Ulrike Bonati1, Maurice Schmid1, Patricia Hafner2, Tanja Haas3, Oliver Bieri3, Monika Gloor3, Arne Fischmann4, Dirk Fischer1,2,5. 1. Division of Neuropaediatrics, University Children's Hospital Basel, Spitalstrasse 33, Basel, 4031, Switzerland. 2. Department of Neurology, University Hospital Basel, Basel, Switzerland. 3. Division of Radiological Physics, Department of Radiology, University of Basel Hospital, Basel, Switzerland. 4. Division of Neuroradiology, University Hospital Basel, Basel, Switzerland. 5. University Clinic of Internal Medicine, Kantonsspital Baselland, Bruderholz, Switzerland.
Abstract
INTRODUCTION: Quantitative MRI techniques detect disease progression in myopathies more sensitively than muscle function measures or conventional MRI. To date, only conventional MRI data using visual rating scales are available for measurement of disease progression in Becker muscular dystrophy (BMD). METHODS: In 3 patients with BMD (mean age 36.8 years), the mean fat fraction (MFF) of the thigh muscles was assessed by MRI at baseline and at 1-year follow-up using a 2-point Dixon approach (2PD). The motor function measurement scale (MFM) was used for clinical assessment. RESULTS: The mean MFF of all muscles at baseline was 61.6% (SD 7.6). It increased by 3.7% to 65.3% (SD 4.7) at follow-up. The severity of muscle involvement varied between various muscle groups. CONCLUSIONS: As in other myopathies, 2PD can quantify fatty muscle degeneration in BMD and can detect disease progression in a small sample size and at relatively short imaging intervals.
INTRODUCTION: Quantitative MRI techniques detect disease progression in myopathies more sensitively than muscle function measures or conventional MRI. To date, only conventional MRI data using visual rating scales are available for measurement of disease progression in Becker muscular dystrophy (BMD). METHODS: In 3 patients with BMD (mean age 36.8 years), the mean fat fraction (MFF) of the thigh muscles was assessed by MRI at baseline and at 1-year follow-up using a 2-point Dixon approach (2PD). The motor function measurement scale (MFM) was used for clinical assessment. RESULTS: The mean MFF of all muscles at baseline was 61.6% (SD 7.6). It increased by 3.7% to 65.3% (SD 4.7) at follow-up. The severity of muscle involvement varied between various muscle groups. CONCLUSIONS: As in other myopathies, 2PD can quantify fatty muscle degeneration in BMD and can detect disease progression in a small sample size and at relatively short imaging intervals.
Authors: Pierre G Carlier; Benjamin Marty; Olivier Scheidegger; Paulo Loureiro de Sousa; Pierre-Yves Baudin; Eduard Snezhko; Dmitry Vlodavets Journal: J Neuromuscul Dis Date: 2016-03-03
Authors: Giacomo P Comi; Erik H Niks; Claudia M Cinnante; Hermien E Kan; Krista Vandenborne; Rebecca J Willcocks; Daniele Velardo; Michela Ripolone; Jules J van Benthem; Nienke M van de Velde; Simone Nava; Laura Ambrosoli; Sara Cazzaniga; Paolo U Bettica Journal: Muscle Nerve Date: 2021-12-30 Impact factor: 3.852