Quantitative Muscle Mri Biomarkers in Duchenne Muscular Dystrophy: Cross-Sectional Correlations with Age and Functional Tests

Sherlock, Sarah P.; Zhang, Yao; Binks, Michael; Marraffino, Shannon

doi:10.2217/bmm-2020-0801

Cited by 16 publications

(15 citation statements)

References 36 publications

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“…As we expected, muscle T1-value was also closely related to a series of clinical assessments. The decreasing of muscle T1-values was correlated with the increase of age, height, weight in our study, which was in accord with the known clinical discovery that DMD was an age-dependent and progressive disease [ 3 , 31 ]. The muscle T1-value correlated with wheelchair use negatively.…”

Section: Discussionsupporting

confidence: 91%

Utilization of T1-Mapping for the pelvic and thigh muscles in Duchenne Muscular Dystrophy: a quantitative biomarker for disease involvement and correlation with clinical assessments

Peng

Song

et al. 2022

BMC Musculoskelet Disord

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Background Little is known about the disease distribution and severity detected by T1-mapping in Duchenne muscular dystrophy (DMD). Furthermore, the correlation between skeletal muscle T1-values and clinical assessments is less studied. Hence, the purposes of our study are to investigate quantitative T1-mapping in detecting the degree of disease involvement by detailed analyzing the hip and thigh muscle, future exploring the predicting value of T1-mapping for the clinical status of DMD. Methods Ninety-two DMD patients were included. Grading fat infiltration and measuring the T1-values of 19 pelvic and thigh muscles (right side) in axial T1-weighted images (T1WI) and T1-maps, respectively, the disease distribution and severity were evaluated and compared. Clinical assessments included age, height, weight, BMI, wheelchair use, timed functional tests, NorthStar ambulatory assessment (NSAA) score, serum creatine kinase (CK) level. Correlation analysis were performed between the muscle T1-value and clinical assessments. Multiple linear regression analysis was conducted for the independent association of T1-value and motor function. Results The gluteus maximus had the lowest T1-value, and the gracilis had the highest T1-value. T1-value decreased as the grade of fat infiltration increased scored by T1WI (P < 0.001). The decreasing of T1-values was correlated with the increase of age, height, weight, wheelchair use, and timed functional tests (P < 0.05). T1-value correlated with NSAA (r = 0.232-0.721, P < 0.05) and CK (r = 0.208-0.491, P < 0.05) positively. T1-value of gluteus maximus, tensor fascia, vastus lateralis, vastus intermedius, vastus medialis, and adductor magnus was independently associated with the clinical motor function tests (P < 0.05). Interclass correlation coefficient (ICC) analysis and Bland-Altman plots showed excellent inter-rater reliability of T1-value region of interest (ROI) measurements. Conclusion T1-mapping can be used as a quantitative biomarker for disease involvement, further assessing the disease severity and predicting motor function in DMD.

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Section: Discussionsupporting

confidence: 91%

Utilization of T1-Mapping for the pelvic and thigh muscles in Duchenne Muscular Dystrophy: a quantitative biomarker for disease involvement and correlation with clinical assessments

Peng

Song

et al. 2022

BMC Musculoskelet Disord

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“…The same target leg of each participant (typically the right leg) was evaluated at each imaging visit (baseline and weeks 17, 33, 49, and 97). Additional details on the MRI setup and scanning protocol have been described previously [ 29 ].…”

Section: Methodsmentioning

confidence: 99%

“…All images were evaluated at a central facility (BioTelemetry Research, Rochester, NY) as described previously [ 29 ]. Upon receipt, all images went through a quality inspection process to ensure images were compliant with the scanning guide, were high-quality acquisitions suitable for assessment (e.g., no significant artifacts), and that T2 phantoms were within the expected T2 relaxation ranges.…”

