Outcome measures and rehabilitation treatment in patients affected by Charcot-Marie-Tooth neuropathy: a pilot study.

OBJECTIVE: : We evaluated the sensitivity of various rehabilitation and lung function scales to detect differences between people with Charcot-Marie-Tooth (CMT) disease and healthy controls. We also studied whether these measurements are sensitive to disclose changes in patients with CMT disease after rehabilitative treatment. DESIGN: : Eight patients with different types of CMT participated in the study. Data were gathered at baseline; at the end of the treadmill training, stretching, respiratory, and proprioceptive exercise (TreSPE) treatment period; and after a washout period of 6 mos. The following instruments were used for data collection: Medical Research Council scale for lower limb strength; Tinetti Balance Scale; Physical Performance Battery; ankle angle, oxygen consumption, and lung function tests; peak treadmill velocity and slope; time to walk 6 m; and CMT Neuropathy Score. The participants underwent TreSPE treatment twice every week for 8 wks. RESULTS: : All rehabilitative measures were significantly worse in subjects with CMT disease than in healthy controls. Lung function was always normal except for the maximum expiratory pressure and maximum inspiratory pressure. No dropouts or worsening in any of the different outcome measures were observed after TreSPE. The ankle angle and the time to walk 6 m were the only measures that significantly improved after treatment. CONCLUSIONS: : The rehabilitative outcome measures used in this protocol are useful in detecting clinical impairment in people with CMT disease. Lung function tests were confirmed to be minimally abnormal in patients with CMT disease. The TreSPE treatment, besides being safe and well tolerated, induced some training effects in CMT neuropathy, in the absence of lung function amelioration and effort tolerance. Follow-up showed that CMT patients should be treated at least twice every year because a regression of all outcome measures to the baseline state was found after a 6-mo washout period.

Does a low-density gas mixture or oxygen supplementation improve exercise training in COPD?

BACKGROUND: Low-density gas mixtures and oxygen (O2) supplementation are known to improve physical performance and ventilatory adaptation during incremental maximal exercise in COPD. We investigated whether their combined use during intense physical training is also effective in ameliorating exercise tolerance in patients affected by moderate to severe COPD. METHODS: Thirty patients (FEV1 < 60% of predicted) underwent a 2-month rehabilitation program. Leg-cycle training was conducted thrice weekly at 80% of the initial peak work rate for at least 20 min breathing room air, a 60% helium and 40% O2 mixture, or supplemental O2 (40%) inhaled from a Douglas bag. The study was randomized with a double-blind design. Before and at the end of the training period the subjects were tested for spirometry, arterial gas tension, diffusing lung capacity for CO, and incremental and constant work rate exercise test on a cycle ergometer. RESULTS: Physical training resulted in a significant improvement in peak oxygen consumption and power output (P < .01 for both) and in exercise endurance time (P < .05) independently of the kind of inhaled gas during the rehabilitation program (P ≥ .42). No changes were observed in lung function tests or gas exchange. CONCLUSIONS: We concluded that the use of a low-density gas mixture or O2 supplementation does not contribute to improved exercise performance in patients with moderate to severe COPD without a significantly decreased diffusion lung capacity for CO who are able to tolerate intense physical training.

Bronchodilator effects of exercise hyperpnea and albuterol in mild-to-moderate asthma.

In asthmatic patients, either bronchodilatation or bronchoconstriction may develop during exercise. In 18 patients with mild-to-moderate asthma, we conducted two studies with the aims to 1) quantify the bronchodilator effect of hyperpnea induced by incremental-load maximum exercise compared with effects of inhaled albuterol (study 1, n=10) and 2) determine the time course of changes in airway caliber during prolonged constant-load exercise (study 2, n=8). In both studies, it was also investigated whether the bronchodilator effects of exercise hyperpnea and albuterol are additive. Changes in airway caliber were measured by changes in partial forced expiratory flow. In study 1, incremental-load exercise was associated with a bronchodilatation that was approximately 60% of the maximal bronchodilatation obtainable with 1,500 microg of albuterol. In study 2, constant-load exercise was associated with an initial moderate bronchodilatation and a late airway renarrowing. In both studies, premedication with inhaled albuterol (400 microg) promoted sustained bronchodilatation during exercise, which was additive to that caused by exercise hyperpnea. In conclusion, in mild-to-moderate asthmatic individuals, hyperpnea at peak exercise was associated with a potent yet not complete bronchodilatation. During constant-load exercise, a transient bronchodilatation was followed by airway renarrowing, suggesting prevalence of constrictor over dilator effects of hyperpnea. Finally, the bronchodilator effect of hyperpnea was additive to that of albuterol.

Airway responsiveness to methacholine and deep inhalations in subjects with rhinitis without asthma.

BACKGROUND: Airway hyperresponsiveness in asthma is believed to be caused in part by the inability of deep inspirations to modulate airway narrowing. OBJECTIVE: We investigated whether deep inspirations taken before or after methacholine inhalation attenuate bronchoconstriction in subjects with rhinitis. The results were compared with a group of healthy subjects. METHODS: Ten subjects with rhinitis without asthma and 10 healthy subjects were studied on 3 different occasions at random. Bronchial challenges were performed with a single dose of methacholine known to decrease the FEV(1) by 17% to 40%. Challenges were performed with avoidance of deep inspirations, or with 5 deep inspirations preceding or following the inhalation of methacholine. Lung function measurements were specific airway conductance, forced expiratory flow at 30% to 40% of vital capacity on a maneuver started from end-tidal inspiration (partial flow), and residual volume (partial residual volume). RESULTS: In healthy subjects, deep inspirations taken after methacholine caused less changes in specific airway conductance, partial flow, and partial residual volume (P < .005 for all) than deep inspirations taken before methacholine or avoidance. In subjects with rhinitis, methacholine produced similar functional changes independently of the presence or absence of any deep inspirations. Compared with normal subjects, the attenuating effects of deep inspirations after methacholine on partial flow and partial residual volume were blunted in the subjects with rhinitis (P = .02 and P = .05, respectively). CONCLUSION: The ability to dilate methacholine-constricted airways by deep inspirations is impaired in subjects with rhinitis, possibly because of an abnormal behavior of airway smooth muscle.