Clinical trial NCT02528071: Prognostic Value of a Diaphragmatic Endurance Test in Patients With Amyotrophic Lateral Sclerosis
Official title: Prognostic Value of a Diaphragmatic Endurance Test in Patients With Amyotrophic Lateral Sclerosis : Impact and Prognosis. Single-center Prospective Pilot Study
Study type: OBSERVATIONAL
Overall status: TERMINATED
Conditions: ALS (Amyotrophic Lateral Sclerosis)
Keywords: Amyotrophic Lateral Sclerosis
ALS
Diaphragmatic Endurance Test
Lead sponsor: Centre Hospitalier Universitaire de Saint Etienne
Sponsor class: OTHER
First posted: 2015-08-19
Enrollment: 136
Sex: ALL
Minimum age (years): 18
Maximum age (years): 80
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patients with ALS Healthly volonteers

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


Eligibility criteria: 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

Exclusion Criteria for control group :

* Chest wall deformation with spirometric defect

Primary outcomes:
measure: Endurance time
timeFrame: Day 1
Secondary outcomes:
measure: Reference values of the diaphragmatic endurance test
timeFrame: Day 1
measure: Reference values of phrenic nerve activity
timeFrame: Day 1
measure: Slope of endurance time decrease
timeFrame: At Day 1 and every 3 months of follow-up (3 years)
measure: Amplitude of phrenic nerve
timeFrame: At Day 1 and every 3 months of follow-up (3 years)
measure: Latency of phrenic nerve
timeFrame: At Day 1 and every 3 months of follow-up (3 years)
Other outcomes:
Locations:
city: Saint-etienne
country: France
facility: CHU de SAINT-ETIENNE
Study officials:
affiliation: CHU de Clermont-Ferrand
name: Frederic COSTES, MD PhD
role: STUDY_CHAIR
affiliation: CHU de Saint-Etienne
name: Isabelle COURT-FORTUNE, MD
role: PRINCIPAL_INVESTIGATOR
References: