Related Experiment Video
Updated: Feb 24, 2026
Lower Limb Biomechanical Analysis of Healthy Participants
Published on: April 15, 2020
Characterization of Strength and Function in Ambulatory Adults With GNE Myopathy
Zohar Argov1, Faye Bronstein, Alicia Esposito
1*Department of Neurology, Hadassah University Medical Center, Jerusalem, Israel; †Physical Medicine and Rehabilitation Department, NYU Langone Medical Center-Rusk Institute, New York, NY; ‡Clinical Research Unit, Hadassah University Medical Center, Jerusalem, Israel; §Washington University School of Medicine, Neuromuscular Division, St. Louis, MO; ¶UCLA Department of Orthopaedic Surgery, Kameron Gait and Motion Analysis Laboratory, Los Angeles, CA; ‖NYU Division of Neurogenetics, New York, NY; **UCLA Department of Neurology, Los Angeles, CA; and ††Clinical Outcomes Research and Evaluation, Ultragenyx Pharmaceutical, Inc, Novato, CA.
Objective:
To characterize the pattern and extent of muscle weakness and impact on physical functioning in adults with GNEM.
Methods:
Strength and function were assessed in GNEM subjects (n = 47) using hand-held dynamometry, manual muscle testing, upper and lower extremity functional capacity tests, and the GNEM-Functional Activity Scale (GNEM-FAS).
Results:
Profound upper and lower muscle weakness was measured using hand-held dynamometry in a characteristic pattern, previously described. Functional tests and clinician-reported outcomes demonstrated the consequence of muscle weakness on physical functioning.
Conclusions:
The characteristic pattern of upper and lower muscle weakness associated with GNEM and the resulting functional limitations can be reliably measured using these clinical outcome assessments of muscle strength and function.
Related Concept Videos
01:15Myasthenia Gravis: Diagnostic Tests
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
01:28Disorders of the Skeletal Muscle
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
01:20Myasthenia Gravis: Overview and Treatment
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...