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1、Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.第十七章第十七章 神经神经-肌肉接头和肌肉疾病肌肉接头和肌肉疾病第十七章 神经-肌肉接头和肌肉疾病Diseas
2、es of Neuromuscular Junction and muscules Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.神经神经-肌肉接头肌肉接头
3、 神经神经 肌肉接头肌肉接头 一个运动神经元的轴突可以分一个运动神经元的轴突可以分出数十至数千个出数十至数千个细小的分支细小的分支,这些分这些分支与所支配的肌纤维以支与所支配的肌纤维以突触突触的形式的形式相联系相联系,这样的突触称为神经这样的突触称为神经 肌肉肌肉接头。接头。Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the we
4、bmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.神经神经-肌肉接头肌肉接头Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les
5、Laboratoires Servier-all rights reserved.神经神经-肌肉接头肌肉接头 突触的组成突触的组成 突触前膜突触前膜(神经末梢神经末梢)突触间隙突触间隙 突触后膜突触后膜(肌膜肌膜)结构特点结构特点皱褶皱褶Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permiss
6、ion.Copyright 2006,Les Laboratoires Servier-all rights reserved.神经神经-肌肉接头肌肉接头Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servie
7、r-all rights reserved.骨骼肌的解剖生理骨骼肌的解剖生理静息电位静息电位（钠内流、钾外流）（钠内流、钾外流）终板电位终板电位-肌肉收缩肌肉收缩 （钠外流、钾内流）静息电位恢复（钠外流、钾内流）静息电位恢复Ach的去向的去向 1/3结合结合、1/3 被被AchE破坏破坏 1/3被被重新摄取重新摄取+Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,
8、please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.突触后膜AChR病变（自身抗体）重症肌无力肉毒杆菌中毒高镁血症阻碍钙离子进入神经末梢抑制胆碱酯酶活力,导致突触后膜过度去极化ACh释放减少有机磷中毒Lambert-Eaton综合征氨基甙类药物ACh合成&释放减少美洲箭毒与AChR结合,阻断ACh与AChR结合 骨骼肌的解剖生理骨骼肌的解剖生理Non Commercial Use OnlyInformation and material o
9、n slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.重症肌无力重症肌无力第一节 重症肌无力Myasthenia GravisNon Commercial Use OnlyInformation and material on slide kit may be
10、reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.是一种是一种神经神经-肌肉接头传递功能障碍的获肌肉接头传递功能障碍的获得性自身免疫性疾病。得性自身免疫性疾病。临床表现为部分或全身骨骼肌易于疲劳，活临床表现为部分或全身骨骼肌易于疲劳，活动后加重，休息后减轻。晨轻暮重动后加重，休息后减轻。晨轻暮重
11、神经肌肉接头 重症肌无力重症肌无力Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.自身免疫性疾病自身免疫性疾病 证据如下证据如下Non Commercial
12、Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.4 1 用从鳗鱼放电器官提取的AChR注射给动物可注射给动物可 制成自身免疫性重症肌无力的动物模型，患病的动动物模型，患病的动物物血清中可测
13、到AChR-Ab,可与突触后膜AChR结合4AChR免疫荧光法检测患病动物AChR数目显著减少4 2 MG病人血清输入小鼠可产生类似MG的症状 和电生理改变 重症肌无力重症肌无力Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laborat
14、oires Servier-all rights reserved.43 80%90%的MG患者外周血可检出AChR-Ab,血浆交换清除AChR-AbAChR-Ab后MG症状可暂时改善 重症肌无力重症肌无力Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 20
15、06,Les Laboratoires Servier-all rights reserved.4 1020%的MG患者合并胸腺瘤,约80%的MG患者 胸腺肥大,淋巴滤泡增生。胸腺切除后症状可减轻或痊愈 5MG患者常合并甲亢、甲状腺炎、SLE等自身免疫性疾病 重症肌无力重症肌无力Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the
16、webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.重症肌无力重症肌无力病因&发病机制 自身免疫反应是如何启动的？2种情况种情况 突触后膜突触后膜AchR 免疫原性免疫原性 胸腺肌样细胞胸腺肌样细胞AchR构型构型 （分子模拟机制）（分子模拟机制）Non Commercial Use OnlyInformation and material on slide kit may be reproduced and distributed for educational use only For commercial usage,please contact the webmaster for permission.Copyright 2006,Les Laboratoires Servier-all rights reserved.重症肌无力重症肌无力分子模拟机制 正常正常&增生的胸腺可发现增生的胸腺可发现“肌样细肌样细胞胞”(myoid cell),具有横纹具有横纹 并载
