神经节苷脂治疗后吉兰巴雷综合征

• GBS following intravenous use of gangliosides in Europe several decades ago
• led to its withdrawal from European market
Figueras A, Morales-Olivas FJ, Capella D, Palop V, Laporte JR (1992) Bovine gangliosides and acute motor polyneuropathy. BMJ 305:1330–1331.
• 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 strength)
Grouping
• Enrolled subjects were divided inFra Baidu biblioteko the ganglioside+ group (ganglioside-associated) and the ganglioside- group (non-gangliosideassociated) according to whether they received exogenous gangliosides before disease onset.
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 humans
• Gangliosides as an exclusive component or part of a compound have never been used in our department and all the enrolled patients were referred to our department from other departments or from other hospitals.
Pathology
• AMAN and AMASN are associated with Campylobacter jejuni enteritis and antibodies against gangliosides
chemical structure of gangliosides
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
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 humans and may act as an immunogenic agent.
• 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
Evaluation of clinical severity and functional impairment
• Motor function deficits of patients were scored by the Hughes Functional Grading Scale(HFGS) score
• 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
We identified 7 patients who developed GBS after intravenous use of gangliosides
• Department of Neurology of the First Hospital of Jilin University
• 2013
• The association between exogenous gangliosides and GBS was further evidenced by the positivity of anti-GM1 and anti-GT1a antibodies in CSF and plasma
Guillain-Barre ´ syndrome (GBS)
• AIDP • AMAN • AMSAN • MFS
Ganglioside
• 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GQ