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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| ENMG and muscle analyses on whole body muscles MRI. | Experimental | 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 |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Whole Body MRI and ENMG | Diagnostic Test | Whole body muscle MRI lasting 30 to 45 min without injection of contrast medium an ENMG |
|
| 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 |
| 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 |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Pascal CINTAS, MD PHD | Contact | 05 61 77 94 40 | cintas.p@chu-toulouse.fr | |
| Anne Cecile Coville | Contact | (05345) 58700 |
| Name | Affiliation | Role |
|---|---|---|
| Pascal CINTAS, MD | University Hospital, Toulouse | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Pascal CINTAS | Recruiting | Toulouse | 31000 | France |
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| ID | Term |
|---|---|
| D000690 | Amyotrophic Lateral Sclerosis |
| D012598 | Sclerosis |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D016472 | Motor Neuron Disease |
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| D019636 | Neurodegenerative Diseases |
| D057177 | TDP-43 Proteinopathies |
| D009468 | Neuromuscular Diseases |
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |