Longitudinal Study of Outcomes Measures in ALS Trials

Study Overview
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Brief Summary
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A new strength measurement device called Accurate Test of Limb Isometric Strength (ATLIS) was developed to precisely and conveniently measure static limb strength in patients with ALS. The investigators will compare ATLIS data with data from two commonly used ALS outcomes measures, the ALS Functional Rating Scale-Revised (ALSFRS-R) and slow vital capacity (SVC) in a prospective, longitudinal study. All three outcomes measures will be performed on 100 subjects collected preferably at bi-monthly clinic visits during the study period.

Detailed Description
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Four sites will participate in data collection for this study and will enroll approximately 20-30 subjects per site. Subjects will be tested every 1 to 4 months for up to two years. Subjects will be tested at each visit using ATLIS, ALSFRS-R questionnaire and slow vital capacity testing. Inclusion criteria: Subjects 18 years or older diagnosed with clinically possible, laboratory supported probable, probable or clinically definite ALS according to the World Federation of Neurology Revised El Escorial Criteria. Capable of providing informed consent and complying with trial procedures. Exclusion criteria: - Patients, judged by the site clinical investigator to have medical conditions that may limit their ability to safely exert maximal force using the muscles in their arms and legs will be excluded from this study (e.g. unstable hypertension, significant cardiac disease, significant musculoskeletal disorders, or other medical conditions).

Official Title
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Longitudinal Study Comparing the Responsiveness of Outcomes Measures in ALS Trials

Conditions
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Amyotrophic Lateral Sclerosis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: 18 years or older clinically possible, laboartory supported probable, probable or clinically definite ALS according to El Escorial Criteria capable of providing informed consent Exclusion Criteria: persons with medical conditions that would limit their ability to safely exert maximal force using muscles in their arms and legs (e.g. unstable cardiac, musculoskeletal, or other medical conditions)

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 90 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in Accurate Test of Limb Isometric Strength (ATLIS) between each visit | ATLIS measures isometric strength in 12 muscle groups in the arms and legs. The subject is tested seated in a specially designed chairs with adjustable uprights. The subject's limb is placed in a standard position and maximal force is exerted on the wireless load cell fixed to the upright. Strength data is digitally captured and expressed as a percentage of expected normal. | every 1 to 4 months for up to two years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in ALS Functional Rating Scale - Revised (ALSFRS-R) between each visit | This questionnaire consists of 12 questions about the subject's ability to function in certain daily activities. Questions are asked in an interview format. | every 1 to 4 months for up to 2 years | 
| Change in Slow Vital Capacity (SVC) between each visit | SVC measures the amount of air you can exhale following a deep breath. For this test, we will ask you to hold a mouthpiece in your mouth, breathe in deeply, and breathe out as much air as you can, up to 5 times. | Every 1 to 4 months for up to two years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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outcomes measures, strength measurement, disease progression