Abstract
Thirty-two patients with facioscapulohumeral dystrophy (FSHD) underwent a prospective, longitudinal, observational study assessing MRI, quantitative strength measurements, and ambulatory functional outcome measures over a 12-month interval. While there was no significant decline in functional outcome measures or quantitative tibialis anterior strength, there was a small overall shift to higher fat fractions. The greatest increase in fat fraction were observed in muscles having intermediate fat replacement; with minimally (baseline fat fraction < 0.10) or severely (> 0.70) affected muscles less likely to progress.
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