[Revised amyotrophic lateral sclerosis functional rating scale at time of diagnosis predicts survival time in amyotrophic lateral sclerosis]

Abstract

Objective: To 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.

Methods: A 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.

Results: The 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.

Conclusion: ALSFRS-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.