樊东升：ALS研究的进展与思考汇编.pptVIP

(1) ALSFRS-R; (2) Body Weight, FVC, MRC score, etc; (3) Electrodiagnositc measures (MUNE; CMAP) Rapid progression: the % decrease in both MUNE and CMAP amplitude during the first 3 months ＞ 50% (2) Moderate progression: the % decrease in MUNE ＞ 50% but CMAP amplitude ＜ 50% during the first 3 months (3) Slow progression: the % decrease in both MUNE and CMAP amplitude during the first 3 months ＜ 50% FALS分类 染色体定位 基因 蛋白 遗传方式 临床特征 突变位点 与SALS有关 ALS1. 21q. SOD1. Cu/Zn超氧化物歧化酶 AD. 典型ALS 139 是 ALS2. 2q33. ALSIN. Alsin AR. 青少年ALS，进展缓慢，以上运动神经