重症肌无力(诊疗,结合2015版指南).ppt

重症肌无力(myasthenia gravis/MG ) Neurology & Neurosurgery Ward 3 2 Neurology & Neurosurgery 中国指南 2015 3 Neurology & Neurosurgery 重症肌无力是神经肌肉接头处的烟碱乙酰胆碱受体受到抗体介导的侵袭或 Musk 抗体而引起的一种神经肌肉传递功能缺失。本病的特征是波动性肌无力,经胆碱脂酶抑制剂可以改善经典教科书介绍 4 Neurology & Neurosurgery ? December 22 .— Great improvement ? January 17,1891 .— She is slightly worse . The tongue appears atrophied. During the next month fluctuations in severity of the symptoms occurred ? Feb , 1891 — Is much worse again . She now has ptosis, diplopia , difficulty in mastication , and atrophy of the masseters. The movements of the soft palate are sluggish; speech is nasal … the voice is weak, especially after lengthy speech . There is great weakness and fatigue in the legs …. Improvement again occurred ? but later she became worse and died suddenly on the night of April 24 …. Erb W. Zur casuistic der bulba ¨ren La ¨ hmungen . Arch Psychiat 1891;9:325. 5 Neurology & Neurosurgery 1672 年, 牛津医生 Thomas Willis 描述了一名构音障碍的女性病人 187 9年, 德国医生 Wilheim Erb 描述了一种“新的球麻痹综合征”,表现为咀嚼费力,颈肌抬举困难而无肌肉病变的特点 1893 年, Samuel Goldflam 第一次用“myasthenia gravis ”描述此病,并发现肌肉在休息后改善,疲劳后加重 1935 年, 波士顿 Henry Viets 诊断了第一例重症肌无力 Neurology & Neurosurgery 7 Neurology & Neurosurgery 肌无力分布?最常见为眼外肌,40% 为眼肌首先受累, 85% 最终累及眼肌。可单侧或双侧,眼睑下垂和复视; ?其次是面肌和口咽肌,出现构音障碍,吞咽困难。?肢体和颈肌。?不单独累及肢体。 Neurology & Neurosurgery 症状?肌无力症状波动性,劳累性无力,休息后好转,晨轻暮重。变化有时和体力活动有关,即易疲劳性。肌无力症状逐渐出现和加重,而非突然的瘫痪。?患者主诉一般为无力,而非疲劳感。?对胆碱能药物反应:可见到症状的改善。为确诊的一部分。 Neurology & Neurosurgery 查体和常规检查?生命体征和全身体检基本正常。?神经系统查体取决于无力的分布。最常见为眼睑下垂,眼肌无力时可有复视或动眼受限。可有吞咽或肢体无力的表现。感觉正常,反射保留。长期重症病人有肌肉萎缩。?疲劳试验:眼睑、肢体?血尿常规、脑脊液常规检查正常 Neurology & Neurosurgery 发病机制