Muscular involvement assessed by MRI correlates to motor function measurement values in oculopharyngeal muscular dystrophy
Fischmann A, Gloor M, Fasler S, Haas T, Rodoni Wetzel R, Bieri O, Wetzel S, Heinimann K, Scheffler K, Fischer D.
J Neurol 2011. 258: 1333–1340.
Oculopharyngeal muscular dystrophy (OPMD) is a progressive skeletal muscle dystrophy characterized by ptosis, dysphagia, and upper and lower extremity weakness. Muscles of 8 patients with a genetically confirmed OPMD were analyzed by MRI and patients’ physical capacities were assessed using the MFM. A high (negative) correlation between the validated clinical scores and the visual imaging scores was observed, suggesting that quantitative and more objective muscle MRI might serve as outcome measure for clinical trials in muscular dystrophies.
Keywords: MFM, Outcome measure, Muscle, Oculopharyngeal muscular dystrophy, OPMD
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