Quantification of the inflammatory process in muscles of patients with facioscapulohumeral muscular dystrophy.

The aim of this study was to develop a method to quantify measures of severity of inflammation as seen on TIRM images in patients with fascioscapulohumeral muscular dystrophy (FSHD). Intensity (in standardized z-scores) and volume of inflammation were quantified on TIRM images in six muscles of three patients at baseline and follow-up. We observed that three processes occurred over time. The volume and intensity of inflammation both 1) increased, 2) decreased, 3) volume decreased, while intensity increased. Thus, the proposed method is able to quantify features of the inflammatory process in FSHD patients, which may be valuable to evaluate treatment.

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