OBJECTIVE: To assess the utility of rate of disease progression (  @entity241 ) as a prognostic biomarker in @entity746 ( @entity746 ). METHODS: A total of 203 @entity1 with @entity746 were prospectively recruited over a 10-year period. At initial visit, the following variables were collected: demographic details, symptom duration, site of onset, phenotype, @entity10891 use and Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) scores. The   @entity241 score at initial visit was calculated by dividing the ALSFRS-R total score by symptom duration (months). The primary end point was survival. Kaplan-Meier survival curves were used to illustrate the distribution of survival from a specified point, while multiple Cox proportional hazards modelling with backward stepwise variable selection was used to identify the independent predictors of survival at initial visit. RESULTS: The   @entity241 score at initial visit was a significant predictor of survival in @entity746 (p<0.001), and remained significant when adjusted for age and site of onset (p<0.001). 3 prognostic subgroups emerged, with a   @entity241 score of <0.47 associated with a median survival of 2.4   years, which was significantly greater when compared with an initial   @entity241 score of between 0.47 and 1.11 (1.6   years, p<0.05) and a score >1.11 (0.7   years, p<0.001). Importantly, multiple Cox proportional hazards modelling identified   @entity241 as a highly significant independent predictor of survival in @entity746 (p<0.001) along with site of disease onset (p<0.01). CONCLUSIONS: Rate of disease progression appears to be a simple and sensitive clinical prognostic biomarker in @entity746 that could be potentially utilised in clinical practice and future therapeutic trials.

Rate of disease progression: a prognostic biomarker in XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity241 :: ('MESH:D018223', 'Disease') :: ['FS'] @entity10891 :: ('MESH:D019782', 'Chemical') :: ['riluzole'] @entity746 :: ('MESH:D000690', 'Disease') :: ['ALS', 'amyotrophic laterals sclerosis']
@entity746 :: (MESH:D000690,Disease) :: ['ALS']
