Stojan Peric1, Ivo Bozovic1, Tanja Nisic2, Marija Banovic1, Milorad Vujnic3, Tamara Svabic1, Jovan Pesovic4, Marija Brankovic1, Ivana Basta1, Milena Jankovic1, Dusanka Savic-Pavicevic4, Vidosava Rakocevic-Stojanovic5. 1. Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr. Subotic Street, Belgrade, 11000, Serbia. 2. Clinic for Endocrinology, Diabetes and Metabolic Diseases, Clinical Center of Serbia, Dr Subotica starijeg 13, Belgrade, Serbia. 3. Neurology Clinic, Clinical Center Banja Luka, 12 beba street, Banja Luka, Bosnia and Herzegovina. 4. Center for Human Genetics, University of Belgrade - Faculty of Biology, Studentski trg 16, Belgrade, Serbia. 5. Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr. Subotic Street, Belgrade, 11000, Serbia. stojan.peric@gmail.com.
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.
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 DM1patients and 12 DM2patients. 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 DM2patients 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 DM2patients 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:DM1patients had visceral obesity, and percentage of FM correlated with a degree of muscle weakness in upper legs. In DM2patients, degree of muscle weakness was in correlation with higher FM index and lower bone mineral density.
Entities:
Keywords:
Body composition; Bone mineral density; Muscle weakness; Myotonic dystrophy type 1; Myotonic dystrophy type 2; Visceral obesity
Authors: Sebastiaan Dhont; Rutger Callens; Dieter Stevens; Fre Bauters; Jan L De Bleecker; Eric Derom; Eva Van Braeckel Journal: Acta Neurol Belg Date: 2020-10-14 Impact factor: 2.396