Characterization of Strength and Function in Ambulatory Adults With GNE Myopathy.
|Title||Characterization of Strength and Function in Ambulatory Adults With GNE Myopathy.|
|Publication Type||Journal Article|
|Year of Publication||2017|
|Authors||Argov Z, Bronstein F, Esposito A, Feinsod-Meiri Y, Florence JM, Fowler E, Greenberg MB, Malkus EC, Rebibo O, Siener CS, Caraco Y, Kolodny EH, Lau HA, Pestronk A, Shieh P, Skrinar AM, Mayhew JE|
|Journal||J Clin Neuromuscul Dis|
|Date Published||2017 Sep|
|Keywords||Adolescent, Adult, Aged, Female, Humans, Lower Extremity, Male, Middle Aged, Motor Activity, Muscle Strength, Muscle Weakness, Muscle, Skeletal, Myositis, Inclusion Body, N-Acetylneuraminic Acid, Young Adult|
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.
|Alternate Journal||J Clin Neuromuscul Dis|
|PubMed Central ID||PMC5636058|