Section: Methodsmentioning

confidence: 99%

Quantitative magnetic resonance imaging measures as biomarkers of disease progression in boys with Duchenne muscular dystrophy: a phase 2 trial of domagrozumab

et al. 2022

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Duchenne muscular dystrophy (DMD) is a progressive, neuromuscular disorder caused by mutations in the DMD gene that results in a lack of functional dystrophin protein. Herein, we report the use of quantitative magnetic resonance imaging (MRI) measures as biomarkers in the context of a multicenter phase 2, randomized, placebo-controlled clinical trial evaluating the myostatin inhibitor domagrozumab in ambulatory boys with DMD (n = 120 aged 6 to < 16 years). MRI scans of the thigh to measure muscle volume, muscle volume index (MVI), fat fraction, and T2 relaxation time were obtained at baseline and at weeks 17, 33, 49, and 97 as per protocol. These quantitative MRI measurements appeared to be sensitive and objective biomarkers for evaluating disease progression, with significant changes observed in muscle volume, MVI, and T2 mapping measures over time. To further explore the utility of quantitative MRI measures as biomarkers to inform longer term functional changes in this cohort, a regression analysis was performed and demonstrated that muscle volume, MVI, T2 mapping measures, and fat fraction assessment were significantly correlated with longer term changes in four-stair climb times and North Star Ambulatory Assessment functional scores. Finally, less favorable baseline measures of MVI, fat fraction of the muscle bundle, and fat fraction of lean muscle were significant risk factors for loss of ambulation over a 2-year monitoring period. These analyses suggest that MRI can be a valuable tool for use in clinical trials and may help inform future functional changes in DMD.Trial registration: ClinicalTrials.gov identifier, NCT02310763; registered December 2014.

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“…According to previous histopathological studies of paraspinal muscles in AS, different severities and extents of inflammatory cell infiltration, muscular edema, fatty infiltration, and fibrosis, which may potentially affect the T2 value, have been observed according to differences in the disease duration and severity of AS [ 20 – 22 ]. It is known that edema/inflammation and fat infiltration have longer T2 values, while fibrosis lies on the opposite side of the T2 spectrum (i.e., a shorter mean T2 value) [ 24 , 26 ]. In this study, the mean T2 non-fatsat values of the paraspinal muscle at both the L3/L4 and L4/L5 levels were remarkably elevated in AS patients compared to that of matched healthy controls.…”

Section: Discussionmentioning

confidence: 99%

T2 mapping and fat quantification of lumbar paraspinal muscle in ankylosing spondylitis: a case control study

Huang

Yang

Chen

et al. 2022

BMC Musculoskelet Disord

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Background To compare changes in the composition of paraspinal muscles of patients with ankylosing spondylitis (AS) and matched healthy controls using T2 mapping and T2 IDEAL and correlate the quantitative magnetic resonance imaging (qMRI) results with clinical assessments of AS patients. Method In total, 37 AS patients and 37 healthy controls were enrolled in the case control study. T2 mapping with and without fat saturation and IDEAL imaging were used to assess the multifidus (MF) and erector spinae (ES) at the levels of L3/L4 and L4/L5 for all subjects. Mean T2non-fatsat, T2fat, T2fatsat, cross-sectional area (CSA), and fat fraction (FF) were compared between AS and healthy controls. Correlations of qMRI results with clinical assessments were analyzed in AS. Results Significantly elevated mean T2non-fatsat values and the FF of the MF and ES at both levels were observed in AS and compared to the controls (p < 0.05). The mean T2fatsat values of ES and MF were significantly higher only at the level of L3/L4 in AS compared to healthy controls (p < 0.05). A loss of muscle CSA compatible with atrophy was present in MF and ES at both levels in AS compared to the controls (p < 0.05). Weak to moderate positive correlations were found between FF and age and disease duration in AS (r = 0.318–0.415, p < 0.05). However, such positive correlation was not observed between FF and disease duration after adjusting for age (p > 0.05). Conclusions Our findings indicate that using a combination of IDEAL and T2 mapping may provide deeper insights into the pathophysiological degeneration of paraspinal muscles in AS.

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Quantitative Muscle Mri Biomarkers in Duchenne Muscular Dystrophy: Cross-Sectional Correlations with Age and Functional Tests

Cited by 16 publications

References 36 publications

Utilization of T1-Mapping for the pelvic and thigh muscles in Duchenne Muscular Dystrophy: a quantitative biomarker for disease involvement and correlation with clinical assessments

Utilization of T1-Mapping for the pelvic and thigh muscles in Duchenne Muscular Dystrophy: a quantitative biomarker for disease involvement and correlation with clinical assessments

Quantitative magnetic resonance imaging measures as biomarkers of disease progression in boys with Duchenne muscular dystrophy: a phase 2 trial of domagrozumab

T2 mapping and fat quantification of lumbar paraspinal muscle in ankylosing spondylitis: a case control study

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