Mauro Monforte1, Eugenio Mercuri2, Francesco Laschena3, Enzo Ricci4, Giorgio Tasca5. 1. Institute of Neurology, Catholic University School of Medicine, Rome, Italy. 2. Pediatric Neurology Unit, Catholic University School of Medicine, Rome, Italy. 3. Department of Radiology, Istituto Dermopatico dell'Immacolata, IRCCS, Rome, Italy. 4. Institute of Neurology, Catholic University School of Medicine, Rome, Italy. Electronic address: ericci@rm.unicatt.it. 5. Don Carlo Gnocchi Onlus Foundation, Italy.
Abstract
INTRODUCTION: Calf hypertrophy is a common feature in Becker muscular dystrophy (BMD), and it is still debated to which extent fatty degeneration or true muscle hypertrophy account for it. We wanted to investigate the relative contribution of these two components using a simple image analysis approach and their possible correlation with disease severity. METHODS: Twenty-nine BMD patients' MRI scans were analyzed. A semiquantitative visual score assessing fatty replacement of calf muscles (calf MRI score, CMS) was calculated and correlated with the cross sectional area (CSA) of lower leg posterior compartment muscles, digitally measured on acquired images. RESULTS: The correlation between CSA and CMS was not significant. CMS in contrast correlated with disease severity (p<0.001) while CSA did not (p=0.969). CONCLUSIONS: In BMD, a major contribution to calf hypertrophy is provided by real muscle hypertrophy rather than by fatty degeneration. CMS appears to be a potential surrogate marker of disease severity.
INTRODUCTION:Calfhypertrophy is a common feature in Becker muscular dystrophy (BMD), and it is still debated to which extent fatty degeneration or true muscle hypertrophy account for it. We wanted to investigate the relative contribution of these two components using a simple image analysis approach and their possible correlation with disease severity. METHODS: Twenty-nine BMDpatients' MRI scans were analyzed. A semiquantitative visual score assessing fatty replacement of calf muscles (calf MRI score, CMS) was calculated and correlated with the cross sectional area (CSA) of lower leg posterior compartment muscles, digitally measured on acquired images. RESULTS: The correlation between CSA and CMS was not significant. CMS in contrast correlated with disease severity (p<0.001) while CSA did not (p=0.969). CONCLUSIONS: In BMD, a major contribution to calfhypertrophy is provided by real muscle hypertrophy rather than by fatty degeneration. CMS appears to be a potential surrogate marker of disease severity.
Authors: Sarah Finlayson; Jasper M Morrow; Pedro M Rodriguez Cruz; Christopher D J Sinclair; Arne Fischmann; John S Thornton; Steve Knight; Ray Norbury; Mel White; Michal Al-Hajjar; Nicola Carboni; Sandeep Jayawant; Stephanie A Robb; Tarek A Yousry; David Beeson; Jacqueline Palace Journal: Muscle Nerve Date: 2016-02-22 Impact factor: 3.217
Authors: Melissa T Hooijmans; Martijn Froeling; Zaida Koeks; Jan J G M Verschuuren; Andrew Webb; Erik H Niks; Hermien E Kan Journal: NMR Biomed Date: 2020-08-05 Impact factor: 4.044