Quantitative 2-point Dixon MRI detected disease progression in Becker muscular dystrophy, with mean fat fraction of thigh muscles increasing from 61.6% at baseline to 65.3% at 1-year follow-up.
Observational (n=3)
Does 2-point Dixon MRI detect disease progression in patients with Becker muscular dystrophy?
2-point Dixon MRI can quantify fatty muscle degeneration and detect disease progression over 1 year in patients with Becker muscular dystrophy.
Effect estimate: 3.7% increase
Absolute Event Rate: 65.3% vs 61.6%
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.
Schmid et al. (2015) conducted an observational in Becker muscular dystrophy (n=3). 2-point Dixon approach (2PD) MRI vs. Baseline was evaluated on Mean fat fraction (MFF) of the thigh muscles (3.7% increase). Quantitative 2-point Dixon MRI detected disease progression in Becker muscular dystrophy, with mean fat fraction of thigh muscles increasing from 61.6% at baseline to 65.3% at 1-year follow-up.