神经节苷脂治疗后吉兰巴雷综合征课件.ppt

1、Guillain-Barre syndrome(GBS)AIDP AMAN AMSAN MFSGanglioside 神经节苷脂广泛分布于全身各组织细胞的外表面,在神经系统中含量丰富,在周围神经系统中主要存在于轴索，而在胶质和髓鞘中浓度较低 根据其糖基结合唾液酸分子的数目及部位分为GM、GD、GT 及GQPathology AMAN and AMASN are associated with Campylobacter jejuni enteritisand antibodies against gangliosides chemical structure of gangliosides不同

2、种类的神经节苷脂在周围神经系统的分布具有各自不同的特点 GM1除广泛分布于周围神经的轴索，还分布于郎飞氏节及施万细胞近轴索侧细胞膜，在运动神经髓鞘内GM1的含量多于其在感觉神经髓鞘内的含量 GD1b在后根神经节大感觉神经元分布较多 GQ1b在动眼、滑车、外展神经的髓鞘中含量高于其他颅神经特定抗神经节苷脂抗体和具有某些特异临床特点的神经系统疾病相关 抗GM1抗体见于急性运动轴索性神经病（AMAN）及脱髓鞘性GBS，IgM类GM1抗体见于多灶性运动神经病（MMN），症状学研究提示GM1抗体阳性的患者更多出现肢体无力，而感觉障碍相对较少 抗GD1b抗体与感觉性共济失调等感觉障碍为主的疾病相关 GQ1

3、b与Miller-Fisher 综合征（MFS),Bickerstaff脑干脑炎（BEE）等存在眼肌麻痹或球部肌肉受累的疾病相关After exogenous gangliosides injection High titers of anti-GM1 antibodies were found in patients who developed GBS following exogenous gangliosides injection,leading to the suspicion that exogenous gangliosides might be foreign to human

4、s and may act as an immunogenic agent.GBS following intravenous use of gangliosides in Europe severaldecades ago led to its withdrawal from European marketFigueras A,Morales-Olivas FJ,Capella D,Palop V,Laporte JR(1992)Bovinegangliosides and acute motor polyneuropathy.BMJ 305:13301331.We identified 7

5、 patients who developed GBS after intravenous use of gangliosides Department of Neurology of the First Hospital of Jilin University 2013 Gangliosides as an exclusive component or part of a compoundhave never been used in our department and all the enrolled patients were referred to our department fr

6、om other departments or from other hospitals.Grouping Enrolled subjects were divided into the ganglioside+group(ganglioside-associated)and the ganglioside-group(non-ganglioside-associated)according to whether they received exogenousgangliosides before disease onset.Evaluation of clinical severity an

7、d functional impairment Motor function deficits of patients were scored by the Hughes Functional Grading Scale(HFGS)score Neurologic function was also evaluated by using the Medical Research Council(MRC)sum score of six bilateral muscles in arms and legs,ranging from 0(tetraparalytic)to 60(normal st

8、rength)The association between exogenous gangliosides and GBS high titers of anti-GM1 antibodies were found in some of the patients who developed GBS after receiving a ganglioside therapy,leading to the suspicion that exogenous gangliosides might be foreign to humans and might be neuritogenic in hum

9、ans The association between exogenous gangliosides and GBS wasfurther evidenced by the positivity of anti-GM1 and anti-GT1aantibodies in CSF and plasmaSum In sum,exogenous gangliosides may be associated withdevelopment of GBS due to incompletely recognized pathogenesis.Ganglioside-associated GBS is more severe in clinical course withpoorer short-term prognosis as compared with non-gangliosideassociated GBS in northeast China.