Non-uniform muscle fat replacement along the proximodistal axis in Duchenne muscular dystrophy

Hooijmans, Melissa T.; Niks, Erik H.; Burakiewicz, Jedrzej; Anastasopoulos, Constantin; Berg, S.I. van den; Zwet, Erik W. van; Webb, Andrew; Verschuuren, Jan J.; Kan, Hermien E.

doi:10.1016/j.nmd.2017.02.009

Cited by 54 publications

(63 citation statements)

References 34 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Standard deviations describe the variation in mean relaxation times over different volunteers. The corrected T 2 /WF (85% ± 5%) values in the muscle are slightly higher/lower compared with literature values, and the corrected T 1 values in the muscle are in good agreement with literature . In the bone marrow, the water T 1 value is measured as 327 ± 7 ms, the water T 2 value as 155 ± 9 ms, and the FF as 85% ± 2%, agreeing with literature …”

Section: Resultssupporting

confidence: 88%

Water–fat separation in spiral magnetic resonance fingerprinting for high temporal resolution tissue relaxation time quantification in muscle

Koolstra

Webb

Veeger

et al. 2020

Magnetic Resonance in Med

View full text Add to dashboard Cite

Purpose: To minimize the known biases introduced by fat in rapid T 1 and T 2 quantification in muscle using a single-run magnetic resonance fingerprinting (MRF) water-fat separation sequence. Methods: The single-run MRF acquisition uses an alternating in-phase/out-of-phase TE pattern to achieve water-fat separation based on a 2-point DIXON method.Conjugate phase reconstruction and fat deblurring were applied to correct for B 0 inhomogeneities and chemical shift blurring. Water and fat signals were matched to the on-resonance MRF dictionary. The method was first tested in a multicompartment phantom. To test whether the approach is capable of measuring small in vivo dynamic changes in relaxation times, experiments were run in 9 healthy volunteers; parameter values were compared with and without water-fat separation during muscle recovery after plantar flexion exercise. Results: Phantom results show the robustness of the water-fat resolving MRF approach to undersampling. Parameter maps in volunteers show a significant (P < .01) increase in T 1 (105 ± 94 ms) and decrease in T 2 (14 ± 6 ms) when using water-fat-separated MRF, suggesting improved parameter quantification by reducing the well-known biases introduced by fat. Exercise results showed smooth T 1 and T 2 recovery curves. Conclusion: Water-fat separation using conjugate phase reconstruction is possible within a single-run MRF scan. This technique can be used to rapidly map relaxation times in studies requiring dynamic scanning, in which the presence of fat is problematic. K E Y W O R D Sconjugate phase reconstruction, exercise, fat, magnetic resonance fingerprinting, muscle, spiral | 647 KOOLSTRA eT AL.

show abstract

Section: Resultssupporting

confidence: 88%

Water–fat separation in spiral magnetic resonance fingerprinting for high temporal resolution tissue relaxation time quantification in muscle

Koolstra

Webb

Veeger

et al. 2020

Magnetic Resonance in Med

View full text Add to dashboard Cite

show abstract

“…The excellent interobserver reliability found is important in view of differences in fat replacement along the proximodistal axis of muscles in DMD. 23 In facioscapulohumeral muscular dystrophy, FF determination also yielded an excellent interobserver reliability (ICC 0.992). 33 Thus, ROIs can confidently be drawn at local sites in clinical trials across multiple institutions, while decisions on scan inclusion and which slices to use should be made with central reading.…”

Section: Discussionmentioning

confidence: 91%

“…21 Selected MRI results of this study have been previously reported. 13,22,23 The CCHMC participants were recruited from the international Prospective Natural History Study of Progression of Subjects With Duchenne Muscular Dystrophy (PRO-DMD-01) that started in 2012. For this work, LUMC, CCHMC, and the Institute of Myology collaborated as part of the BIOIMAGE-Neuromuscular Diseases (BIOIMAGE-NMD) consortium (project identifier 602485, funded under FP7-HEALTH).…”

Section: Participants and Study Designmentioning

confidence: 99%

“…At the LUMC, images of the right thigh were acquired on a 3T MRI scanner (Ingenia, Philips Healthcare, Best, the Netherlands), as described previously. 23…”

Section: Mri Acquisitionmentioning

confidence: 99%

“…Data were reconstructed with the manufacturer's software assuming a single peak in the lipid spectrum and without T2* relaxation correction. Recent studies have shown differences in fat replacement along the proximodistal axis of the muscle in DMD, 23,24 which necessitated precise definition of the region of interest (ROI) along this axis for comparison of MRI data from both sites. Two observers from LUMC (K.J.N.)…”

