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.

Diffusion tensor imaging reveals subclinical alterations in muscles of patients with Becker muscular dystrophy / S. Nava, G. Conte, F.M. Triulzi, G.P. Comi, F. Magri, D. Velardo, C.M. Cinnante. - In: BRITISH JOURNAL OF RADIOLOGY. - ISSN 0007-1285. - 97:1157(2024 May 07), pp. 947-953. [10.1093/bjr/tqae070]

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

G. Conte
Secondo
;
F.M. Triulzi;G.P. Comi;C.M. Cinnante
Ultimo
2024

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.
Becker muscular disease; diffusion tensor imaging; subclinical; tractography;
Settore MED/37 - Neuroradiologia
7-mag-2024
4-apr-2024
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/1076793
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