Exercise training alone or in combination with high-protein diet in patients with late onset Pompe disease: results of a cross over study.

BACKGROUND: Late onset Pompe disease (LOPD) is a lysosomal neuromuscular disorder which can progressively impair the patients' exercise tolerance, motor and respiratory functions, and quality of life. The available enzyme replacement therapy (ERT) does not completely counteract disease progression. We investigated the effect of exercise training alone, or associated with a high-protein diet, on the exercise tolerance, muscle and pulmonary functions, and quality of life of LOPD patients on long term ERT. METHODS: The patients were asked to participate to a crossover randomized study comprehending a control period (free diet, no exercise) followed by 2 intervention periods: exercise or exercise + diet, each lasting 26 weeks and separated by 13 weeks washout periods. Exercise training included moderate-intensity aerobic exercise on a cycle ergometer, stretching and balance exercises, strength training. The diet was composed by 25-30% protein, 30-35% carbohydrate and 35-40% fat. Before and after each period patients were assessed for: exercise tolerance test on a cycle-ergometer, serum muscle enzymes, pulmonary function tests and SF36 questionnaire for quality of life. Compliance was evaluated by training and dietary diaries. Patients were contacted weekly by researchers to optimize adherence to treatments. RESULTS: Thirteen LOPD patients, median age 49 ± 11 years, under chronic ERT (median 6.0 ± 4.0 years) were recruited. Peak aerobic power (peak pulmonary O2 uptake) decreased after control, whereas it increased after exercise, and more markedlyafter exercise + diet. Serum levels of lactate dehydrogenase (LDH) significantly decreased after exercise + diet; both creatine kinase (CK) and LDH levels were significantly reduced after exercise + diet compared to exercise. Pulmonary function showed no changes after control and exercise, whereas a significant improvement of forced expiratory volume in 1 sec (FEV1) was observed after exercise + diet. SF36 showed a slight improvement in the "mental component" scale after exercise, and a significant improvement in "general health" and "vitality" after exercise + diet. The compliance to prescriptions was higher than 70% for both diet and exercise. CONCLUSIONS: Exercise tolerance (as evaluated by peak aerobic power) showed a tendency to decrease in LOPD patients on long term ERT. Exercise training, particularly if combined with high-protein diet, could reverse this decrease and result in an improvement, which was accompanied by improved quality of life. The association of the two lifestyle interventions resulted also in a reduction of muscle enzyme levels and improved pulmonary function.

Changes in Skeletal Muscle Oxidative Capacity After a Trail Running Race.

PURPOSE: to evaluate the effects of a trail-running race on muscle oxidative function by measuring pulmonary gas exchange variables and muscle fractional O2 extraction. METHODS: Eighteen athletes were evaluated before (PRE) and after (POST) a trail running competition of 32-km or 50-km with 2000 m or 3500 m of elevation gain, respectively. During the week before the race, runners performed an incremental uphill running test and an incremental exercise by utilizing a one-leg knee-extension (KE) ergometer. The KE exercise was repeated after the end of the race. During the KE test we measured oxygen uptake (V'O2) and micromolar changes in deoxygenated hemoglobin (Hb)+myoglobin (Mb) concentrations (Δ[deoxy(Hb+Mb)]) on vastus lateralis with a portable near-infrared spectroscopy. RESULTS: V'O2peak was lower at POST vs. PRE (-23.9±9.0%, p<0.001). V'O2peak at POST was lower than V'O2 at the same workload at PRE (-8.4±15.6%, p<0.050). Peak power output and time to exhaustion decreased at POST by -23.7±14.3% and -18.3±11.3%, respectively (p<0.005). At POST the increase of Δ[deoxy(Hb + Mb)] as a function of work rate, from unloaded to peak, was less pronounced (from 20.2±10.1 to 64.5±21.1% of limb ischemia at PRE to 16.9±12.7 to 44.0±18.9% at POST). Peak Δ[deoxy(Hb+Mb)] values were lower at POST (by -31.2±20.5%; p<0.001). CONCLUSIONS: trail running leads to impairment in skeletal muscle oxidative metabolism, possibly related to muscle damage from repeated eccentric contractions. In association with other mechanisms, the impairment of skeletal muscle oxidative metabolism is likely responsible of the reduced exercise capacity and tolerance during and following these races.

Aging effects on prefrontal cortex oxygenation in a posture-cognition dual-task: an fNIRS pilot study.

BACKGROUND: The aging process alters upright posture and locomotion control from an automatically processed to a more cortically controlled one. The present study investigated a postural-cognitive dual-task paradigm in young and older adults using functional Near-Infrared Spectroscopy (fNIRS). METHODS: Twenty healthy participants (10 older adults 72 ± 3 y, 10 young adults 23 ± 3 y) performed a cognitive (serial subtractions) and a postural task (tandem stance) as single-tasks (ST) and concurrently as a dual-task (DT) while the oxygenation levels of the dorsolateral prefrontal cortex (DLPFC) were measured. RESULTS: In the cognitive task, young adults performed better than older adults in both conditions (ST and DT) and could further increase the number of correct answers from ST to DT (all ps ≤ 0.027) while no change was found for older adults. No significant effects were found for the postural performance. Cerebral oxygenation values (O2Hb) increased significantly from baseline to the postural ST (p = 0.033), and from baseline to the DT (p = 0.031) whereas no changes were found in deoxygenated hemoglobin (HHb). Finally, the perceived exertion differed between all conditions (p ≤ 0.003) except for the postural ST and the DT (p = 0.204). CONCLUSIONS: There was a general lack of age-related changes except the better cognitive performance under motor-cognitive conditions in young compared to older adults. However, the current results point out that DLPFC is influenced more strongly by postural than cognitive load. Future studies should assess the different modalities of cognitive as well as postural load.

Short-Term Effects of Rolling Massage on Energy Cost of Running and Power of the Lower Limbs.

PURPOSE: Self-myofascial release (SMFR) is a type of self-massage that is becoming popular among athletes. However, SMFR's effects on running performance have not yet been investigated. The aim of this study was to evaluate the effects of SMFR on the cost of running (Cr). In addition, the authors evaluated the effects of SMFR on lower-limb muscle power. METHODS: Cr and lower-limb muscle power during squat jump (SJ) and countermovement jump (CMJ) were measured before (PRE), immediately after (POST), and 3 h after (POST 3h) an SMFR protocol (experimental condition). In the control-condition testing session, the same measurements were performed without undergoing the SMFR protocol. Experimental and control conditions were tested in a randomized order. RESULTS: Cr at POST trended to increase compared with PRE (+6.2% [8.3%], P = .052), whereas at POST 3h, Cr was restored to PRE values (+0.28% [9.5%], P = .950). In the experimental condition, no significant effect of time was observed for maximal power exerted during SJ. By contrast, maximal power exerted during CMJ at POST and at POST 3h was significantly higher than that observed at PRE (+7.9% [6.3%], P = .002 and +10.0% [8.7%], P = .004, respectively). The rate of force development measured during CMJ also increased after SMFR, reaching statistical significance at 200 ms from force onset at POST 3h (+38.9%, P = .024). CONCLUSIONS: An acute use of foam rollers for SMFR performed immediately prior to running may negatively affect endurance running performance, but its use should be added before explosive motor performances that include stretch-shortening cycles.