Prognostic Value of a Diaphragmatic Endurance Test in Patients With Amyotrophic Lateral Sclerosis

NCT02528071 | Terminated

• 2 other identifiers: 13081882014-A00309-38
• Study Type: observational
• Target: 136
• Locations: 1 country
• Sites: 1

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

Conditions

ALS (Amyotrophic Lateral Sclerosis)

Keywords

Amyotrophic Lateral Sclerosis; ALS; Diaphragmatic Endurance Test

Outcome Measures

Primary Outcomes (1)

• Endurance time

  Difference in endurance time during the IHT between healthy controls and ALS patients when diagnosis is established

  Day 1

Secondary Outcomes (5)

• Reference values of the diaphragmatic endurance test

  Day 1

• Reference values of phrenic nerve activity

  Day 1

• Slope of endurance time decrease

  At Day 1 and every 3 months of follow-up (3 years)

• Amplitude of phrenic nerve

  At Day 1 and every 3 months of follow-up (3 years)

• Latency of phrenic nerve

  At Day 1 and every 3 months of follow-up (3 years)

Study Arms (2)

Patients

ALS patients performing measurements of maximal respiratory forces, diaphragmatic endurance during a diaphragmatic endurance test and phrenic nerve activity at the onset of the disease and repeated every 3 months up to respiratory failure or death

Other: Diaphragmatic endurance test

Control

Healthy controls performing measurements of maximal respiratory forces, diaphragmatic endurance during a diaphragmatic endurance test and phrenic nerve activity. This arm will enable to establish reference values of IHT

Other: Diaphragmatic endurance test

Interventions

Diaphragmatic endurance test OTHER

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

Control; Patients

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with ALS Healthly volonteers

You may qualify if:

• Older than 18 years old and younger than 80 years old
• ALS patient seen during the diagnostic assessment
• Older than 25 years old and younger than 80 years old
• No respiratory or neurologic active pathology

You may not qualify if:

• 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
• Chest wall deformation with spirometric defect

Sponsors & Collaborators

• Centre Hospitalier Universitaire de Saint Etienne: lead

Study Sites (1)

CHU de SAINT-ETIENNE

Saint-Etienne, 42000, France

Location

MeSH Terms

Conditions

Amyotrophic Lateral Sclerosis

Condition Hierarchy (Ancestors)

Spinal Cord Diseases; Central Nervous System Diseases; Nervous System Diseases; Motor Neuron Disease; Neurodegenerative Diseases; TDP-43 Proteinopathies; Neuromuscular Diseases; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases

Study Officials

• Frederic COSTES, MD PhD

  CHU de Clermont-Ferrand

  STUDY CHAIR

• Isabelle COURT-FORTUNE, MD

  CHU de Saint-Etienne

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: August 18, 2015
• First Posted: August 19, 2015
• Study Start: September 29, 2014
• Primary Completion: October 28, 2020
• Study Completion: October 28, 2020
• Last Updated: January 5, 2021

Record last verified: 2020-12

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)