Trajectories of impairment in amyotrophic lateral sclerosis: Insights from the Pooled Resource Open‐Access ALS Clinical Trials cohort
2017; Wiley; Volume: 57; Issue: 6 Linguagem: Inglês
10.1002/mus.26042
ISSN1097-4598
AutoresNimish Thakore, Brittany Lapin, Erik P. Pioro,
Tópico(s)Neurogenetic and Muscular Disorders Research
ResumoABSTRACT Introduction : 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
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