title: The R of ALSFRS-R: does it really mirror functional respiratory involvement in amyotrophic lateral sclerosis?
abstract: Clinical assessment of the respiratory function is critical in amyotrophic lateral sclerosis (ALS). A standardized process is to monitor the respiratory subscore of the revised ALSFRS (RofALSFRS-R). We tested the utility of RofALSFRS-R and its individual questions in evaluating respiratory functionality. Three hundred and fifty-seven consecutive ALS patients were assessed at entry (T0), three and six months later (T1 and T2). ALSFRS and its subscores decayed significantly (p < 0.001) over time. Improvement in the first respiratory question (dyspnoea, QR1) was found in about 10% of the patients in each period. Between T0 and T1 a significant negative correlation was found between decrease in gait score and QR1 (p = 0.021, r = -0.395) in the subgroup of ALS patients who showed QR1 improvement (n = 34). An improvement in the other respiratory questions was noticed in about 6% of the patients, related to non-invasive ventilation. Possibly, decreased mobility and metabolic demand can cause fewer respiratory symptoms in a subset of patients. The respiratory questions included in ALSFRS-R should be re-addressed by the ALS community.
authors.affiliation: Institute of Physiology and Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon , Lisbon , Portugal.
authors.forename: Susana
authors.initials: S
authors.lastname: Pinto
authors.forename: Mamede
authors.initials: M
authors.lastname: de Carvalho
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D012120:Respiration Disorders / Q000209:etiology*; D012720:Severity of Illness Index
keywords: ALSFRS-R; amyotrophic lateral sclerosis; functional scale; motor neuron disease; respiratory function