Cardiac and Sternocleidomastoid Muscle Involvement in Duchenne Muscular Dystrophy

Mavrogeni, Sophie; Tzelepis, George E.; Athanasopoulos, George; Maounis, Themistoklis; Douskou, Marouso; Papavasiliou, Antigoni; Cokkinos, Dennis V.

doi:10.1378/chest.127.1.143

Cited by 42 publications

(43 citation statements)

References 22 publications

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“…The gels were selected to best correspond with the reported T 2 values of plaque [9] and SCM tissue [15],[16] found in vivo. Since no in vivo T 2 measurement of SCM in 1.5 T was available, its T 2 value was approximated using values from 0.5 T and 3 T [15],[16]. …”

Section: Methodsmentioning

confidence: 99%

Optimization of Improved Motion-sensitized Driven-equilibrium (iMSDE) blood suppression for carotid artery wall imaging

Zhu

Graves

Yuan

et al. 2014

Journal of Cardiovascular Magnetic Resonance

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BackgroundImproved motion-sensitized driven-equilibrium (iMSDE) preparations have been successfully used in carotid artery wall imaging to achieve blood suppression, but it causes notable signal loss, mostly due to inherent T2 decay, eddy current effects and B1+ inhomogeneity. In this study, we investigate the signal to noise ratio (SNR) and blood suppression performance of iMSDE using composite RF pulses and sinusoidal gradients. Optimized first moment (m1) values for iMSDE prepared T1- and T2- weighted (T1- and T2-w) imaging are presented.MethodsTwelve healthy volunteers and six patients with carotid artery disease underwent iMSDE and double inversion recovery (DIR) prepared T1- and T2-w fast spin echo (FSE) MRI of the carotid arteries. Modified iMSDE module using composite RF pulses and sinusoidal gradients were evaluated with a range of m1. SNR of adjacent muscle, vessel wall and the lumen were reported. The optimized iMSDE module was also tested in a 3D variable flip angle FSE (CUBE) acquisition.ResultsThe SNR of muscle was highest using sinusoidal gradients, and the relative improvement over the trapezoidal gradient increased with higher m1 (p<0.001). Optimal SNR was observed using an iMSDE preparation scheme containing two 180° composite pulses and standard 90° and -90° pulses (p=0.151). iMSDE produced better blood suppression relative to DIR preparations even with a small m1 of 487 mT*ms2/m (p<0.001). In T1-w iMSDE, there was a SNR decrease and an increased T2 weighting with increasing m1. In T2-w iMSDE, by matching the effective echo time (TE), the SNR was equivalent when m1 was <= 1518 mT*ms2/m, however, higher m1 values (2278 – 3108 mT*ms2/m) reduced the SNR. In the patient study, iMSDE improved blood suppression but reduced vessel wall CNR efficiency in both T1-w and T2-w imaging. iMSDE also effectively suppressed residual flow artifacts in the CUBE acquisition.ConclusionsiMSDE preparation achieved better blood suppression than DIR preparation with reduced vessel wall CNR efficiency in T1-w and T2-w images. The optimized m1s are 487 mT*ms2/m for T1-w imaging and 1518 mT*ms2/m for T2-w imaging. Composite 180° refocusing pulses and sinusoidal gradients improve SNR performance. iMSDE further improves the inherent blood suppression of CUBE.

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Section: Methodsmentioning

confidence: 99%

Optimization of Improved Motion-sensitized Driven-equilibrium (iMSDE) blood suppression for carotid artery wall imaging

Zhu

Graves

Yuan

et al. 2014

Journal of Cardiovascular Magnetic Resonance

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“…Adult male mice between the age of 3 months and 6 months were used in this study except for the PDE5I experiment. A previous study that showed DMD involves a pathologic change of sternocleidomastoid muscles in humans [56] was the basis for our experiments to investigate the pathophysiological changes in the corresponding mouse sternomastoid muscle. All the procedures related to animal experiments were reviewed and approved by the Subcommittee on Research Animal Care of Massachusetts General Hospital.…”

