Skeletal muscle inflammation/necrosis and fat infiltration are strong indicators of disease activity and progression in many neuromuscular disorders. They can be assessed by muscle T2 relaxometry and water-fat separation techniques, respectively. In the present work, we exploited differences between water and fat T1 and T2 relaxivities by applying a bi-component extended phase graph (EPG) fitting approach to simultaneously quantify the muscle water T2 and fat fraction from standard multi-slice multi-echo (MSME) acquisitions in the presence of stimulated echoes. Experimental decay curves were adjusted to the theoretical model using either an iterative non-negative least-squares (NNLS) procedure or a pattern recognition approach. Twenty-two patients (age, 49 ± 18 years) were selected to cover a large range of muscle fat infiltration. Four cases of chronic or subchronic juvenile dermatomyositis (age, 8 ± 3 years) were investigated before and 3 months following steroid treatment. For control, five healthy volunteers (age, 25 ± 2 years) were recruited. All subjects underwent the MSME sequence and EPG fitting procedure. The EPG fitting algorithm allowed a precise estimation of water T2 and fat fraction in diseased muscle, even in the presence of large B1(+) inhomogeneities. In the whole cohort of patients, there was no overall correlation between water T2 values obtained with the proposed method and the fat fraction estimated inside muscle tissues (R(2) = 0.02). In the patients with dermatomyositis, there was a significant decrease in water T2 (-4.09 ± 3.7 ms) consequent to steroid treatment. The pattern recognition approach resulted in a 20-fold decrease in processing time relative to the iterative NNLS procedure. The fat fraction derived from the EPG fitting approach correlated well with the fat fraction derived from a standard three-point Dixon method (≈1.5% bias). The bi-component EPG fitting analysis is a precise tool to monitor muscle tissue disease activity and is able to handle bias introduced by fat infiltration and B1(+) inhomogeneities.

Neuromuscular diseases are characterized by progressive muscle degeneration and muscle weakness resulting in functional disabilities. While each of these diseases is individually rare, they are common as a group, and a large majority lacks effective treatment with fully market approved drugs. Magnetic resonance imaging and spectroscopy techniques (MRI and MRS) are showing increasing promise as an outcome measure in clinical trials for these diseases. In 2013, the European Union funded the COST (co-operation in science and technology) action BM1304 called MYO-MRI ( www.myo-mri.eu ), with the overall aim to advance novel MRI and MRS techniques for both diagnosis and quantitative monitoring of neuromuscular diseases through sharing of expertise and data, joint development of protocols, opportunities for young researchers and creation of an online atlas of muscle MRI and MRS . In this report, the topics that were discussed in the framework of working group 3, which had the objective to: Explore new contrasts, new targets and new imaging techniques for NMD are described. The report is written by the scientists who attended the meetings and presented their data. An overview is given on the different contrasts that MRI can generate and their application, clinical needs and desired readouts, and emerging methods.

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The spin-spin (T2) relaxation of (1)H-NMR signals in human skeletal muscle has been previously hypothesized to reveal information about myowater compartmentation. Although experimental support has been provided, no consensus has yet emerged concerning the attribution of specific anatomical compartments to the observed T2 components. Potential application of a noninvasive tool that might offer such information urges the quest for a definitive answer to this question. The purpose of this work was to obtain new information that might help elucidate the mechanism of T2 distribution in muscle. To do so, in vivo T2 relaxation data was acquired from the soleus of eight healthy volunteers using a localized Carr-Purcell-Meiboom-Gill technique. Each acquisition contained 1000 echoes with an interecho spacing of 1 ms. Data were acquired from each subject under different vascular filling preparations expected to change exclusively the extracellular water fraction. Two exponential components were systematically observed: an intermediate component (T2 ~ 32 ms) and a long component (100 < T2 < 210 ms). The relative fraction and T2 value characterizing the long component systematically increased after progressive augmentation of extracellular water volume. Characteristic relaxation behavior for each vascular filling condition was analyzed with a two-site exchange model and a three-site two-exchange model. We show that a two-site exchange model can only predict the observations for small exchange rates, much more representative of transendothelial than transcytolemmal exchange regimes. The three-site two-exchange model representing the intracellular, interstitial, and vascular spaces was capable of precisely predicting the observations for realistic transcytolemmal and transendothelial exchange rates. The estimated intrinsic relative fractions of each of these compartments corroborate with estimations from previous works and strongly suggest that the T2 relaxation from water within the intracellular and interstitial spaces is described by the intermediate component, whereas the long component represents water within the vascular space.