格林巴利吉兰巴雷综合征综合征.ppt

1、吉兰-巴雷综合征,王宗文 上海天佑医院神经内科 2019年8月23日,定 义,吉兰-巴雷综合征（GBS）是一类免疫介导的急性炎性周围神经病。 临床特征为急性起病，临床症状多在2周左右达到高峰，表现为多发神经根及周围神经损害，常有脑脊液蛋白-细胞分离现象，多呈单时相自限性病程， 静脉注射免疫球蛋白（IVIg）和血浆交换（PE）治疗有效。,Pathogenesis,Infection: C jejuni , cytomegalovirus, Epstein-Barr virus, inuenza A, Mycoplasma pneumoniae, Haemophilus inuenzae, hep

2、atitis (A, B, and E) and Zika virus. Trauma: Surgery and trauma may predispose patients to GBS (more likely in patients with prior malignant or autoimmune disorders) but is exceedingly rare. 创伤相关性吉兰-巴雷综合征的诊断首先应该符合吉兰-巴雷综合征的诊断标准，同时符合创伤后1个月内发病，并排除其他诱因所致。创伤类型包括足外伤、颅脑创伤、骨折、阑尾切除术、剖宫产术、开胸术、脊柱手术、开颅术等。,Curre

3、nt understanding of Guillain-Barr syndrome pathogenesis and clinical variants. In demyelinating Guillain-Barr syndrome, unequivocal antigens have yet to be identied but are inferred by complement activation, myelin destruction, and cleanup by macrophages. In axonal and Miller Fisher variants, specic

4、 gangliosides (GM1, GD1a, GQ1b) are targeted by immunoglobulins and share antigenic epitopes with various bacterial and viral antigens. These antigenic targets are at nodal structures, at roots, and located at the end organs. In Miller Fisher syndrome, the GQ1b antigen also exists within the brain s

5、tem. In this variant, the macrophages clean up the axon debris and come in from the nodes.,分 类,急性炎性脱髓鞘性多发神经根神经病（acute inflammatory demyelinating polyneuropathies, AIDP） 急性运动轴索性神经病（acute motor axonal neuropathy, AMAN） 急性运动感觉轴索性神经病（acute motor-sensory axonal neuropathy, AMSAN） Miller Fisher综合征（Miller

6、Fisher syndrome, MFS） 急性泛自主神经病（acute panautonomic neuropathy） 急性感觉神经病（acute sensory neuropathy, ASN）,Guillain and Barr,1916年George Guillain, Jean-Alexandre Barr, Andr Strohl在索姆战役后（WW I）的部队医院内发现了2名急性起病、快速进展，肢体无力的患者，并对此进行描述和总结： 肢体无力 、腱反射丧失和蛋白细胞分离。,Miller Fisher,Charles Miller Fisher, usually known as

7、C. Miller Fisher (December 5, 1913, Waterloo, Ontario April 14, 2012, Albany, New York) was a neurologist whose notable contributions include the first detailed descriptions of lacunar strokes, the identification of transient ischemic attacks as stroke precursors, the identification of the link betw

8、een carotid atherosclerosis and stroke, and the description of a variant form of GuillainBarr syndrome which bears his name.-From Wikipedia,教材中的讲述,八年制第二版 2010,教材中的讲述,本科第7版 吉兰-巴雷综合征 主要参照指南（2010），但未将指南中第5类泛自主神经病纳入教材,Differential diagnosis of GBS,GBS的鉴别：脊髓炎、周期性麻痹、多发性肌炎、脊髓灰质炎、重症肌无力、急性横纹肌溶解症、白喉神经病、莱姆病、卟啉

9、病周围神经病、癔症性瘫痪以及中毒性周围神经病，如重金属、药物、肉毒毒素中毒等。 MFS的鉴别：与CQ1b 抗体相关的Bickerstaff脑干脑炎、急性眼外肌麻痹、脑干梗死、脑干出血、NMO、MS、MG等。,2014年8月 NAT REV NEUROL发表GBS分类专家组对GBS和MFS的新分类和诊断标准，将GBS、MFS和Bickstaffer 脑干脑炎（BBE）作为一个疾病谱，并按照临床受累部位对此疾病谱中的表型进行了分类，并提出了诊断标准。,Wakerley, B. R. et al. GuillainBarr and Miller Fisher syndromesnew diagnos

10、tic classification. Nature Reviews Neurology 10, 537 (2014).,Antibody,神经节苷脂为一类含唾液酸的酸性糖鞘脂，由神经酰胺和低聚糖基两部分组成。 根据糖基结合唾液酸分子的数目及部位进行分类。含 1 分子 NeuAc 的神经节苷脂称为单唾液酸神经节苷脂，为 GM，含有 2 个、3 个或 4 个 NeuAc 分子的神经节苷脂分别为 GD、GT、GQ。GM 又 根 据 包 含 己 糖 的 数 目 分为GM1、GM2、GM3。 不同的神经节苷脂在神经系统中有其独特的分布：,Suggested triage criteria in hos

11、pitalized patients with Guillain-Barr syndrome,IVIG=intravenous immunoglobulin; PF =pulmonary function; 20-30-40= vital capacity decrease to 20 mL/kg, maximal inspiratory pressure decrease to -30 cm H2O, and maximal expiratory pressure decrease to 40 cm H2O.,Treatment,Outcome,Ref:van Doorn PA. Diagn

12、osis, treatment and prognosis of Guillain-Barr syndrome (GBS)J. La Presse Mdicale, 2013, 42(6): e193-e201.,Referrence,Wakerley BR, Uncini A, Yuki N, et al. GuillainBarr and Miller Fisher syndromesnew diagnostic classificationJ. Nature Reviews Neurology, 2014, 10(9): 537. 李海峰. Guillain-Barre综合征和Mille

13、r Fisher综合征的新诊断分类和标准J. 中国神经免疫学和神经病学杂志, 2014, (6): 441-443. 中国吉兰-巴雷综合征诊治指南J. 中华神经科杂志, 2010, 43(8): 583-586. Puma A, Benoit J, Sacconi S, et al. Miller Fisher syndrome, Bickerstaff brainstem encephalitis and Guillain-Barr syndrome overlap with persistent non-demyelinating conduction blocks: a case reportJ. BMC neurology, 2018, 18(1): 101. Mori M, Kuwabara S, Fukutake T, et al. Clinical features and prognosis of Miller Fisher syndromeJ. Neurology, 2001, 56(8): 1104-1106.