脱髓鞘病讲课阮喜云2012课件.pptVIP

概念 脱髓鞘疾病（demyelinative diseases）是一组脑和脊髓以髓鞘破坏或脱髓鞘病变为主要特征的疾病，脱髓鞘是其病理过程中具有特征性的突出表现。 罕见症状 如失语症、偏盲、锥体外系运动障碍、严重肌萎缩和肌束颤动等 1.Benign Multiple Sclerosis Mild infrequent sensory exacerbations with full recovery. 2.Relapsing Remitting Multiple Sclerosis Episodes of exacerbations and remissions during which not all symptoms resolve completely. The patient may be left with permanent disability which may vary in severity. relapses are often more severe than in the previous group. Relapses also become more severe with time.? 3.Secondary Chronic Progressive Condition of patients with relapsing/remitting disease begins to gradually worsen over time with resulting accumulation of neurologic signs and symptoms. In this form of the disease, relapses become more severe while remissions are less complete, shorter in duration, and eventually non-existent. The course of MS becomes steadily progressive. 4.Primary Progressive There is no history of relapse in these patients. Disease begins with a slow progression of neurologic deficits. Problems appear and gradually worsen over time. Common problems include spastic paraparesis, cerebellar ataxia, urinary incontinence. MS（经典型） MS（经典型） 多发性硬化诊断标准（1983，Poser） 诊断分类 诊断标准（符合其中1条）OB/IgG 临床确诊 ①病程中两次发作和两个分离病灶临床证据 ②两次发作， 一处临床和另一处亚临床病变证据 实验支持确诊 ①病程中两次发作，一个临床或亚临床病变证据， CSF OB/IgG ②病程中两次发作，两个分离病灶临床证据， CSF OB/IgG ③病程中一次发作，一处病变临床证据和另一部位 病变亚临床证据，CSF OB/IgG 临床可能 ①病程中两次发作，一处病变的临床证据 ②病程中一次发作，两个不同部位病变临床证据 ③病程中一次发作，一处病变临床证据和另一部位 病变亚临床证据 实验支持可能 病程中两次发作， CSF OB/IgG，两次发作须累及 CNS不同部位，间隔至少1个月，每次须持续24h 注： OB/IgG代表电泳寡克隆区带阳性或IgG指数或24小时鞘内合成率升高 尼古拉?奥斯特洛夫斯基（1904-1936） Mans dearest possession is life, and since it is given to him t