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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting both upper and lower motor neurons. Electroneuromyography is an important tool for the diagnosis. Previous studies have shown that different components of the blink reflex, such as the latencies of homo- and contralateral R2 responses can be affected. Studies have found that a contralateral R1 component can appear in neurological diseases with affection of the central nervous system especially upper motor neuron, such as HTLV1 infection. Thus, you aim to determine if a contralateral R1 component could be present in ALS.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| ALS patients | Patients with ALS (Awaji criteria) |
| |
| Non-ALS patients | Patients referred to the neurophysiology unit for a suspicion of ALS du to motor weakness, but in whom the diagnosis is ruled out. |
| |
| Healthy volunteers | Healthy volunteers who will undergo a blink reflex evaluation on ENMG. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| blink reflex evaluation | Diagnostic Test | A blink reflex evaluation will be performed in all subjects |
|
| Measure | Description | Time Frame |
|---|---|---|
| Presence of a contralateral R1 component on blink reflex evaluation. | A blink reflex evaluation will be performed in all subjects : Blink reflex will be recorded with surface electrodes placed medially over the lower part of the eyelid, on the orbicularis oculi muscle, with a reference electrode 2 cm laterally. Stimuli will be delivered to the supraorbital nerve by surface electrodes with an intensity required to generate a consistent homolateral R1 response. A contralateral R1 component will be defined as an early compound muscle action potential recorded in the contralateral orbicularis oculi muscle. | Day 1 |
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Inclusion Criteria:
For patients
For healthy subjects:
Exclusion Criteria:
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Patients referred to the neurophysiology unit from the Hospices civils de Lyon, for a suspicion of motor neuron disease.
And healthy volunteers for the third group.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital neurologique Pierre Wertheimer | Bron | Rhone | 69500 | France | ||
| CHU de Nantes |
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| ID | Term |
|---|---|
| D000690 | Amyotrophic Lateral Sclerosis |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D016472 | Motor Neuron Disease |
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| Nantes |
| 44093 |
| France |
| D019636 | Neurodegenerative Diseases |
| D057177 | TDP-43 Proteinopathies |
| D009468 | Neuromuscular Diseases |
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |