S08 神经肌肉接头病(20130228学生教案).ppt

神经-肌肉接头疾病DISORDERS OF NEUROMUSCULAR TRANSMISSION ;重症肌无力Myasthenia Gravis;What is Myasthenia Gravis (MG)?;*;*;突触前膜;*;NMJ病变：;Autoimmune basis and pathology of MG;MG is an acquired immunological disorder;Why does MG cause muscle weakness?;*;;胸腺是MG启动部位!;MG is closely associated with thymic pathology. 60-70% thymus hyperplasia(胸腺增生), typically for young women. 15% thymoma(胸腺瘤), typically for old men. ;EPIDEMIOLOGY OF MG;*;CLINICAL PRESENTATION of MG;*;Ocular;2. Bulbar球部肌群 ;3 Axial轴向肌群;4. Extremity四肢肌群;5. Respiratory呼吸肌群;*;7. Concurrent disorders in MG;CLINICAL EVALUATION OF MG;;Special type of MG;Neonatal myasthenia新生儿MG;Neonatal myasthenia新生儿MG; Congenital (Genetic) Myasthenic Syndromes;药物性MG;Diagnostic Tests;1. Edrophonium (Tensilon)依酚氯铵(腾喜龙) test;2.新斯的明（Neostigmine)试验;3． Electrical tests;*;3． Electrical tests;4. Serum antibody assay;5. Evaluation of thymus胸腺, CT, MRI;CT in a patient with MG revealing large necrotic mass in the left anterior mediastinum (white arrows) and bilateral hilar lymphadenopathy;CT ;6. Office Tests诊室内检查 ;Fatigue test （Jolly test );Sleep test;Ice test; Diagnosis of MG！;DIFFERENTIATION of MG;Lambert-Eaton Myasthenic Syndrome;Lambert-Eaton Myasthenic syndrome;Lambert-Eaton Myasthenic syndrome;;2.肉毒杆菌中毒Botulism;Antitoxin抗毒素血清 is begun as soon as possible. Guanidine hydrochloride盐酸胍 Respiratory function must be monitored frequently in all cases. Nasogastric鼻胃管 or endotracheal intubation气管插管. Prognosis in treated patients is about 80% survival. ;肉毒毒素Botulinum toxin (BOT)注射;3.脑干梗死;4.眼咽型肌营养不良症;TREATMENT OF MG;Treatment strategies六大治疗方法！;Pyridostigmine吡啶斯的明, 60mg(range 30-120mg), q4-8h. 适应症： 各型 MG. 尤其是新近诊断的MG患者的起始治疗 以及中等程度病情MG患者长期对症治疗;Anticholinesterase drug (2) —Adverse effects ;First thymectomy, 1939;2.Thymectomy (TE) ;3.Corticosteroids ;4.Immunosuppressants;5 Plasma exchange (PE) ;6 Intravenous Immune Globulin (IVIG);MG治疗策略与目标;Basic therapies used to treat MG四项基本策略:;Treatment of MG——Recommendations (1);Caution! Contraindicated medica