神经节苷脂治疗后吉兰巴雷综合征课件.ppt

神经节苷脂治疗后吉兰巴雷综合征
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 外表面,在神经系统中含量丰富,在周围神经系统中关键存在于轴索, 而在胶质和髓鞘中浓度较低
依据其糖基结合唾液酸分子 数目及部位分为GM、GD、GT 及GQ
Pathology
AMAN and AMASN are associated with Campylobacter jejuni enteritisand antibodies against gangliosides
chemical structure of gangliosides
不一样种类 神经节苷脂在周围神经系统 分布含有各自不一样 特点
GM1除广泛分布于周围神经 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 含量多于其在感觉神经髓鞘内 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 髓鞘中含量高于其她颅神经
特定抗神经节苷脂抗体和含有一些特异临床特点 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）, 症状学研究提醒GM1抗体阳性 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 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 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.
GBS following intravenous use of gangliosides in Europe severaldecades ago
led to its withdrawal from European market
Figueras A, Morales-Olivas FJ, Capella D, Palop V, Laporte JR (1992) Bovinegangliosides 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

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 from other departments or from other hospitals.