Respiratory insufficiency in facioscapulohumeral muscular dystrophy has rarely been studied. We compared two age- and sex-matched groups of 29 patients, with and without respiratory dysfunction.… (More)
INTRODUCTION
Mouthpiece ventilation represents a valuable treatment for patients needing daytime non-invasive ventilation. This modality is however underused, in part because of limitations in the… (More)
The aim of our study was to evaluate efficacy and reliability of currently available ventilators for mouthpiece ventilation (MPV). Five life-support home ventilators were assessed in a bench test… (More)
Respiratory muscle weakness and chest wall abnormalities in neuromuscular diseases (NMD) may lead to decreased pulmonary volumes. We assessed the reversibility of vital capacity (VC) reduction with… (More)
BACKGROUND
Many patients with DMD undergo tracheostomy. Tracheostomy is associated with certain complications, however its effect on prognosis is not known.
METHODS
The relationship between type of… (More)
This study evaluated compliance with non-invasive ventilation in patients with myotonic dystrophy type 1 and identified predictors of cessation at 5 years in a cohort of patients followed in a… (More)
Twitch mouth pressure using magnetic stimulation of the phrenic nerves and an automated inspiratory trigger is a noninvasive, non-volitional assessment of diaphragmatic strength. Our aims were to… (More)