Characterization of Strength and Function in Ambulatory Adults With GNE Myopathy.

TitleCharacterization of Strength and Function in Ambulatory Adults With GNE Myopathy.
Publication TypeJournal Article
Year of Publication2017
AuthorsArgov 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
JournalJ Clin Neuromuscul Dis
Date Published2017 Sep
KeywordsAdolescent, 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 JournalJ Clin Neuromuscul Dis
PubMed ID28827485
PubMed Central IDPMC5636058