神经节苷脂治疗后吉兰巴雷综合征
合集下载
相关主题
- 1、下载文档前请自行甄别文档内容的完整性，平台不提供额外的编辑、内容补充、找答案等附加服务。
- 2、"仅部分预览"的文档,不可在线预览部分如存在完整性等问题,可反馈申请退款(可完整预览的文档不适用该条件!)。
- 3、如文档侵犯您的权益，请联系客服反馈,我们会尽快为您处理(人工客服工作时间：9:00-18:30)。
• 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
Grouping
• Enrolled subjects were divided into the ganglioside+ group (ganglioside-associated) and the ganglioside- group (non-gangliosideassociated) according to whether they received exogenous gangliosides before disease onset.
• 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
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 • The association between exogenous gangliosides and GBS was further evidenced by the positivity of anti-GM1 and anti-GT1a antibodies in CSF and plasma
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.
• 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.
We identified 7 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 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.
Evaluation of clinical severity and 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 strength)
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
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
Hale Waihona Puke Baidu Sum
• In sum, exogenous gangliosides may be associated with development of GBS due to incompletely recognized pathogenesis. Ganglioside-associated GBS is more severe in clinical course with poorer short-term prognosis as compared with nongangliosideassociated GBS in northeast China.
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
Grouping
• Enrolled subjects were divided into the ganglioside+ group (ganglioside-associated) and the ganglioside- group (non-gangliosideassociated) according to whether they received exogenous gangliosides before disease onset.
• 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
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 • The association between exogenous gangliosides and GBS was further evidenced by the positivity of anti-GM1 and anti-GT1a antibodies in CSF and plasma
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.
• 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.
We identified 7 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 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.
Evaluation of clinical severity and 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 strength)
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
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
Hale Waihona Puke Baidu Sum
• In sum, exogenous gangliosides may be associated with development of GBS due to incompletely recognized pathogenesis. Ganglioside-associated GBS is more severe in clinical course with poorer short-term prognosis as compared with nongangliosideassociated GBS in northeast China.