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Trajectories of impairment in amyotrophic lateral sclerosis: Insights from the Pooled Resource Open‐Access ALS Clinical Trials cohort
Author(s) -
Thakore Nimish J.,
Lapin Brittany R.,
Pioro Erik P.
Publication year - 2018
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.26042
Subject(s) - amyotrophic lateral sclerosis , clinical trial , medicine , cohort , physical medicine and rehabilitation , rating scale , cohort study , physical therapy , statistics , disease , mathematics
: Rate 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. Methods : Observed 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. Results : Mean 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. Discussion : Variable 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