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| ID | Type | Description | Link |
|---|---|---|---|
| 1-10-72-153-23 | Other Identifier | Regional Research Ethics Committee Region Midtjylland |
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| Name | Class |
|---|---|
| Aarhus University Hospital | OTHER |
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The aim of this study is to describe the changes in the neuromuscular connection in patients with amyotrophic lateral sclerosis (ALS). The study consist of three substudies that have the following main hypothesis:
There will be 3 inclusion groups.
All participants will be invited for at least 1 visit (baseline). If participants in group 1 eventually receive the diagnosis of ALS they will be invited for 2 additional visits 4 og 8 months after baseline visit, respectively.
Examinations will consist of:
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| ALS patients | Patients enrolled prior to determination of diagnosis on referral to neurophysiological examination. When the diagnosis is later established they get categorized as ALS patients. ALS patients with recent diagnosis might also be included directly. |
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| ALS mimic disease patients | Patients enrolled prior to determination of diagnosis on referral to neurophysiological examination. When diagnosis is later established and the diagnosis is NOT ALS they get categorized as ALS mimic disease patients. |
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| Healthy controls | Healthy controls. |
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| Disease controls | Patients with another motor neuron disease than ALS with slow progression. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Observational study | Other | Observational study. |
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| Measure | Description | Time Frame |
|---|---|---|
| Reinnervation | Difference between fast and slow progressing patients in change in mean amplitude size of motor units as estimated by MScanFit motor unit number estimation. | From baseline and 8 months |
| Blood biomarkers | Difference in blood concentration of c-terminal agrin fragment and neural cell adhesion molecule at baseline between ALS patients, healthy controls and ALS mimic disease patients. | Baseline |
| Fatigue and decrement | Difference in proportion of participants with decrement between ALS patients and ALS mimic disease patients as well as degree of fatigue among ALS patients with and without neuromuscular transmission deficiency. | Baseline |
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| Measure | Description | Time Frame |
|---|---|---|
| Difference in mean amplitude size over time in patients. | Mean amplitude size measured by MScanFit technique. | Baseline, 4 months and 8 months. |
| Difference in motor unit number estimation over time in patients. |
Inclusion Criteria:
Exclusion Criteria:
For disease controls the exclusion criteria are the same, but the inclusion criteria:
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Patients refered for neurophysiological examination or patients followed at out-patient clinics.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Jesper Storgaard, MD | Contact | 004520231903 | jesstg@rm.dk |
| Name | Affiliation | Role |
|---|---|---|
| Jesper H Storgaard, MD | Aarhus University and Department of Neurology, Aarhus University Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Department of Neurology, Aarhus University Hospital | Recruiting | Aarhus | Central Jutland | 8200 | Denmark |
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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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| ID | Term |
|---|---|
| D019370 | Observation |
| ID | Term |
|---|---|
| D008722 | Methods |
| D008919 | Investigative Techniques |
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Blood samples stored for batch analysis.
Motor unit number estimation measured by MScanFit technique.
| Baseline, 4 months and 8 months. |
| Difference in mean amplitude size between patients and control groups | Mean amplitude size measured by MScanFit technique. | Baseline. |
| Muscle strength assessed with manual muscle strength testing | Difference in measures from manual muscle strength testing. | Baseline, 4 months and 8 months. |
| Difference in isometric ankle dorsiflexion strength measured on dynamometer. | Measured on dynamometer. | Baseline, 4 months and 8 months. |
| Difference in handgrip strength measured on dynamometer. | Measured with handgrip dynamometer. | Baseline, 4 months and 8 months. |
| Correlation between decrement at repetitive nerve stimulation and signs of reinnervation as measured by mean amplitude size. | Decrement measured with repetitive nerve stimulation and mean amplitude size measured with MScanFit technique. | Baseline, 4 months and 8 months. |
| Blood concentration of c-terminal agrin fragment and neural cell adhesion molecule. | Difference in blood measurements of c-terminal agrin fragment, neural cell adhesion molecule, neurofilament light chain and routine kidney and liver markers. | Baseline, 4 months and 8 months. |
| Difference in muscle thickness as measured by ultrasound examination of muscles | Measured by ultrasound examination. | Baseline, 4 months and 8 months. |
| Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised scores | Difference in scores from Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised. Scale 0-48, with 48 being the best. | Baseline, 4 months and 8 months. |
| Multidimensional Fatigue Inventory scores | Difference in scores from Multidimensional Fatigue Inventory. Scale 4-20, with 20 indicating worst degree of fatigue. | Baseline, 4 months and 8 months. |
| Fatigue Severity Scale scores | Difference in scores from Fatigue Severity Scale. Scale 9-63, with 63 indicating worst degree of fatigue. | Baseline, 4 months and 8 months. |
| Difference in free fatt mass as measured by bioelectrical impedance analysis. | Measured by bioelectrical impedance analysis. | Baseline, 4 months and 8 months. |
| D019636 | Neurodegenerative Diseases |
| D057177 | TDP-43 Proteinopathies |
| D009468 | Neuromuscular Diseases |
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |