Magnetic Imaging for Diagnostic of Amyotrophic Lateral Sclerosis (MIDALS)

Contribution of Whole Body Muscle MRI for Early Diagnosis of Amyotrophic Lateral Sclerosis.

Nearly 60% of Amyotrophic Lateral Sclerosis (ALS) patients have a low level of diagnostic certainty (possible, probable) at the time of diagnosis. In the absence of biomarkers, this diagnosis is based, among other things, on the demonstration of the diffusion of signs of denervation by electroneuromyography (ENMG). The objective of this study is to improve the earliness and the level of diagnostic certainty by better demonstrating the diffusion of the denervation process by whole body muscular MRI.

Study Overview

• Status: Recruiting
• Conditions: Amyotrophic Lateral Sclerosis With Dementia
• Intervention / Treatment: Diagnostic test: Whole Body MRI and ENMG

Detailed Description

The objective of this study is to show that the combination of ENMG and whole body muscle MRI (WB-MRI) can increase the diagnosis of definite ALS at the onset of the disease For 50 consecutive patients with suspected ALS, the present study will prospectively perform needle electromyography (EMG) and muscle analyses on whole body MRI. The diagnostic category will be determined by revised El Escorial criteria and Awaji criteria. On whole body MRI acquisition, for 83 muscles, will be explored fatty infiltration and atrophy on fat images (chronic denervation) and muscle edema on water images (acute denervation). EMG studies will be performed in at least 10 muscles (tongue, biceps brachii, first dorsalis interosseous, T10 paraspinalis, vastus lateralis, and tibialis anterior). Diagnostic classification according EMG and/ or MRI abnormalities in bulbar and the 3 spinal regions will be compared independently.

• Study Type: Interventional
• Enrollment (Estimated): 50
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Pascal CINTAS, MD PHD; Phone Number: 05 61 77 94 40; Email: cintas.p@chu-toulouse.fr
• Study Contact Backup: Name: Anne Cecile Coville; Phone Number: (05345) 58700

Study Locations

• France
  • Toulouse, France, 31000
    • Recruiting
    • Pascal CINTAS
    • Contact: Pascal CINTAS, MD PHD; Phone Number: 0561779440; Email: cintas.p@chu-toulouse.fr
    • Contact: Anne Cecile Coville; Phone Number: 0534558700

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 99 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

• Consent form signed by the patient
• Patients suspected ALS defined according Awaji Shima criteria (possible, probable, defined)
• Clinical assessment of upper motor neuron involvement
• Electrophysiologic assessment of lower motor neuron involvement

Exclusion Criteria:

• inability to give informed consent
• a contraindication to MRI
• respiratory failure impairing ability to lie flat in the scanner.
• Patient placed under judicial protection or under another protective regime,
• Females who are pregnant

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
Experimental: ENMG and muscle analyses on whole body muscles MRI.; Diffusion of active and chronic muscle denervation will be assessed on ENMG and whole body muscle MRI. The diagnostic category will be determined by revised El Escorial criteria and Awaji criteria	Diagnostic test: Whole Body MRI and ENMG; Whole body muscle MRI lasting 30 to 45 min without injection of contrast medium an ENMG

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Diagnostic certainty 1.ENMG according to El Escorial revised criteria using ENMG alone, MRI alone and ENMG+MRI to define denervation diffusion	number of patients classified as possible, probable or certain according to El Escorial criteria using ENMG alone, IMR alone and ENMR+MRI	1 month

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Define precise topographic and characteristic of muscle involvement in ALS by MRI	number of patients with concordance between ENMG and MRI for the detection of denervation	1 month

Collaborators and Investigators

• Sponsor: University Hospital, Toulouse
• Investigators: Principal Investigator: Pascal CINTAS, MD, University Hospital, Toulouse

Study record dates

Study Major Dates

• Study Start (Actual): June 1, 2021
• Primary Completion (Estimated): December 1, 2024
• Study Completion (Estimated): December 1, 2024

Study Registration Dates

• First Submitted: March 26, 2021
• First Submitted That Met QC Criteria: April 27, 2021
• First Posted (Actual): May 3, 2021

Study Record Updates

• Last Update Posted (Actual): August 2, 2024
• Last Update Submitted That Met QC Criteria: August 1, 2024
• Last Verified: August 1, 2024

More Information

Terms related to this study

• Keywords: Sclerosis; Amyotrophic
• 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: RC31/20/0217

Drug and device information, study documents

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