外科学MG.ppt

Myasthenia Gravis MG 重症肌无力 Etiology and Pathogenesis In 1973 Patrick and Lindstrom successfully made an animal mode named EAMG.(experimental auto- immune myasthenia gravis),which supports powerful evidence for immunologic mechanism .It shows the basic defect in MG -a marked reduction in the number of Ach-R on the postsynaptic membrane（突触后膜） of the neuromuscular junction.The myasthenic weakness and fatigability are due to the failure of effective neuromuscular transmission. Clinical Picture MG can occur at any age ,but there is a peak incidence in females in the second and third decades and in the sixth and seventh decades.females are affected twice as often as males .The onset is usually insidious（潜伏）,with fluctuating weakness that tends to be worse at the end of the day or following exercise .Most commonly the bulbar（延髓） muscles are involved early ,with diplopia（复视）,ptosis of the eyelid（眼睑下垂） and /or difficulty in chewing（咀嚼），disarthria(构音） and dysphagia（咽下） constituting the most common presenting symptoms.As the disease advance ,limb and neck muscles , eventually respiratory muscles become involved.In occasional cases,there is an acute fulminant（爆发） onset with the rapid development of respiratory failure . Presenting symptom in myasthenia gravis Examination 1.Blood urine and CSF routine are normal ,thymoma found by chest CT scan or X-ray in more age of 40 . 2. Decremental（衰减） response (stimulation at 2 or 5Hz) and “jitter”（颤抖） wave (single-fiber) emerge in the electromyography. 3.Ach-R antibody can be measured in the 85-90%generalize MG. Classification I Ocular myasthenia (15%)眼肌型 IIA Mild generalized myasthenia with slow progression;no crisis ;drug-responsive(30%)轻度全身型 IIB Moderate generalized severe skeletal and bulbar involvement but no crisis;drug-response is less satisfactory.(25%)中度全身型 III Acute fulminating myasthenia;rapid progression of severe symptoms with respiratory crisis and poor drug response;high incidence of thymoma;high mortality.(