An interventional study of diagnosis variables in Amyotrophic Lateral Sclerosis, sponsored by University Hospital, Toulouse. Status unknown at 1 site in France. Open to participants aged 18 Years and older. Per ClinicalTrials.gov, last updated 2021-09-01.
Sponsored by University Hospital, Toulouse · Not applicable, Interventional, and Diagnostic
To compensate for insufficiency of diagnostic tools, the present study propose to look for the predictive factors of an early fitting by noninvasive ventilation.
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease. The diagnosis of this diaphragmatic dysfunction in the ALS subject is done all too often on the occasion of a hospitalization for acute respiratory insufficiency, which aggravates then the prognosis of the patients.
The presence of these factors at diagnosis will allow more surveillance to detect early diaphragmatic insufficiency and establish a NIV (Non Invasive Ventilation), the only therapeutic measure definitely improving the quality of life and survival of patients. A clinical score will be established to determine the risk of early Non Invasive Ventilation (NIV) equipment.
Exclusion Criteria:
Determination of diagnosis variables in a group of patient who benefit of the Non Invasive ventilation equipment one year after the diagnostic
Diagnostic Test: diagnosis variables
Determination of diagnosis variables in a group of patient who not benefit of the Non Invasive ventilation equipment one year after the diagnostic
Diagnostic Test: diagnosis variables
The study compare the diagnostic variables in patients who received Non invasive ventilation equipment one year after diagnosis with the patient who don't need it. For the independent variables, the present study determine a threshold at the diagnosis for which an early fitting risk appears The diagnostic variable are : * Age of the patient * Sex * Presence of bulbar involvement or not * Time from onset of first symptoms to diagnosis of ALS * Score ALSFRS-R * Presence of another underlying respiratory pathology (COPD, asthma ...) * FVC measurement (Forced vital capacity) * PaCO2 measurement * Measure SNIP (Sniff nasal inspiratory pressure) * Measure of Pimax (Pressure inspiratory maximal) * Measurement of the nocturnal desaturation index (number of desaturation less than 90% per hour of sleep)
Early Surgical Clinical Score
An Early Surgical Clinical Score will be established taking into account the independent variables correlated to diagnosis at early fitting. Clinical Variables : * Presence of bulbar involvement or not * Time from onset of first symptoms to diagnosis * Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (ALSFRS-R) score : The Amyotrophic Lateral Sclerosis Functional Ra ting Scale (ALSFRS) is an instrument for evaluating the functional status of patients with Amyotrophic Lateral Sclerosis. It can be used to monitor functional change in a patient over time. * Presence of another underlying respiratory disease Functional Variables : * Time from onset of first symptoms to diagnosis of ALS * Presence of another underlying respiratory pathology * Forced Vital Capacity (FVC) measurement * PaCO2 measurement * Measure Sniff Nasal Inspiratory Pressure (SNIP) * Measurement of diaphragmatic activity on ultrasound * Parameters of the Polysomnography (PSG)
Time frame: One year after diagnostic
Sensitivity, specificity, negative and positive predictive values
Sensitivity, specificity, negative and positive predictive values of the diaphragmatic ultrasound (fraction of shortening and diaphragmatic stroke) compared to the SNIP test and for the values of PSG (Polysomnography) compared to SNIP test
Time frame: One year after the diagnostic
Percentage of decrease
% decrease in shortening fraction and diaphragmatic stroke between two quarterly reevaluations correlated with early fitting and % decrease in FVC, SNIP test et Pimax
Time frame: One year after the diagnostic
Slope of FVC decline
Slope of FVC decline before and after NIV implementation and slope of decline of shortening fraction and diaphragmatic stroke before and after NIV
Time frame: One year after the diagnostic
Quality of life score
Quality of life score by the Severe Respiratory Insufficiency (SRI) questionnaire :before and after implementation of the NIV The Severe Respiratory Insufficiency (SRI) Questionnaire: a specific measure of health-related quality of life in patients receiving home mechanical ventilation.
Time frame: One year after the diagnostic
Plan to share: No
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University Hospital, Toulouse