Contribution of Diaphragmatic Ultrasound for Monitoring Diaphragmatic Function in Patients With Amyotrophic Lateral Sclerosis

NCT ID: NCT05352958

Last Updated: 2025-05-15

Basic Information

• Recruitment Status: RECRUITING
• Total Enrollment: 50 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2023-05-16
• Study Completion Date: 2028-11-30

Brief Summary

Amyotrophic lateral sclerosis (ALS) is a rare neuromuscular disease that occurs in adults. It is characterized by a progressive degeneration of the first and second motor neurons leading to muscle failure. In its spinal form, ALS manifests by a progressive worsening of limb involvement, whereas the bulbar form presents with swallowing disorders, dysarthria and feeding difficulties.

Respiratory impairment is the most serious feature of ALS. Phrenic nerve damage causes diaphragmatic weakness, which inevitably leads to chronic restrictive respiratory failure. At the stage of symptomatic nocturnal or diurnal alveolar hypoventilation, non-invasive ventilation (NIV) prolongs survival while improving quality of life by relieving respiratory symptoms.

The indication for the initiation of NIV is based on the appearance of respiratory symptoms but also on the demonstration of diaphragmatic insufficiency. A quarterly follow-up of diaphragmatic function has been recommended by the French Health Authority since 2006. It is based on functional respiratory explorations (VC in sitting and lying position, measurement of maximal inspiratory pressure) and screening for diurnal or nocturnal hypoventilation with the measurement of PaCO2 or the nocturnal recording of SpO2.

Access to these examinations remains limited and they are sometimes complex to perform (in particular issues with mouth occlusion during respiratory manoeuvres in case of bulbar damage). Thus, only 60% of patients undergo a complete evaluation. Moreover, these explorations are only late markers of diaphragmatic dysfunction, and it has recently been shown that they do not correlate with histological diaphragmatic amyotrophy.

The development of new, reliable, and easily available tools for the evaluation of diaphragmatic function, and that are capable of detecting diaphragmatic insufficiency early in the course of the disease, are therefore necessary. Such tools would make it easier to implement NIV at the optimal time, preventing episodes of acute respiratory distress.

Recently, diaphragmatic ultrasound has appeared in the ICU as a new tool for assessing diaphragmatic function. It has the advantage of being highly available, inexpensive, non-irradiating, quick to perform, reproducible and very sensitive and specific for the diagnosis of diaphragmatic dysfunction.

In ALS, few studies have investigated the contribution of ultrasound for the diagnosis or follow-up of diaphragmatic dysfunction. In addition, no study so far has compared diaphragmatic ultrasound to complete pulmonary function test (PFT) data or to direct measurement of diaphragmatic pressure (Pdi). Very few publications report the how the diaphragm changes on ultrasound imaging during the disease. Moreover, these studies do not analyse the interest of diaphragmatic ultrasound in the prediction of progression towards respiratory failure with respiratory support, or death. Finally, these studies use different ultrasound measurements of the diaphragm (stroke, thickness, thickening fraction, and thickening fraction ratio, among others) rather than a simple, consensual parameter.

The aim of this study is to describe the evolution of diaphragmatic ultrasound parameters, to identify the parameter that best correlates with other respiratory measures (PFT, PaCO2, nocturnal oximetry) and to determine the prognostic value of diaphragmatic ultrasound in predicting the initiation of NIV or death at 6 and 12 months.

Conditions

Amyotrophic Lateral Sclerosis

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

Patients with Amyotrophic Lateral Sclerosis

ALS

• certain or probable according to the revised Escorial criteria
• no indication for non-invasive ventilation

Diaphragmatic ultrasound

Intervention Type: PROCEDURE

duration 15 minutes, performed at M0, M3, M6 and M9

Electro-physiological exploration of the diaphragm

Intervention Type: OTHER

duration between 30 and 60 minutes performed at M0

Interventions

Diaphragmatic ultrasound

duration 15 minutes, performed at M0, M3, M6 and M9

Intervention Type: PROCEDURE

Electro-physiological exploration of the diaphragm

duration between 30 and 60 minutes performed at M0

Intervention Type: OTHER

Eligibility Criteria

Inclusion Criteria

• Patient with Amyotrophic Lateral Sclerosis

• Certain or probable according to the revised Escorial criteria
• no indication for non-invasive ventilation
• Adult patient
• Patient who has given oral consent
• Patient who speaks and reads French

Exclusion Criteria

• Patients with pre-existing diaphragmatic dysfunction requiring non-invasive ventilation
• Person who is not affiliated to national health insurance
• Person subject to a legal protection measure (curatorship, guardianship)
• Patients deprived of their liberty
• Pregnant, parturient or breastfeeding women

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Centre Hospitalier Universitaire Dijon

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Locations

Chu Dijon Bourogne

Dijon, , France

Site Status: RECRUITING

Countries

France

Central Contacts

Marjolaine GEORGES

Role: CONTACT

marjolaine.georges@chu-dijon.fr

03.80.29.37.72

Facility Contacts

Marjolaine GEORGES

Role: primary

marjolaine.georges@chu-dijon.fr

03.80.29.37.72

Other Identifiers

GEORGES 2021

Identifier Type: -

Identifier Source: org_study_id