脱髓鞘疾病教材教学课件.pptVIP

脱髓鞘疾病 Demyelinating diseases; 脱髓鞘疾病是指一类病因或发病机制尚未彻底阐明的疾病，其主要原发病理改变为有髓鞘神经纤维的髓鞘脱失;髓鞘(myelin)的概念和构成 1.神经纤维分为有髓鞘和无髓鞘两种 2.髓鞘指有髓鞘神经轴索周围的髓鞘膜 3.髓鞘由髓鞘细胞的细胞膜组成 4.髓鞘细胞在周围神经是雪旺细胞，中 枢神经为少突胶质细胞 5.髓鞘的主要化学成分： 蛋白质 22%（碱性、脂、糖蛋白） 脂类 78% （胆固醇、神经鞘磷脂） 6.髓鞘的功能保护轴突，帮助传导神 经冲动，绝缘;脱髓鞘病按病因分两大类;多发性硬化(multiple sclerosis，MS);MS;病理特点;;;;;;;诊断标准;1982年MS专题会上制定诊断标准;1982年MS专题会上制定诊断标准;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.;; 鉴别诊断;治疗原则 1.免疫治疗 1)肾上腺皮质激素：甲基强的松龙冲击治疗，1g／d*3天或5-7天，以后改口服强的松逐渐减量乃至停用，可以反复治疗 2)硫唑嘌呤： 1mg/kg/d，2-3个月一个疗程，可长期治疗 3）β干扰素治疗 IFN –β1a(Rebif) 4)IVIg 0.4g/kg/d*3-5d ; 2.症状治疗 急性发作期： 加强护理，防止并发症 痛性强直性痉挛发作：可用巴氯芬卡马西 平、氯硝安定 肢体功能障碍：保持肢体功能位置，以 防止肌肉挛缩畸形 ;预后 取决于病人的发病频率及对药物的敏感程度 ;思考题