞也可受到靶向性侵入;Classification of GBS and related disorders and typical antiganglioside antibodies, by pathology根据病理对GBS及其相关病变分类以及典型抗GM抗体;GBS is known to occur at all ages GBS在任何年龄均可发生 Most surveys show a slight peak in late adolescence and young adulthood, and a second peak in the elderly 轻微高峰：青春期晚期、成人早期 另一个高峰：老年期; GBS incidence increased by 20% for every 10-year increase in age GBS 发病率随年龄增长，每增加10岁升高20% The risk of GBS was higher for males than females 男性患GBS的风险高于女性 ;罗瑛, 卢祖能, 等. 2012, 待发表资料;Ho TW, Mishu B, Li CY, et al. Guillain-Barré syndrome in northern China. Brain, 1995, 118 (Pt 3): 597-605.;Seasonal distribution of GBS patients GBS患者发病季节的分布;Preceding infections detected serologically in two large series of patients with Guillain-Barré syndrome两大系列GBS患者中通过血清学检测到的前驱感染; ;Ye Y, Zhu D, Wang K, et al. Clinical and electrophysiological features of the 2007 Guillain-Barré syndrome epidemic in northeast China. Muscle Nerve, 2010, 42(3): 311-4.;J Neurol Neurosurg Psychiatry. 2011 Mar; 82(3):300-5.;Distribution of prodromic events in 3 main GBS subtypes 三种主要亚型的前驱事件分布;Pluta RM, Lynm C, Golub RM. JAMA patient page. Guillain-Barre syndrome. JAMA, 2011, 305(3): 319.;;Diagnosis of typical GBS 典型GBS的诊断;Deep tendon reflexes (DTRs) could be normal or hyperexcitable during the entire clinical course in approximately 10% of GBS patients This possibility should be added in the diagnostic criteria for GBS to avoid delays in diagnosis and effective treatment to t