LONGITUDINAL FEATURES OF STIR BRIGHT SIGNAL IN FSHD

作者：Friedman Seth D^*; Poliachik Sandra L; Otto Randolph K; Carter Gregory T; Budech Christopher B; Bird Thomas D; Miller Daniel G; Shaw Dennis W W

来源：Muscle & Nerve, 2014, 49(2): 257-260.

DOI：10.1002/mus.23911

• facioscapulohumeral muscular dystrophy
• magnetic resonance
• Muscular dystrophy
• muscle

Introduction: Magnetic resonance imaging of muscle 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. Muscle Nerve 49: 257-260, 2014

• 出版日期2014-2