title: Clinical Muscle Testing Compared with Whole-Body Magnetic Resonance Imaging in Facio-scapulo-humeral Muscular Dystrophy.
abstract: OBJECTIVE
  The objective of this study was to evaluate the clinical usefulness of whole-body magnetic resonance imaging (MRI) in facio-scapulo-humeral muscular dystrophy (FSHD).
  
  
  METHODS
  In 20 patients with genetically proven FSHD1, we prospectively assessed muscular involvement and correlated the results of semi-quantitative manual muscle testing and other parameters such as disease duration, creatine kinase (CK) levels and repeat length of the D4Z4 locus with whole-body MRI.
  
  
  RESULTS
  Clinical muscle testing revealed the trapezius, pectoralis and infraspinatus as the most severely affected muscles in the shoulder, and the knee flexors and gluteus medius in the hip girdle. MRI revealed the trapezius and serratus anterior muscles in the shoulder, and the hamstrings and adductor muscles in the hip girdle, as the most severely affected muscle groups. Overall, degrees of fatty degeneration on MRI scans correlated significantly with clinical weakness. Moreover, we could detect clear affection of the trunk muscles. Corresponding to earlier reports, asymmetric involvement was frequent in both clinical examination and MRI scoring. Moreover, MRI revealed inhomogeneous muscle degeneration in a considerable proportion of both, muscles and patients. Both clinical and MRI scores significantly correlated to disease duration, but not to fragment size or CK levels.
  
  
  CONCLUSIONS
  Fatty degeneration in whole-body MRI correlates well to clinical muscle testing of the extremities but gives more information on deeper or trunk muscles. It shows structural changes in muscular disorders and may become an excellent tool for assessment of muscle involvement and follow-up studies.
authors: Department of Neurology, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany. jens.regula@med.uni-heidelberg.de.
authors: J U
authors: JU
authors: Regula
authors: Department of Neuroradiology, University Hospital Heidelberg, Heidelberg, Germany.
authors: L
authors: L
authors: Jestaedt
authors: Department of Neurology, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany.
authors: F
authors: F
authors: Jende
authors: Department of Neuroradiology, University Hospital Heidelberg, Heidelberg, Germany.
authors: A
authors: A
authors: Bartsch
authors: Department of Neurology, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany.
authors: H-M
authors: HM
authors: Meinck
authors: Department of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Heidelberg, Germany.|Department of Radiology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
authors: M-A
authors: MA
authors: Weber
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D006801:Humans; D007089:Image Enhancement / Q000379:methods; D007090:Image Interpretation, Computer-Assisted / Q000379:methods; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging*; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000175:diagnosis* / Q000473:pathology*; D010808:Physical Examination / Q000379:methods*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D051598:Whole Body Imaging / Q000379:methods*; D055815:Young Adult
keywords: Clinical muscle testing; FSHD; Muscle MRI