Objective: To assess skeletal muscle and fat mass, sarcopenia, and nutritional status in Huntington ́s Disease (HD) patients in different disease stages.

Background: HD is an autosomal dominant, neurodegenerative disorder with characteristic neurological symptoms including motor, behavioral, and cognitive impairment. Mutant Huntingtin and its aggregates may also affect peripheral tissue causing muscle and adipose tissue wasting.

Method: Bioelectrical impedance analysis was exploited to evaluate body composition in a prospective cohort of patients with symptomatic HD. The Unified HD Rating Scale and cognitive assessments were used to characterize the patients clinically.

Results: Twenty HD patients with a median age of 57 years, body mass index of 22 kg/m2, TMS of 17 points, and TFC of 10.5 points were included consecutively and prospectively. Confirmed sarcopenia (15%, n=3) was uncommon. Appendicular skeletal muscle mass was reduced in 60% (n=12) and body fat mass in 35% (n=7). Muscle mass reduction was significantly associated with low weight (p=0.049) and body fat (p=0.048). Patients in disease stage 3 (i.e., 3 ≤ UHDRS-TFC ≤ 6 points; n=14) had a lower weight (p=0.011), body fat mass (p=0.032) and nutritional status (p=0.035) compared to patients in disease stage 2 (i.e., 7 ≤ UHDRS-TFC ≤ 10 points; n=6). 55% were at risk for malnutrition or malnourished (Mini Nutritional Assessment). The latter correlated with weight, skeletal muscle and body fat mass, motor symptoms, independency, and executive function.

Conclusion: Reduction of muscle and fat mass are common in HD patients, which may impact functional capacity and well-being.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/muscle-mass-body-fat-mass-and-nutrition-in-patients-with-huntingtons-disease/