friends of FSH Research

Funding Research for
FSH Muscular Dystrophy Dots

Longitudinal Features of Stir Bright Signal in FSHD

Posted by George Shaw on June 7, 2013

Seth Friedman, Ph.D.
Seth Friedman, Ph.D.


Introduction: Magnetic resonance imaging shows short tau-inversion recovery (STIR) brightness in autosomal dominant facioscapulohumeral muscular dystrophy (FSHD1) suggestive of active inflammation/injury. We measured the longitudinal stability/progression of this potential disease biomarker. Methods: Nine subjects underwent calf MRI imaging over 2 years. Two radiologists evaluated qualitative muscle changes. Results: In 3/9 subjects, calf muscles demonstrated moderate/severe STIR hyperintensity at Time 1 that had progressed to fatty replacement 2 years later (Time 2). In the remaining subjects, moderate/severe muscle STIR abnormalities, when present, were consistent between exams. Mild STIR+ elevations had roughly similar patterns between exams. Conclusions: Moderate/severe STIR hyperintensities often foreshadow fatty replacement over a 2-year interval. Whether longer time courses are required to observe muscle degeneration and fatty replacement in some subjects remains to be explored.

To be published in Muscle & Nerve.

See also summary of longitudinal study grant by Friends of FSH Research: Longitudinal Progression of Edema and Fatty Replacement of Lower Extremity Muscles in FSHD.