Body composition analysis in patients with myotonic dystrophy types 1 and 2

Neurol Sci. 2019 May;40(5):1035-1040. doi: 10.1007/s10072-019-03763-0. Epub 2019 Feb 21.

Abstract

Introduction: To date, there are only several reports on body composition in myotonic dystrophy type 1 (DM1) and there are no data for myotonic dystrophy type 2 (DM2). The aim was to analyze body composition of patients with DM1 and DM2, and its association with socio-demographic and clinical features of the diseases.

Methods: There were no statistical differences in sociodemographic features between 20 DM1 patients and 12 DM2 patients. Body composition was assessed by DEXA (dual-energy x-ray absorptiometry). A three-compartment model was used: bone mineral content (BMC), fat mass (FM), and lean tissue mass (LTM).

Results: Patients with DM1 and DM2 had similar total body mass (TBM), BMC, FM, and LTM. Patients with DM1 had higher trunk-limb fat index (TLFI) in comparison to DM2 patients which indicates visceral fat deposition in DM1 (1.16 ± 0.32 for DM1 vs. 0.87 ± 0.23 for DM2, p < 0.05). Right ribs bone mineral density was lower in DM2 group (0.68 ± 0.07 g/cm2 vs. 0.61 ± 0.09 g/cm2, p < 0.05). Higher percentage of FM in legs showed correlation with lower strength of the upper leg muscles in DM1 (ρ = - 0.47, p < 0.05). Higher muscle strength in DM2 patients was in correlation with higher bone mineral density (ρ = + 0.62, p < 0.05 for upper arm muscles, ρ = + 0.87, p < 0.01 for lower arm muscles, ρ = + 0.72, p < 0.05 for lower leg muscles).

Conclusion: DM1 patients had visceral obesity, and percentage of FM correlated with a degree of muscle weakness in upper legs. In DM2 patients, degree of muscle weakness was in correlation with higher FM index and lower bone mineral density.

Keywords: Body composition; Bone mineral density; Muscle weakness; Myotonic dystrophy type 1; Myotonic dystrophy type 2; Visceral obesity.

MeSH terms

  • Absorptiometry, Photon
  • Adult
  • Body Composition*
  • Bone Density
  • Female
  • Humans
  • Male
  • Middle Aged
  • Muscle Strength
  • Muscle Weakness / diagnostic imaging
  • Muscle Weakness / epidemiology
  • Muscle Weakness / pathology
  • Muscle Weakness / physiopathology
  • Muscle, Skeletal / diagnostic imaging
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology
  • Myotonic Dystrophy* / diagnostic imaging
  • Myotonic Dystrophy* / epidemiology
  • Myotonic Dystrophy* / pathology
  • Myotonic Dystrophy* / physiopathology
  • Obesity, Abdominal / diagnostic imaging
  • Obesity, Abdominal / epidemiology
  • Obesity, Abdominal / pathology
  • Obesity, Abdominal / physiopathology