Diffusion tensor imaging reveals subclinical alterations in muscles of patients with Becker muscular dystrophy

Author:

Nava Simone1ORCID,Conte Giorgio1,Triulzi Fabio M1,Comi Giacomo P23,Magri Francesca23,Velardo Daniele23,Cinnante Claudia M4

Affiliation:

1. Neuroradiology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico , via Francesco Sforza 35 , 20122 Milan, Italy

2. Neuromuscular and Rare Diseases Unit, Department of Neuroscience, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico , via Francesco Sforza 35 , 20122 Milan, Italy

3. Dino Ferrari Center, Department of Pathophysiology and Transplantation, University of Milan , via Francesco Sforza 35 , 20122 Milan Italy

4. Radiology Department, Istituto Auxologico Italiano IRCCS , Piazzale Brescia 20 , 20149 Milan, Italy

Abstract

Abstract Objectives Becker muscular dystrophy (BMD) is a relatively less investigated neuromuscular disease, partially overlapping the phenotype of Duchenne dystrophy (DMD). Physiopathological and anatomical patterns are still not comprehensively known, despite recent effort in the search of early biomarkers. Aim of this study was to selectively compare normal appearing muscles of BMD with healthy controls. Methods Among a pool of 40 BMD patients and 20 healthy controls, Sartorius and gracilis muscles were selected on the basis of a blinded clinical quantitative/qualitative evaluation, if classified as normal (0 or 1 on Mercuri scale) and subsequently segmented on diffusion tensor MRI scans with a tractographic approach. Diffusion derived parameters were extracted. Results Non-parametric testing revealed significant differences between normal and normal appearing BMD derived parameters in both muscles, the difference being more evident in sartorius. Bonferroni-corrected P-values (<.05) of Mann-Whitney test could discriminate between BMD and controls for standard deviation of all diffusion parameters (mean diffusivity, fractional anisotropy, axial and radial diffusivity) in both sartorius and gracilis, while in sartorius the significant difference was found also in the average values of the same parameters (with exception of RD). Conclusions This method could identify microstructural alterations in BMD normal appearing sartorius and gracilis. Advances in knowledge Diffusion based MRI could be able to identify possible early or subclinical microstructural alterations in dystrophic patients with BMD.

Publisher

Oxford University Press (OUP)

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