Objective: To study the amyotrophic lateral sclerosis functional rating scale revised(ALSFRS-R) and its progression rate of ALSFRS-R correlation with survival time. Methods:Application of 2000 word Union of Neurology El Escorial diagnostic criteria retrospectively analyzed 64 case of amyotrophic lateral sclerosis clinical date, be ALSFRS-R score and the progress rate(⊿FS)and Kaplan-Meier analysis was used from onset to tracheostomy or death ,the median group and Log-rank analysis was used to estimate the difference between two groups. Application of Cox proportional hazards model,adjust the parameters of multi-factor analysis: sex, age, onset duration , forced vital capacity(FVC). Results:Total 64 ALS patient were followed, 8 lost,56 with incision of tracheal or death, M∶F=2.7∶1, the average survival time of 3.5 years. Log-rank analysis of two groups was statistically significant. Application of Cox proportional hazards model ALSFRS-R(HR:0.87,95%CI:0.82-0.93,P < 0.01),⊿FS(HR:5.52,95%CI:3.13-9.71,P < 0.01 ). Conclusion:ALSFRS-R and ⊿FS predicting the prognosis of patients.