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Electrical impedance myography (EIM) in a natural history study of C9ORF72 mutation carriers.

ABSTRACT: Objective: Electrical Impedance Myography (EIM) was used to evaluate disease progression in subjects with C9ORF72 expansion mutations and to assess correlations with Medical Research Council (MRC) Scale and revised ALS Functional Rating Scale (ALSFRS-R) measurements. Four types of clinical presentations were assessed; Amyotrophic Lateral Sclerosis (ALS), Frontotemporal dementia (FTD) or other dementia, ALS-FTD, and asymptomatic (ASYMP). Methods: Subjects were divided into an ALS Group (ALS/ALS-FTD) and non-ALS Group (FTD/ASYMP) based on initial visit and evaluated at 0, 6, 18, and 30 months with EIM of 4 arm and 4 leg muscles, ALSFRS-R, and MRC scales. The change in EIM from baseline and correlation with the functional scale and strength testing were analyzed. Results: EIM 50kHz phase values significantly declined over time in the ALS group (n = 31) compared to the non-ALS group (FTD/ASYMP) (n = 19). In the ALS group, the decline in EIM was correlated with decline in the ALSFRS-R and MRC scores using within-subject correlations. Conclusion: In clinical trials with small populations of genetically associated ALS such as C9ORF-related ALS, EIM may be a useful quantitative biomarker. We did not detect decline in asymptomatic subjects, but longer term studies may detect early changes in this group.

SUBMITTER: Offit MB

PROVIDER: S-EPMC8340645 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Loss of muscle mass and strength represents one of the most significant contributors to impaired function in older adults. Convenient and non-invasive biomarkers are needed that can readily identify and track age-related muscle change. Previous data has suggested electrical impedance myography (EIM) has the potential to serve in this capacity. In this study we investigated how changes in EIM compared with other standard measures of muscle structure and function in aged compared with young mice. A total of 19 male mice aged approximately 25 months and 19 male mice aged 3 months underwent surface multifrequency EIM of the right gastrocnemius muscle using standard methods. Fore and hind limb grip strength, sciatic compound muscle action potential amplitude, and in-situ force of the gastrocnemius were also measured; after sacrifice, gastrocnemius myofiber size was assessed using standard histology. Spearman correlation coefficients were calculated to investigate the association between EIM and muscle characteristics. EIM in aged animals demonstrated significantly lower 50 kHz impedance phase (p<0.001) and reactance (p<0.01) values as well as reduced multifrequency parameters. In contrast, absolute gastrocnemius muscle mass was no different between young and aged mice (p = 0.58) but was reduced in aged mice after normalization to body mass (p<0.001). Median myofiber size in the aged mice was not different from that of young mice (p = 0.72). Aged mice showed reduced muscle function on the basis of normalized fore limb (p<0.001) and normalized hind limb (p<0.001) grip strength, as well as normalized gastrocnemius twitch (p<0.001) and normalized maximal isometric force (p<0.001). Sciatic compound muscle action potential amplitude was reduced in aged mice (p<0.05). EIM parameters showed good correlation with reduced standard physiological and electrophysiological measures of muscle health. Our study suggests that EIM is sensitive to aged-related muscle change and may represent a convenient and valuable method of quantifying loss of muscle health.

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Electrical impedance myography (EIM) in a natural history study of C9ORF72 mutation carriers.

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