QCT in ALS Diagnosis, Mechanistic Understanding and Follow-up (PEM-SLA)

Contribution of the Combined Quadriceps Test (QCT) in the Diagnosis, Mechanistic Understanding and Follow-up of Amyotrophic Lateral Sclerosis

Multidisciplinary management of amyotrophic lateral sclerosis (ALS) can significantly increase survival but also improve the quality of life of patients. The evaluation of cortical-spinal motor neuron damage is currently based only on the assessment of clinical data. However, the alteration of the central motor pathway and conduction can be identified and quantified by different techniques using motor-evoked potentials (MEP). The combined quadriceps test (QCT) has been developed to assess central and peripheral motor pathway conduction. This test allows to quantify central and peripheral part of a mixed disorder, and to detect physiological hyporeflexia or hyperreflexia which, in the case of suspected ALS, can lead to interpretation problems.

The evolution of the QCT parameters during the course of pathology will lead to determine the preponderance of an initial central involvement, but also its extension throughout the pathology. The study of these parameters as well as the clinical course of the disease could reveal a correlation between peripheral and central involvement. This link would provide arguments in favor of pathophysiological hypotheses of disease onset and progression. From a prognostic point of view and depending on the quantification of central and peripheral involvement, the QCT would make it possible to characterize the different ALS phenotypes. This phenotypic characterization would help identify prognostic factors at diagnosis.

The investigators propose a cohort study with the exploration of central motor neuron damage by QCT during the course of ALS in order to provide arguments for a better mechanistic understanding and follow-up of this disease with a poor prognosis.

Study Overview

• Status: Recruiting
• Conditions: Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Diagnostic test: Quadriceps Combined Test

• Study Type: Interventional
• Enrollment (Estimated): 30
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: LISE LACLAUTRE; Phone Number: +33473754963; Email: promo_interne_drci@chu-clermontferrand.fr

Study Locations

• France
  • Clermont-Ferrand, France, 63000
    • Recruiting
    • CHU de Clermont-Ferrand
    • Contact: LISE LACLAUTRE; Phone Number: +33473754963; Email: promo_interne_drci@chu-clermontferrand.fr
    • Principal Investigator: Nathalie Guy

Participation Criteria

Eligibility Criteria

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

Description

Inclusion Criteria:

• A male or female patient of legal age with suspected ALS (bulbar or spinal) who meets the criteria for "possible", "probable" or "definite" ALS according to the Awaji criteria
• Able to give informed consent to participate in the research
• Enrolled in a Social Security plan

Exclusion Criteria:

• Pregnant or breastfeeding woman
• Severe progressive pathology other than ALS.
• Comorbidities with another neurological disease altering motor skills.
• Contraindication to trans-cranial magnetic stimulation: epilepsy, pacemaker, intracranial ferromagnetic foreign body (clip, aneurysm, implants)...
• Chronic alcoholism
• Cognitive disorders or major incapacity making it impossible to understand the study and sign an informed consent (fronto-temporal dementia, psychiatric conditions of psychotic type, language disorders)
• Refusal to participate.
• Patients under legal protection (guardianship, curators, safeguard of justice)

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Diagnostic
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm

Intervention / Treatment

Other: Quadriceps Combined Test

Diagnostic test: Quadriceps Combined Test; The recording of the motor response on the quadriceps is done using self-adhesive surface electrodes on the motor point of the vastus medialis muscles (active electrodes) and on the patella (reference electrode), using a classic electromyography device that allows calculations and measurements of amplitude, duration and speed.; Other Names: Motor- Evoked Potentials

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Parameters from the QCT (Quadriceps Combined Test)	Measure of amplitude of M	At inclusion
Parameters from the QCT (Quadriceps Combined Test)	Measure of amplitude of M	Month 6
Parameters from the QCT (Quadriceps Combined Test)	Measure of amplitude of M	Month 12
Parameters from the QCT (Quadriceps Combined Test)	Measure of amplitude of M	month 18
Parameters from the QCT (Quadriceps Combined Test)	Measure of amplitude of M	month 24
Parameters from the QCT (Quadriceps Combined Test)	measure of TCP response,	At inclusion
Parameters from the QCT (Quadriceps Combined Test)	measure of TCP response,	month 6
Parameters from the QCT (Quadriceps Combined Test)	measure of TCP response,	month 12
Parameters from the QCT (Quadriceps Combined Test)	measure of TCP response,	month 18
Parameters from the QCT (Quadriceps Combined Test)	measure of TCP response,	month 24
Parameters from the QCT (Quadriceps Combined Test)	measure of TCC response	At inclusion
Parameters from the QCT (Quadriceps Combined Test)	measure of TCC response	month 6
Parameters from the QCT (Quadriceps Combined Test)	measure of TCC response	month 12
Parameters from the QCT (Quadriceps Combined Test)	measure of TCC response	month 18
Parameters from the QCT (Quadriceps Combined Test)	measure of TCC response	month 24
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of amplitude	At inclusion
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of amplitude	month 6
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of amplitude	month 12
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of amplitude	month 18
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of amplitude	month 24
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP latency ratio	At inclusion
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP latency ratio	month 6
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP latency ratio	month 12
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP latency ratio	month 18
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP latency ratio	month 24
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP amplitude ratio.	At inclusion
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP amplitude ratio.	month 6
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP amplitude ratio.	month 12
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP amplitude ratio.	month 18
Parameters from the QCT (Quadriceps Combined Test)	ratio calculation of T/MEP amplitude ratio.	month 24