Section: Methodsmentioning

confidence: 99%

Primary Role of Functional Ischemia, Quantitative Evidence for the Two-Hit Mechanism, and Phosphodiesterase-5 Inhibitor Therapy in Mouse Muscular Dystrophy

et al. 2007

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BackgroundDuchenne Muscular Dystrophy (DMD) is characterized by increased muscle damage and an abnormal blood flow after muscle contraction: the state of functional ischemia. Until now, however, the cause-effect relationship between the pathogenesis of DMD and functional ischemia was unclear. We examined (i) whether functional ischemia is necessary to cause contraction-induced myofiber damage and (ii) whether functional ischemia alone is sufficient to induce the damage.Methodology/Principal Findings In vivo microscopy was used to document assays developed to measure intramuscular red blood cell flux, to quantify the amount of vasodilatory molecules produced from myofibers, and to determine the extent of myofiber damage. Reversal of functional ischemia via pharmacological manipulation prevented contraction-induced myofiber damage in mdx mice, the murine equivalent of DMD. This result indicates that functional ischemia is required for, and thus an essential cause of, muscle damage in mdx mice. Next, to determine whether functional ischemia alone is enough to explain the disease, the extent of ischemia and the amount of myofiber damage were compared both in control and mdx mice. In control mice, functional ischemia alone was found insufficient to cause a similar degree of myofiber damage observed in mdx mice. Additional mechanisms are likely contributing to cause more severe myofiber damage in mdx mice, suggestive of the existence of a “two-hit” mechanism in the pathogenesis of this disease.Conclusions/SignificanceEvidence was provided supporting the essential role of functional ischemia in contraction-induced myofiber damage in mdx mice. Furthermore, the first quantitative evidence for the “two-hit” mechanism in this disease was documented. Significantly, the vasoactive drug tadalafil, a phosphodiesterase 5 inhibitor, administered to mdx mice ameliorated muscle damage.

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“…There is no ionizing radiation used in MRI and with the newer MRI machines high-resoultion images can be obtained. The T2 relaxation time can be used to quantitate the tissue characteristics of the heart as it reflects the differences in water and fat content of the muscle [32]. However, MRI is expensive and not all centers have access to such a facility, but it is certainly more sensitive at detecting the cardiomyopathy when it is in the sub-clinical stage than many of the other methods available.…”

Section: Diagnosis Of Cardiomyopathy In Dmd Patientsmentioning

confidence: 99%

Experimental Models of Duchenne Muscular Dystrophy: Relationship with Cardiovascular Disease

Ameen

Robson²

2010

TOCMJ

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Almost every boy that has Duchenne Muscular Dystrophy (DMD) will develop cardiac problems. Whereas, it used to be respiratory problems that was the main cause of death in these DMD boys; with the advent of better respiratory care it is now the cardiac involvement that is becoming the most common cause of their death. Once the heart is affected, there is progressive deterioration in the function of the heart over time. The main problem is the death of the cardiomyocytes. The cause of the cardiomyocyte death is due to the loss of dystrophin, this makes the sarcolemma more susceptible to damage, and leads to a cascade of calcium influx, calcium activated proteases and ultimately the death of the cardiomyocyte. The dead cardiomyocytes are replaced by fibrotic tissue, which results in a dilated cardiomyopathy (DCM) developing, which begins in the base of the left ventricle and progresses to involve the entire left ventricle. The treatments used for the DMD cardiomyopathy are based on ones designed for other forms of cardiac weakness and include ACE-inhibitors and β-blockers. New therapies based around the pathophysiology in DMD are now being introduced. This review will look at the pathophysiology of the cardiac problems in DMD and how the various animal models that are available can be used to design new treatment options for DMD boys.

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Cardiac and Sternocleidomastoid Muscle Involvement in Duchenne Muscular Dystrophy

Cited by 42 publications

References 22 publications

Optimization of Improved Motion-sensitized Driven-equilibrium (iMSDE) blood suppression for carotid artery wall imaging

Optimization of Improved Motion-sensitized Driven-equilibrium (iMSDE) blood suppression for carotid artery wall imaging

Primary Role of Functional Ischemia, Quantitative Evidence for the Two-Hit Mechanism, and Phosphodiesterase-5 Inhibitor Therapy in Mouse Muscular Dystrophy

Experimental Models of Duchenne Muscular Dystrophy: Relationship with Cardiovascular Disease

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