Amyotrophic Lateral Sclerosis With Dementia
Conditions
Keywords
Amyotrophic, Sclerosis
Brief summary
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.
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.
Interventions
DIAGNOSTIC_TESTWhole Body MRI and ENMG
Whole body muscle MRI lasting 30 to 45 min without injection of contrast medium an ENMG
Sponsors
University Hospital, Toulouse
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 99 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Diagnostic certainty 1.ENMG according to El Escorial revised criteria using ENMG alone, MRI alone and ENMG+MRI to define denervation diffusion | 1 month | number of patients classified as possible, probable or certain according to El Escorial criteria using ENMG alone, IMR alone and ENMR+MRI |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Define precise topographic and characteristic of muscle involvement in ALS by MRI | 1 month | number of patients with concordance between ENMG and MRI for the detection of denervation |
Countries
France
Contacts
Primary ContactPascal CINTAS, MD PHD
Backup ContactAnne Cecile Coville
Outcome results
None listed