title: Quantitative MRI reveals decelerated fatty infiltration in muscles of active FSHD patients.
abstract: OBJECTIVE
  To investigate the effects of aerobic exercise training (AET) and cognitive-behavioral therapy (CBT), directed towards an increase in daily physical activity, on the progression of fatty infiltration and edema in skeletal muscles of patients with facioscapulohumeral muscular dystrophy (FSHD) type 1 by T2 MRI.
  
  
  METHODS
  Quantitative T2 MRI (qT2 MRI) and fat-suppressed T2 MRI of the thigh were performed at 3T on 31 patients, 13 of whom received usual care (UC), 9 AET, and 9 CBT. Muscle-specific fat fractions (%), derived from qT2 MRI, were recorded pretreatment and posttreatment. Intervention effects were analyzed by comparing fat fraction progression rates of the UC with the treated groups using Mann-Whitney tests, and intermuscle differences by a linear mixed model. Edematous hyperintense lesions were identified on the fat-suppressed T2 MRI.
  
  
  RESULTS
  The intraclass correlation coefficient for reproducibility of qT2 MRI fat assessment was 0.99. In the UC group, the fat fraction increased by 6.7/year (95% confidence interval [CI] 4.3 to 9.1). This rate decreased to 2.9/year (95% CI 0.7 to 5.2) in the AET (p = 0.03) and 1.7/year (95% CI -0.2 to 3.6) in the CBT group (p = 0.00015). The treatment effect differed among individual muscles. Fewer new edematous lesions occurred after therapy.
  
  
  CONCLUSIONS
  Fat fraction derived from qT2 MRI is a reproducible and sensitive biomarker to monitor the effects of increased physical activity in individual muscles. This biomarker reports a favorable effect of AET and CBT on the rate of muscular deterioration in FSHD as reflected in decelerated fat replacement.
  
  
  METHODS
  This study provides Class II evidence that for patients with FSHD type 1, both AET and CBT decrease the rate of fatty infiltration in muscles.
authors.affiliation: From the Departments of Rehabilitation (N.V., A.G.), Radiology and Nuclear Medicine (B.J., A.H.), and Neurology (B.v.E.), and Donders Center for Neuroscience (A.G., B.v.E.), Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Barbara
authors.initials: B
authors.lastname: Janssen
authors.affiliation: From the Departments of Rehabilitation (N.V., A.G.), Radiology and Nuclear Medicine (B.J., A.H.), and Neurology (B.v.E.), and Donders Center for Neuroscience (A.G., B.v.E.), Radboud University Medical Center, Nijmegen, the Netherlands. N.Voet@Klimmendaal.nl.
authors.forename: Nicoline
authors.initials: N
authors.lastname: Voet
authors.affiliation: From the Departments of Rehabilitation (N.V., A.G.), Radiology and Nuclear Medicine (B.J., A.H.), and Neurology (B.v.E.), and Donders Center for Neuroscience (A.G., B.v.E.), Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Alexander
authors.initials: A
authors.lastname: Geurts
authors.affiliation: From the Departments of Rehabilitation (N.V., A.G.), Radiology and Nuclear Medicine (B.J., A.H.), and Neurology (B.v.E.), and Donders Center for Neuroscience (A.G., B.v.E.), Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Baziel
authors.initials: B
authors.lastname: van Engelen
authors.affiliation: From the Departments of Rehabilitation (N.V., A.G.), Radiology and Nuclear Medicine (B.J., A.H.), and Neurology (B.v.E.), and Donders Center for Neuroscience (A.G., B.v.E.), Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Arend
authors.initials: A
authors.lastname: Heerschap
mesh_terms: D000273:Adipose Tissue / Q000000981:diagnostic imaging* / Q000503:physiopathology; D015928:Cognitive Behavioral Therapy; D018450:Disease Progression; D004487:Edema / Q000000981:diagnostic imaging / Q000503:physiopathology; D015444:Exercise / Q000502:physiology; D005081:Exercise Therapy / Q000379:methods / Q000401:mortality; D005260:Female; D006801:Humans; D007090:Image Interpretation, Computer-Assisted / Q000379:methods*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging* / Q000503:physiopathology; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000628:therapy*; D015203:Reproducibility of Results; D013848:Thigh / Q000000981:diagnostic imaging / Q000503:physiopathology; D016896:Treatment Outcome