Section: Mri Analysismentioning

confidence: 99%

See 2 more Smart Citations

MRI vastus lateralis fat fraction predicts loss of ambulation in Duchenne muscular dystrophy

et al. 2020

View full text Add to dashboard Cite

ObjectiveWe studied the potential of quantitative MRI (qMRI) as a surrogate endpoint in Duchenne muscular dystrophy by assessing the additive predictive value of vastus lateralis (VL) fat fraction (FF) to age on loss of ambulation (LoA).MethodsVL FFs were determined on longitudinal Dixon MRI scans from 2 natural history studies in Leiden University Medical Center (LUMC) and Cincinnati Children's Hospital Medical Center (CCHMC). CCHMC included ambulant patients, while LUMC included a mixed ambulant and nonambulant population. We fitted longitudinal VL FF values to a sigmoidal curve using a mixed model with random slope to predict individual trajectories. The additive value of VL FF over age to predict LoA was calculated from a Cox model, yielding a hazard ratio.ResultsEighty-nine MRIs of 19 LUMC and 15 CCHMC patients were included. At similar age, 6-minute walking test distances were smaller and VL FFs were correspondingly higher in LUMC compared to CCHMC patients. Hazard ratio of a percent-point increase in VL FF for the time to LoA was 1.15 for LUMC (95% confidence interval [CI] 1.05–1.26; p = 0.003) and 0.96 for CCHMC (95% CI 0.84–1.10; p = 0.569).ConclusionsThe hazard ratio of 1.15 corresponds to a 4.11-fold increase of the instantaneous risk of LoA in patients with a 10% higher VL FF at any age. Although results should be confirmed in a larger cohort with prospective determination of the clinical endpoint, this added predictive value of VL FF to age on LoA supports the use of qMRI FF as an endpoint or stratification tool in clinical trials.

show abstract

Preserved thenar muscles in non‐ambulant Duchenne muscular dystrophy patients

Naarding

Keene

Mishre

et al. 2021

J cachexia sarcopenia muscle

View full text Add to dashboard Cite

Background Clinical trials in Duchenne muscular dystrophy (DMD) focus primarily on ambulant patients. Results cannot be extrapolated to later disease stages due to a decline in targeted muscle tissue. In non‐ambulant DMD patients, hand function is relatively preserved and crucial for daily‐life activities. We used quantitative MRI (qMRI) to establish whether the thenar muscles could be valuable to monitor treatment effects in non‐ambulant DMD patients. Methods Seventeen non‐ambulant DMD patients (range 10.2–24.1 years) and 13 healthy controls (range 9.5–25.4 years) underwent qMRI of the right hand at 3 T at baseline. Thenar fat fraction (FF), total volume (TV), and contractile volume (CV) were determined using 4‐point Dixon, and T2water was determined using multiecho spin‐echo. Clinical assessments at baseline (n = 17) and 12 months (n = 13) included pinch strength (kg), performance of the upper limb (PUL) 2.0, DMD upper limb patient reported outcome measure (PROM), and playing a video game for 10 min using a game controller. Group differences and correlations were assessed with non‐parametric tests. Results Total volume was lower in patients compared with healthy controls (6.9 cm3, 5.3–9.0 cm3 vs. 13.0 cm3, 7.6–15.8 cm3, P = 0.010). CV was also lower in patients (6.3 cm3, 4.6–8.3 cm3 vs. 11.9 cm3, 6.9–14.6 cm3, P = 0.010). FF was slightly elevated (9.7%, 7.3–11.4% vs. 7.7%, 6.6–8.4%, P = 0.043), while T2water was higher (31.5 ms, 30.0–32.6 ms vs. 28.1 ms, 27.8–29.4 ms, P < 0.001). Pinch strength and PUL decreased over 12 months (2.857 kg, 2.137–4.010 to 2.243 kg, 1.930–3.339 kg, and 29 points, 20–36 to 23 points, 17–30, both P < 0.001), while PROM did not (49 points, 36–57 to 44 points, 30–54, P = 0.041). All patients were able to play for 10 min at baseline or follow‐up, but some did not comply with the study procedures regarding this endpoint. Pinch strength correlated with TV and CV in patients (rho = 0.72 and rho = 0.68) and controls (both rho = 0.89). PUL correlated with TV, CV, and T2water (rho = 0.57, rho = 0.51, and rho = −0.59). Conclusions Low thenar FF, increased T2water, correlation of muscle size with strength and function, and the decrease in strength and function over 1 year indicate that the thenar muscles are a valuable and quantifiable target for therapy in later stages of DMD. Further studies are needed to relate these data to the loss of a clinically meaningful milestone.

show abstract

Non-uniform muscle fat replacement along the proximodistal axis in Duchenne muscular dystrophy

Cited by 54 publications

References 34 publications

Water–fat separation in spiral magnetic resonance fingerprinting for high temporal resolution tissue relaxation time quantification in muscle

Water–fat separation in spiral magnetic resonance fingerprinting for high temporal resolution tissue relaxation time quantification in muscle

MRI vastus lateralis fat fraction predicts loss of ambulation in Duchenne muscular dystrophy

Preserved thenar muscles in non‐ambulant Duchenne muscular dystrophy patients

Contact Info

Product

Resources

About