Patient reported outcomes in ALS: characteristics of the self-entry ALS Functional Rating Scale-revised and the Activities-specific Balance Confidence Scale.

Objective: This study characterized two patient-reported outcome measures (PROMs): a patient-facing adaptation of the revised amyotrophic lateral sclerosis (ALS) Functional Rating Scale ("self-entry ALSFRS-R") and the Activities-specific Balance Confidence (ABC) Scale. Methods : ALS patients presenting to clinic completed PROMs that included (1) the self-entry ALSFRS-R, (2) the Activities-specific Balance Confidence Scale (ABC Scale), and (3) a question about falls. PROM data were compared to one another and to the traditional ALSFRS-R collected by trained evaluators in clinic ("standard ALSFRS-R"). Results : Over the data collection period, 449 ALS patients completed at least one of the three PROMs. Self-entry vs. standard ALSFRS-R total scores ( n  = 183) had high agreement (intraclass correlation (ICC)=0.81, 95% CI = 0.67, 0.88). Self-entry ALSFRS-R total scores were significantly higher than standard ALSFRS-R total scores (2.3 points, p  < 0.001). In a subset of participants who contributed data at two timepoints, the average ALSFRS-R decline was not significantly different between methods ( n  = 49). ABC scores correlated highly with self-entry and standard ALSFRS-R Gross Motor subdomain scores (Pearson's r  = 0.72, p  < 0.001 and Pearson's r  = 0.76, p  < 0.001, respectively; n  = 130). ABC score was negatively correlated with the number of reported falls within the last month (Spearman's r =-0.40; p  < 0.001; n  = 130). A 10-point decrease in ABC score increased odds of a reported fall by 16%. Conclusions : In a multidisciplinary clinic setting, self-entry and standard ALSFRS-R scores were similar, but not interchangeable. Self-entry scores were higher than standard ALSFRS-R scores but declined at a similar rate to the standard ALSFRS-R. ABC scores correlated with self-reported fall history and thus may provide useful data for clinical care.