Aerobic capacities and exercise tolerance in neuromuscular diseases: A descriptive study

Abstract

The main limitation factor in exercise tests is peripheral. Ventilatory and cardiovascular limitations can nonetheless be added on; while they are absent when the patient is at rest, they are unmasked in effort situations. Exercise tests could be of great interest in screening and managing the relevant pathologies. Multicenter studies on homogeneous populations could facilitate definition of the procedures specific to exercise tests for patients suffuring from neuromuscular diseases.

Cardiopulmonary exercise test to quantify enzyme replacement response in pediatric Pompe disease

“…Patient #5 improved in all the parameters and was the only one to improve in both 6MWD and peakVO 2 . Similar inconsistency in respiratory limitation parameters were reported post 36 month of ERT treatment and may indicate decreased sensitivity of these respiratory limitation parameters in advanced disease …”

“…To the best of our knowledge, no studies have been published evaluating pediatric Pompe patients using CPET as a tool for exercise capacity evaluation. Rapin et al reported CPET results in metabolic myopathies in adult patients; 5/9 did not meet the criteria for a maximal test . Bartels et al evaluated nine children with muscular dystrophy and concluded that criteria for maximal effort were primarily developed to detect exercise limitations in patients with cardiac and pulmonary disease.…”

Role of the cardio-pulmonary exercise test and six-minute walking test in the evaluation of exercise performance in patients with late-onset Pompe disease

“…In some patients an insufficient effort, as shown by a low RER, could at least partly warrant the low peak VE and HRR values; however, that was not the case for other subjects. Accordingly, a recent paper on neuromuscular patients, including four subjects affected by glycogenosis, reported that limited exercise tolerance was multifactorial due to both an insufficient Vt recruitment and a low cardiac output [23]. The insufficient Vt recruitment may be related to respiratory muscles' weakness, while cardiac abnormalities could depend on central deconditioning due to sedentary lifestyle or to autonomic dysfunction.…”

Fighting Against Disuse of the Masticatory System in Duchenne Muscular Dystrophy

“…[32][33][34] The compliance of the patient group and healthy subjects was high. Moreover, sugar-free chewing gum contains polyols which are alcohol derivates of sugar.…”