• Xiao-xuan Liu, Dong-sheng Fan, Jun Zhang, Shuo Zhang, and Ju-yang Zheng.
• Department of Neurology, Peking University Third Hospital, Beijing, China.
• Zhonghua Yi Xue Za Zhi. 2009 Sep 22;89(35):2472-5.

ObjectiveTo identify the correlation between the revised amyotrophic lateral sclerosis (ALS) functional rating scale (ALSFRS-R) and the progression rate of ALSFRS-R (DeltaFS) at time of diagnosis and survival time in a prospective survey.MethodsA total of 129 consecutive patents with definite or probable ALS were enrolled at our hospital from January 2002 to December 2005. The patients were monitored with a standard evaluation form recording clinical features, ALSFRS-R and forced vital capacity (FVC) every 3 months from visit to death or tracheostomy.ResultsThe mean age at onset was (52 +/- 11) years old. The median survival time from symptom onset was 45.71 months (95% CI = 35 to 51). In univariate analysis of Kaplan-Meier method, the outcome was significantly related to total ALSFRS-R and DeltaFS (P < 0.05). In Cox multivariate model, DeltaFS and FVC% were both statistically significant predictors of survival after adjusting for age and onset site (P < 0.05). Al-unit decrease in DeltaFS was associated with a 73.3% decreased risk of death.ConclusionALSFRS-R is sensitive to the change over time and can predict survival at diagnosis. It can be used as a primary outcome measure in ALS clinical trial. DeltaFS at diagnosis can provide us the information of disease progression at one particular point. It is a more significant predictor of survival in patients with ALS as compared to total ALSFRS-R and time from onset to diagnosis.

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