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Lookup NU author(s): Meredith JamesORCiD, Dr Anna Mayhew, Emerita Professor Katherine Bushby, Professor Volker StraubORCiD
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© 2022 Wiley Periodicals LLC. Introduction/Aims: Dysferlinopathy demonstrates heterogeneity in muscle weakness between patients, which can progress at different rates over time. Changing muscle strength due to disease progression or from an investigational product is associated with changing functional ability. The purpose of this study was to compare three methods of strength testing used in the Clinical Outcome Study (COS) for dysferlinopathy to understand which method and which muscle groups were most sensitive to change over time. Methods: Patients were evaluated at each study visit using functional scales, manual muscle testing, and handheld dynamometry (HHD) at all 15 sites. A fixed-frame system (Fixed) was used at a subset of seven sites. Screening and baseline visits were evaluated for reliability. Data over a 1-year period were analyzed to determine sensitivity to change among strength modalities and individual muscle groups. Results: HHD and Fixed captured significant change across 1 year in summed muscle strength score of four muscle groups (P <.01). Strength summed scores were significantly correlated with functional scales (rho = 0.68-0.92, P <.001). Individual muscle groups, however, showed high levels of variability between visits. Discussion: Although both HHD and Fixed demonstrate change over 12 months, HHD is a less expensive option that provides data on a continuous scale and may be easier to implement. Due to variability in strength measures, researchers should carefully consider use of strength testing as an outcome and may wish to select functional measures with less variability as clinical trial endpoints.
Author(s): Reash NF, James MK, Alfano LN, Mayhew AG, Jacobs M, Iammarino MA, Holsten S, Sakamoto C, Tateishi T, Yajima H, Duong T, de Wolf B, Gee R, Bharucha-Goebel DX, Bravver E, Mori-Yoshimura M, Bushby K, Rufibach LE, Straub V, Lowes LP
Publication type: Article
Publication status: Published
Journal: Muscle and Nerve
Year: 2022
Volume: 66
Issue: 2
Pages: 159-166
Print publication date: 01/08/2022
Online publication date: 04/05/2022
Acceptance date: 02/05/2022
ISSN (print): 0148-639X
ISSN (electronic): 1097-4598
Publisher: John Wiley and Sons Inc.
URL: https://doi.org/10.1002/mus.27570
DOI: 10.1002/mus.27570
PubMed id: 35506767
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