Evaluating the body composition — its total lean body mass — of patients with Charcot-Marie-Tooth (CMT) disease can help determine disease progression, researchers report, with muscle mass in the legs of particular importance.
The study “Body composition and its association with physical performance, quality of life, and clinical indictors in Charcot-Marie-Tooth disease: a pilot study,” published in the journal Disability and Rehabilitation, found that CMT patients with higher lean body mass — calculated by subtracting total body weight from body fat — have better quality of life and lesser clinical symptoms.
CMT affects nerve cells to progressively destroy the communication between nerves and muscles. This results in muscle weakness and eventual disability.
It is well-known that low physical activity impacts a person’s body composition, commonly leading to greater fat deposition and reduced muscular mass. Poor body composition of fat relative to lean muscle can be a vicious cycle, making attempts at physical activity harder.
Researchers in Australia evaluated the impact body composition could have in the overall response and quality of life of patients with CMT.
They evaluated the body mass composition of 10 adults with CMT using a whole-body dual energy X-ray absorptiometry scanner. This method allowed researchers to differentiate and quantify the different types of tissues that constitute the body, such as bone, muscle, and fat.
The team also determined several physical performance features related to disease severity and progression, such falls efficacy, balance, mobility, and muscle strength and power.
Results showed that patients who presented better static balance had greater lean body mass of the lower leg. Superior leg press strength and power was also associated with greater lean body mass of the leg and lower leg. In addition, less total fat mass and accumulated fat in the legs were found to be linked to faster walking speed and greater life quality.
A comparison of body composition between CMT patients and healthy age-matched volunteers showed that only the legs, mainly the lower legs, had significant differences to what concerned lean body mass. No other parts of the body seemed to be significantly affected.
“These associations between lean body mass, fat mass, physical measures, quality of life, and clinical indicators suggests that optimizing favorable body composition profiles (higher lean body mass/lower fat mass) in people with CMT may be highly clinically relevant,” the researchers wrote.
Additional body composition studies in larger cohorts of patients are warranted to better understand its impact on CMT patient outcomes. In addition, assessing the impact of tailored resistance training and dietary interventions could help to identify treatment approaches of possible benefit.