• Nimish J Thakore, Brittany R Lapin, Erik P Pioro, and Pooled Resource Open-Access ALS Clinical Trials Consortium.
• Department of Neurology, Neuromuscular Center, Cleveland Clinic, 9500 Euclid Ave S90 Cleveland, Ohio, 44124, USA.
• Muscle Nerve. 2018 Jun 1; 57 (6): 937-945.

IntroductionRate of decline of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) score is a common outcome measure and a powerful predictor of mortality in ALS.MethodsObserved rate of decline (postslope) of ALSFRS-R, its linearity, and its relationship to decline at first visit (preslope) were examined in the Pooled Resource Open-Access ALS Clinical Trials cohort by using longitudinal mixed effects models.ResultsMean ALSFRS-R postslope in 3,367 patients was -0.99 points/month. Preslope and postslope were correlated and had powerful effects on survival. ALSFRS-R trajectories were slightly accelerated overall, but slope and direction/degree of curvature varied. Subscore decline was sequential by site of onset. Respiratory subscore decline was the least steep.DiscussionVariable curvilinearity of ALSFRS-R trajectories confounds interpretation in clinical studies that assume linear decline. Subscore trajectories recapitulate phenotypic diversity and topographical progression of ALS. ALSFRS-R is better used as a multidimensional measure. Muscle Nerve 57: 937-945, 2018.© 2017 Wiley Periodicals, Inc.

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