View clinical trials related to Neuromuscular Disorder.
Filter by:The purpose of this research repository is to collect, store, and share with other researchers any tissues that subjects with all types of neuromuscular disease are willing to donate. These samples will be stored at Virginia Commonwealth University (VCU) and will be used for future research with this population.
Mechanical ventilation is one of the only treatment that has improved survival of patients with neuromuscular respiratory failure. As disease progresses, some patients may require longer ventilation period. Non invasive mechanical ventilation is the preferred method of ventilation but it may interfere with speech and communication of patients who require ventilation throughout the day. The investigators are evaluating the effect on speech and communication of a ventilation device which allows patients to momentarily and voluntarily withhold ventilation if they want to speak. This should allow the patients to have a more fluid speech.
The aim of this study is to better discriminate respiratory muscle dysfunction by comparing the measurements of thoracoabdominal motion obtained by an optoelectronic recording and the conventional tests of respiratory muscle strength. The final objective is to better select in the future the patients who need more specific assessment of diaphragmatic function like "maximal transdiaphragmatic pressure" measurement and "phrenic nerve stimulation".
Neuromuscular disorders can be associated with swallowing dysfunction secondary to a dysfunction of the airway muscles involved in swallowing. The investigators have shown that respiratory failure may contribute to swallowing dysfunction in patients with neuromuscular respiratory failure. Furthermore, although tracheostomy has been reported as impairing swallowing, the investigators have shown that when a tracheostomy is performed in neuromuscular patients, swallowing improves because it allows the patient to feed while ventilated. The investigators now want to evaluate whether non invasive ventilation may have a beneficial impact on swallowing by making some adjustments to ensure a good synchronisation between ventilation and swallowing. This could allow avoiding the necessity of a tracheostomy or a gastrostomy due to swallowing dysfunction and/or malnutrition in neuromuscular patients. Swallowing improvement under mechanical ventilation depends on improving the synchronisation between the patient and the ventilator during swallowing. For that purpose, the investigators developed a prototype ventilator able to temporarily suspend pressurisation under the patient's control so that when the patient needs to swallow under mechanical ventilation he may do so with an inadequate insufflation of the ventilator. Our objective is to to demonstrate that swallowing is more adapted and easier under nasal noninvasive ventilation than during spontaneous breathing in neuromuscular patients requiring prolonged noninvasive ventilation. In an open monocentric pilot study, the investigators will study 10 neuromuscular patients usually noninvasively ventilated. The patients will be their own control and their swallow will be studied during spontaneous breathing and under ventilation with the adapted ventilator while swallowing boluses of different volumes.