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Clinical and paraclinical likely course data	ALSFRS-R (Amyotrophic Lateral Sclerosis Functional Rating) scale score. Score from 0 to 48.	At inclusion
Clinical and paraclinical likely course data	Spirometry data : Slow Vital Capacity	At inclusion
Clinical and paraclinical likely course data	Spirometry data : Maximal Inspiratory Pressure	At inclusion
Clinical and paraclinical likely course data	Spirometry data : Maximal Exhalation Pressure	At inclusion
Clinical and paraclinical likely course data	Weight	At inclusion
Clinical and paraclinical likely course data	Percentage of weight loss	At inclusion
Clinical and paraclinical likely course data	Oxymetry	At inclusion
Clinical and paraclinical likely course data	Non-Invasive Ventilation implementation (yes or no)	At inclusion
Clinical and paraclinical likely course data	Gastrostomy implementation (yes or no)	At inclusion
Clinical and paraclinical likely course data	Survival	At inclusion
Clinical and paraclinical likely course data	Spirometry data : Slow Vital Capacity	Every 3 months up to 2 years
Clinical and paraclinical likely course data	Spirometry data : Maximal Inspiratory Pressure	Every 3 months up to 2 years
Clinical and paraclinical likely course data	Spirometry data : Maximal Exhalation Pressure	Every 3 months up to 2 years
Clinical and paraclinical likely course data	Weight	Every 3 months up to 2 years
Clinical and paraclinical likely course data	Percentage of weight loss	Every 3 months up to 2 years
Clinical and paraclinical likely course data	Oximetry	Every 3 months up to 2 years
Clinical and paraclinical likely course data	Non-Invasive Ventilation Implementation (yes or no)	Every 3 months up to 2 years
Clinical and paraclinical likely course data	Gastrostomy implementation (yes or no)	Every 3 months up to 2 years
Clinical and paraclinical likely course data	Survival	Every 3 months up to 2 years
Clinical and paraclinical likely course data	MRC (Medical Research Council) scale for the evaluation of muscular strength. Score from 0 to 5. Higher score means normal movement	At inclusion
Clinical and paraclinical likely course data	MRC (Medical Research Council) scale for the evaluation of muscular strength. Score from 0 to 5. Higher score means normal movement	Every 3 months up to 2 years
Clinical and paraclinical likely course data	ALSFRS-R (Amyotrophic Lateral Sclerosis Functional Rating) scale score. Score from 0 to 48.	Every 3 months up to 2 years

Collaborators and Investigators

• Sponsor: University Hospital, Clermont-Ferrand
• Investigators: Principal Investigator: Nathalie Guy, University Hospital, Clermont-Ferrand

Study record dates

Study Major Dates

• Study Start (Actual): June 28, 2022
• Primary Completion (Estimated): June 28, 2025
• Study Completion (Estimated): February 28, 2026

Study Registration Dates

• First Submitted: June 21, 2022
• First Submitted That Met QC Criteria: February 21, 2024
• First Posted (Actual): February 28, 2024

Study Record Updates

• Last Update Posted (Actual): July 3, 2024
• Last Update Submitted That Met QC Criteria: July 1, 2024
• Last Verified: February 1, 2024

More Information

Terms related to this study

• Keywords: Amyotrophic Lateral Sclerosis; Quadriceps Combined Test
• Additional Relevant MeSH Terms: Pathologic Processes; Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Sclerosis; Motor Neuron Disease; Amyotrophic Lateral Sclerosis
• Other Study ID Numbers: RBHP 2020 GUY; 2020-A02765-34 (Other Identifier: ANSM)

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No