title: Regional body composition and functional impairment in patients with myotonic dystrophy.
abstract: BACKGROUND
  In this study we determined regional body composition in myotonic dystrophy (DM1) and able-bodied controls and evaluated the relationship between fat and lean tissue mass and functional impairment in DM1 patients.
  
  
  METHODS
  Dual-energy X-ray absorptiometry (DEXA) was used to obtain regional measurements of fat-free mass index (FFMI) and fat mass index (FMI) in 48 DM1 and anthropometrically matched control pairs.
  
  
  RESULTS
  DM1 patients had lower regional FFMI and higher FMI than controls (P < 0.01-0.001). In DM1 patients, total FMI increased significantly with increased muscular disability rating, decreased motor function measurement, and with both decreasing vital capacity and total lung capacity. Hypertriglyceridemia correlated with increasing FMI.
  
  
  CONCLUSIONS
  Regional FFMI is decreased in DM1, whereas FMI is underestimated by body mass index and is negatively correlated with patients' functional capacity. DEXA may provide valuable supporting evidence in the management of DM1.
authors.affiliation: Centre de Référence des Maladies Neuromusculaires, Médecine Interne Orientée vers les Maladies Orphelines et Systémiques, Tour Drouet, CHU de Nancy, Hôpitaux de Brabois, 54511 Vandoeuvre Cedex, France.
authors.forename: Lelia
authors.initials: L
authors.lastname: Pruna
authors.forename: Jerome
authors.initials: J
authors.lastname: Chatelin
authors.forename: Veronique
authors.initials: V
authors.lastname: Pascal-Vigneron
authors.forename: Pierre
authors.initials: P
authors.lastname: Kaminsky
mesh_terms: D015502:Absorptiometry, Photon / Q000379:methods; D000273:Adipose Tissue / Q000473:pathology; D000293:Adolescent; D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D000886:Anthropometry / Q000379:methods; D001823:Body Composition; D015992:Body Mass Index; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008176:Lung Volume Measurements / Q000379:methods; D008297:Male; D008875:Middle Aged; D009043:Motor Activity / Q000502:physiology; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology*; D009223:Myotonic Dystrophy / Q000473:pathology* / Q000503:physiopathology; D012720:Severity of Illness Index; D055815:Young Adult