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Amyotrophic lateral sclerosis (ALS) is a rare and serious neurodegenerative disease causing degeneration of motor neurons. . It leads to a progressive paralysis of the muscles involved in voluntary motricity. In France, its incidence is 2.5/100,000 inhabitants per year.
The death of patients is mainly caused by a progressive attack of the respiratory muscles. Indeed, the thorax is no longer actively mobilized to the maximum amplitude, it will lose its flexibility. A restrictive syndrome sets in followed by alveolar hypoventilation. Bronchial congestion may be concomitant.
Management is then based on non-invasive ventilation (NIV). This step, which is difficult for patients to accept psychologically, must be delayed as much as possible. However, to date, there are no precise recommendations on preventing the appearance of this restrictive syndrome and on slowing down the deterioration of lung function in patients.
The pressure relaxer (RLX) is an instrumental aid allowing on the one hand to mobilize the thorax thanks to hyper insufflations, and on the other hand to increase the effectiveness of the cough. The use of this device in physiotherapy is part of the HAS recommendations to promote decluttering.
However, we believe that RLX in patients with ALS, through the pulmonary alveolar recruitment it induces, could be relevant at an earlier phase, for the prevention of the decline in pulmonary functions: the restrictive syndrome, bronchial congestion and alveolar hypoventilation. So ultimately, the quality of life and survival of these patients would be improved.
It is in this context that this multicenter randomized controlled study RELAX'SLA takes place in order to evaluate the effects of the early use of the pressure relaxer on the respiratory impairment of patients with ALS.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| standard care associated with daily and early use of (IPPB) intermittent positive pressure breathing | Experimental |
| |
| standard care alone | No Intervention |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| early use of intermittent positive pressure breathing | Device | standard care associated with daily and early use of intermittent positive pressure breathing |
|
| Measure | Description | Time Frame |
|---|---|---|
| assess the early use of the intermittent positive pressure breathing combined with standard management slows down the deterioration of lung volumes in patients with ALS | Comparison of changes in lung volumes between the two groups based on Forced Vital Capacity (FVC), measured by spirometry during a Functional Respiratory Test (LFE) | EVERY 3 MONTHS DURING 24 MONTHS |
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Inclusion Criteria:
Exclusion Criteria:
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| ID | Term |
|---|---|
| D000690 | Amyotrophic Lateral Sclerosis |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D016472 | Motor Neuron Disease |
| D019636 | Neurodegenerative Diseases |
| D057177 | TDP-43 Proteinopathies |
| D009468 | Neuromuscular Diseases |
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
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