Body composition and its association with physical performance, quality of life, and clinical indictors in Charcot-Marie-Tooth disease: a pilot study.

Abstract

To investigate whether the amount and distribution of lean body mass and fat mass is associated with disease severity in adults with Charcot-Marie Tooth. Ten participants (age 46 ± 13 y, height 1.7 ± 0.1 m, and body mass 77 ± 17 kg) with Charcot-Marie Tooth disease were involved in this study. Participants were evaluated for quality of life, falls efficacy, balance, mobility, muscle strength, and power. Body composition was measured using dual energy x-ray absorptiometry. Statistical analyses were conducted on subsets of all participants. Better static balance was associated with higher lean body mass of the lower leg (r = 0.73, p = 0.03), while superior leg press strength and power was associated with greater lean body mass of the leg and lower leg (r ≥ 0.80, p ≤ 0.01). Faster habitual walking speed and enhanced quality of life was associated with lower fat mass of several regions. Our study seems to suggest that assessing of body composition could assist with monitoring of disease progression in people with Charcot-Marie Tooth; however these findings need to be substantiated in a larger cohort. Implications for Rehabilitation Higher lean body mass and lower fat mass of the legs is associated with better physical performances in people with Charcot-Marie-Tooth disease. Lower fat mass is related to greater quality of life and reduced clinical symptoms in people with Charcot-Marie-Tooth disease. Optimising favorable body composition profiles (higher lean body mass and lower fat mass) in people with Charcot-Marie-Tooth disease may be highly clinically relevant.