Accurate Test of Limb Isometric Strength (ATLIS) in ALS
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A 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 investigator will compare ATLIS data with data from the commonly used ALS outcomes measure, the ALS Functional Rating Scale-Revised (ALSFRS-R), as well as an exploratory measure, electrical impedance myography (EIM), in a prospective, longitudinal study. Both outcomes measures will be performed on 100 subjects collected preferably at bi-monthly clinic visits during the study period.
Condition or disease
Amyotrophic Lateral Sclerosis
Cedars-Sinai Medical Center will enroll and collect data on 100 subjects. Subjects will ideally be tested every 1 to 4 months according to each patient's site visit schedule. Subjects will be tested at each visit using ATLIS, the ALSFRS-R questionnaire and EIM.
Change in Accurate Test of Limb Isometric Strength (ATLIS) between each visit [ Time Frame: every 1 to 4 months according to each patient's site visit schedule ]
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.
Secondary Outcome Measures :
Change in ALS Functional Rating Scale - Revised (ALSFRS-R) between each visit [ Time Frame: every 1 to 4 months according to each patient's site visit schedule ]
This questionnaire consists of 12 questions about the subject's ability to function in certain daily activities. Questions are asked in an interview format.
Other Outcome Measures:
Exploratory measure: Electrical impedance myography (EIM) data will be compared to ATLIS data. [ Time Frame: every 1 to 4 months according to each patient's site visit schedule ]
EIM evaluates how electrical current flows through muscle which assists in determining muscle health by measuring electrical current in muscle. 14 muscle groups in the arms and legs will be tested in this study.
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Ages Eligible for Study:
18 Years and older (Adult, Older Adult)
Sexes Eligible for Study:
Accepts Healthy Volunteers:
Adults with ALS
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.
Active movement of at least two limbs at the time of study entry
Intend to routinely attend clinic for neurological care at the clinical site
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).
Patients judged by the site clinical investigator to have significant clinical evidence of unstable medical or psychiatric illness will be excluded.