中文摘要：

目的研究系统化临床检查对重症肌无力（MG）受累范围和临床分型判断的价值,评价其获得的分型在不同医师间的可重复性。方法由1名有10年MG诊治经验的医师A对64例MG患者依次询问病史（步骤1）、进行疲劳试验（步骤2）、新斯的明试验（步骤3）和重复神经电刺激（RNS;步骤4）检查,每步检查后均汇总现有资料判断受累肌群以及Osserman和MGFA分型,通过比较各步结果的改变来评价上述各项目在判断受累肌群和临床分型中的价值。将A医师获得的原始资料发给4名不同MG诊治经验的医师,由他们独立判断受累肌群和分型,通过Kappa检验评价他们之间分型的可重复性。结果步骤2与步骤1比较,35例患者受累肌群改变,11例Osserman分型和21例MGFA分型改变;步骤3与步骤2比较,9例受累范围减少,6例Osserman分型和MGFA分型改变;步骤4与步骤3比较,25例受累范围增加。不同医师间Osserman和MGFA分型的Kappa值均〉0.5（均P〈0.01）,各步骤Osserman分型的重复性均优于MGFA分型。结论疲劳试验可发现潜在的受累肌群,较准确地判断临床分型。新斯的明试验可提高受累肌群判断的特异性,进一步修正临床分型。RNS检查可更全面评价受累肌群。系统化临床检查有助于对MG患者准确分型。不同MG诊治经验的医师间Osserman和MGFA分型判断的可重复性较好。

英文摘要：

Objective To study the value of systematic clinical examinations in determining the involved muscles and clinical classification of myasthenia gravis（MG） patients,and to evaluate the consistency of this diagnostic protocol in physicians with various experience in managing MG. Methods A total of 64 MG patients were examined by physician A,who has experience of more than 10 years in managing MG,with history-taking,Jouy test,neostigmine test and repetitive nerve stimulation（RNS） successively.The value of each step mentioned above in identifying the involved muscles and clinical classification of MG was evaluated by comparing the results of each step.The original data of examinations were sent to four other physicians with various experiences in managing MG for independent evaluation.Agreement in determining clinical classification between each physician was analyzed with Kappa test. Results After Step 2,the involved muscles,the Osserman classification and the MGFA classification were revised in 35 patients,11 patients and 21 patients.After Step 3,the scope of muscle involvement was reduced in 9 patients,and 6 patients were further revised in both Osserman and MGFA classification.After Step 4,however,the scope of muscle involvement was increased in 25 patients.Kappa values of both classifications among different physicians were greater than 0.5（P0.01 in all comparison）,and agreement on Osserman classification is better than that of MGFA classification in each step. Conclusions Jouy test is important in identifying involved muscles and determining clinical classification accurately.Neostigmine test improves the specificity for determining the involved muscles and further revises classification.RNS evaluates the muscle involvement of MG patients more comprehensively.Systematic clinical examinations can help to determine classification of MG exactly.There is relatively good agreement in both Osserman and MGFA classifications among different physicians with different experiences in managing MG.