ALS (Amyotrophic Lateral Sclerosis)
Conditions
Keywords
Amyotrophic Lateral Sclerosis, ALS, Diaphragmatic Endurance Test
Brief summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease which involves respiratory muscles and can lead at short term to respiratory failure. The occurrence of respiratory failure is associated with morbidity and an increased mortality. To date, respiratory muscle weakness is predicted from the reduction of vital capacity, maximal inspiratory force, nocturnal symptoms and hypercapnia. Even taken together, the predictive value of these indices is low. The investigators hypothesize that an endurance test of diaphragmatic work would be more sensitive to respiratory muscle involvement than maximal respiratory force. Consequently, the investigators assessed diaphragmatic performance through an isocapnic hyperventilation test (IHT) in patients at the onset of ALS and, then regularly up to the occurrence of respiratory failure. The investigators make the hypothesis that IHT will be altered earlier than maximal inspiratory force
Interventions
OTHERDiaphragmatic endurance test
This consists of hyperventilation in a flask while maintaining the constant level of Carbon Dioxide (CO2). The patient will realize hyperventilation to increasing levels of Maximum Minute Ventilation (MMV), corresponding to increasing levels of respiratory rate, until exhaustion
Sponsors
Centre Hospitalier Universitaire de Saint Etienne
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
Yes
Inclusion criteria
for Patients : * Older than 18 years old and younger than 80 years old * ALS patient seen during the diagnostic assessment Inclusion Criteria for control group : * Older than 25 years old and younger than 80 years old * No respiratory or neurologic active pathology
Exclusion criteria
for Patients : * Bulbar ALS (inability to perform maximal respiratory maneuvers) * Dementia * Respiratory failure at diagnosis (arterial carbon dioxide partial pressure (pCO2) \> 45 mmHg) * Respiratory or neurologic active pathology
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Endurance time | Day 1 | Difference in endurance time during the IHT between healthy controls and ALS patients when diagnosis is established |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Reference values of the diaphragmatic endurance test | Day 1 | Reference values of the diaphragmatic endurance test in healthy controls according to age, by 10 years range. |
| Reference values of phrenic nerve activity | Day 1 | Reference values of phrenic nerve activity in healthy controls according to age. It is measured with diaphragmatic Electromyogram by cervical electrical stimulation. |
| Slope of endurance time decrease | At Day 1 and every 3 months of follow-up (3 years) | Slope of endurance time decrease with time in ALS patients measured during IHT |
| Amplitude of phrenic nerve | At Day 1 and every 3 months of follow-up (3 years) | It is the relationship between phrenic nerve activity and diaphragmatic endurance in ALS patients |
| Latency of phrenic nerve | At Day 1 and every 3 months of follow-up (3 years) | It is the relationship between phrenic nerve activity and diaphragmatic endurance in ALS patients |
Countries
France
Outcome results
None listed