Abstract

Imaging has become a valuable tool in the assessment of neuromuscular diseases, and, specifically, quantitative MR imaging provides robust biomarkers for the monitoring of disease progression. Quantitative evaluation of fat infiltration and quantification of the T2 values of the muscular tissue's water component (wT2) are two of the most essential indicators currently used. As each voxel of the image can contain both water and fat, a two-component model for the estimation of wT2 must be used. In this work, we present a fast method for reconstructing wT2 maps obtained from conventional multi-echo spin-echo (MESE) acquisitions and released as Free Open Source Software. The proposed software is capable of fast reconstruction thanks to extended phase graphs (EPG) simulations and dictionary matching implemented on a general-purpose graphic processing unit. The program can also perform more conventional biexponential least-squares fitting of the data and incorporate information from an external water-fat acquisition to increase the accuracy of the results. The method was applied to the scans of four healthy volunteers and five subjects suffering from facioscapulohumeral muscular dystrophy (FSHD). Conventional multi-slice MESE acquisitions were performed with 17 echoes, and additionally, a 6-echo multi-echo gradient-echo (MEGE) sequence was used for an independent fat fraction calculation. The proposed reconstruction software was applied on the full datasets, and additionally to reduced number of echoes, respectively, to 8, 5, and 3, using EPG and biexponential least-squares fitting, with and without incorporating information from the MEGE acquisition. The incorporation of external fat fraction maps increased the robustness of the fitting with a reduced number of echoes per datasets, whereas with unconstrained fitting, the total of 17 echoes was necessary to retain an independence of wT2 from the level of fat infiltration. In conclusion, the proposed software can successfully be used to calculate wT2 maps from conventional MESE acquisition, allowing the usage of an optimized protocol with similar precision and accuracy as a 17-echo acquisition. As it is freely released to the community, it can be used as a reference for more extensive cohort studies.

Highlights

  • Neuromuscular disorders encompass genetic and acquired diseases of lower motor neurons, peripheral nerves, neuromuscular junction, or skeletal muscle, generally causing different degrees of motor impairment in the affected patients

  • As the method is intended for multi-echo spin-echo acquisitions, a Carr-Purcell-Meiboom-Gill (CPMG) [31, 32] simulation is performed at every run of the program, adapting the timing and the number of echoes to the actual sequence parameters

  • The software fitted the multi-echo spin-echo (MESE) datasets with extended phase graph (EPG) dictionary matching in an average time of 2 s/slice, plus 12 s per dataset for the generation of the dictionary

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Summary

Introduction

Neuromuscular disorders encompass genetic and acquired diseases of lower motor neurons, peripheral nerves, neuromuscular junction, or skeletal muscle, generally causing different degrees of motor impairment in the affected patients. Muscular dystrophies are hereditary degenerative disorders of skeletal muscles, causing progressive replacement of muscle tissue by fat This happens through disparate pathological processes and molecular mechanisms that are, at least to some extent, disease-specific and related to the peculiar genetic defect that characterizes each of them [1]. Some of these broad pathological processes are shared by most muscular dystrophies. Different pathological processes are generally present simultaneously in the same patient or even in the same muscle group

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