Myotonic dystrophy type 1 (DM1) is an inherited muscle disorder characterized by slowly progressive weakness due to muscle degeneration. The Muscular Impairment Rating Scale (MIRS) is validated to assess clinical muscle severity of patients with DM1, although the scale is not sensitive enough to assess disease progression in time intervals fit for clinical trials. The aim of this study was to analyze bioelectrical whole body and arm segmental parameters in patients with DM1 to explore a correlation between bioelectrical impedance analysis (BIA) parameters and disease stage.
Forty patients with DM1 were enrolled in a cross-sectional study. In all patients, MIRS, handgrip strength (HGS), and BIA were assessed. A Kruskal-Wallis test was used to assess the difference in continuous variables according to MIRS. Correlation between BIA values and HGS were made by Pearson's coefficient analysis. A linear regression analysis was performed.
Eighteen of 40 patients were men (45%). The median age of the cohort was 42 y (30-58 y). Four patients (10%) were classified as MIRS 1; 20 (50%) MIRS 2; 11 (27.5%) MIRS 3; and 5 (12.5%) as MIRS 4. A correlation was observed between phase angle and MIRS (P = 0.0001). MIRS correlated with other BIA values such as resistance, impedance ratio, and capacitance (P = 0.005, P = 0.0001, P = 0.0006, respectively). At linear regression analysis, segmental resistance, phase angle, impedance ratio, and capacitance of both arms significantly correlated with HGS.
Results from the study support the use of BIA as a suitable procedure for staging DM1 muscle involvement and as a measure of muscle disease outcome, in clinical practice and in clinical trial design of therapeutic drugs.
- Handgrip strength test
- Impedance ratio
- Muscular Impairment Rating Scale (MIRS)
- Personalized medicine
- Phase angle
- Steinert's disease