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

Aim: 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.

Methods: 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.

Results: 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.

Conclusion: 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.