Relationship Between Respiratory Functional Tests and Image Thoracic Techniques in Patients With Neuromuscular Diseases

Relationship Between Respiratory Functional Tests and Image Thoracic Techniques in Patients With Neuromuscular Diseases.

It is very important monitoring respiratory muscle function in patients with amyotrophic lateral sclerosis (ALS). We have at our disposal Respiratory Functional Tests (forced vital capacity (FVC), maximal mouth-inspiratory force (MIF), maximal mouth-expiratory force (MEF), Cough Peak expiratory flow (cPEF), maximal voluntary ventilation (MVV), arterial blood gases and nocturnal pulsioxymetry) and Thoracic Image Techniques (inspiratory/expiratory Thorax x-ray and x-ray scope). But all this explorations present some technique limitations. They are two new methods to explore diaphragmatic function: SNIF test and Diaphragm magnetic resonance imaging (dMRI). SNIF test is simple and easy to be done and it can avoid some problems that have the other respiratory functional test in this kind of patients. dMRI seems to correlate with respiratory functional test and it can be useful monitoring diaphragmatic mobility. The objective of our study is to compare and to correlate SNIF test and dMRI with x-ray techniques and respiratory functional tests that measure diaphragmatic force and function. First of all we will study 10 healthy people with a forced spirometry, MIF, MEF and dMRI, trying to obtain diaphragmatic mobility reference values with dMRI. Lately, we will study patients with ALS. In the first place, we will perform a transversal study with 30 patients. We will do a forced spirometry, MIF, MEF, MVV, cPEF, SNIF, arterial blood gases and nocturnal pulsioxymetry, forced Inspiratory and expiratory Thorax x-ray, diaphragmatic x-ray scope and dMRI. Secondly, we will perform a prospective study, where we will analyze these variables evolution at 3 and 6 months. We hope we can apply the two new techniques in the future because we think they are simpler, more accurate and more objective to evaluate diaphragmatic mobility.

Study Overview

• Status: Completed
• Conditions: Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Other: No intervention

• Study Type: Observational
• Enrollment (Actual): 5

Contacts and Locations

Study Locations

• Spain
  • Barcelona, Spain, 08025
    • Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant Pau

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients with amyotrophic lateral sclerosis.

Description

Inclusion Criteria:

• Amyotrophic lateral sclerosis patients.
• Clinically stable, at least for one month.

Exclusion Criteria:

• Claustrophobic patients.
• Patients using continuous non-invasive ventilation treatment.
• Incapacity to tolerate supine decubitus.
• Incapacity to execute respiratory functional tests.

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
1; Patients with amyotrophic lateral sclerosis.	Other: No intervention; We perform in all patients respiratory functional tests, thoracic x-ray, SNIF test and diaphragmatic magnetic resonance imaging.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Different measures of diaphragmatic mobility, force and function.	To compare and correlate all different image techniques that evaluate diaphragmatic mobility with all different respiratory functional variables that measure diaphragmatic (and muscular in general) force and function.	One year

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Diaphragmatic MRI usefulness	To evaluate dMRI usefulness in monitoring diaphragmatic mobility.; To evaluate if we can establish a value of diaphragm mobility (in mm) observed with dMRI to suggest the initiation of non invasive ventilation treatment.; To evaluate if dMRI can predict ALS prognosis.	One year

Collaborators and Investigators

• Sponsor: Fundació Institut de Recerca de l'Hospital de la Santa Creu i Sant Pau

Study record dates

Study Major Dates

• Study Start: June 1, 2007
• Primary Completion (Actual): July 1, 2013
• Study Completion (Actual): July 1, 2013

Study Registration Dates

• First Submitted: March 19, 2010
• First Submitted That Met QC Criteria: March 19, 2010
• First Posted (Estimate): March 22, 2010

Study Record Updates

• Last Update Posted (Estimate): February 24, 2014
• Last Update Submitted That Met QC Criteria: February 21, 2014
• Last Verified: February 1, 2014

More Information

Terms related to this study

• Keywords: mobility; Diaphragmatic force
• Additional Relevant MeSH Terms: Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Neuromuscular Diseases
• Other Study ID Numbers: PR01/01/2007