神经节苷脂治疗后吉兰巴雷综合征.ppt

1Guillain-Barre syndrome(GBS)AIDPAMANAMSANMFS2Ganglioside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GQ3PathologyAMAN and AMASN are associated with Campylobacter jejuni enteritisand antibodies against gangliosides 4chemical structure of gangliosides5不同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6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GQ1b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7After exogenous gangliosides injectionHigh 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 humans and may act as an immunogenic agent.8GBS following intravenous use of gangliosides in Europe severaldecades agoled to its withdrawal from European marketFigueras A,Morales-Olivas FJ,Capella D,Palop V,Laporte JR(1992)Bovinegangliosides and acute motor polyneuropathy.BMJ 305:13301331.9We identified 7 patients who developed GBS after intravenous use of gangliosidesDepartment of Neurology of the First Hospital of Jilin University 2013Gangliosides 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 from other departments or from other hospitals.1011GroupingEnrolled 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.121314Evaluation of clinical severity and functional impairmentMotor function deficits of patients were scored by the Hughes Functional Grading Scale(HFGS)scoreNeurologic 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 strength)151617The association between exogenous gangliosides and GBShigh 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 humansThe association between exogenous gangliosides and GBS wasfurther evidenced by the positivity of anti-GM1 and anti-GT1aantibodies in CSF and plasma18SumIn 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.19此课件下载可自行编辑修改，供参考！感谢您的支持，我们努力做得更好！20