Background Little is known about the energy needs in Huntington’s disease (HD).

Aims To analyse and compare the total energy expenditure (TEE) and energy balance (EB) in a representative sample of HD patients included in the European Huntington’s Disease Registry with healthy controls, homogeneous in age, gender and body mass index.

Methods Observational, case-control single centre study. To estimate the EB, food caloric-energy intake (EI) and TEE were considered. To assess EI, a 24-hour dietary recall questionnaire was collected. TEE was computed as the sum of resting energy expenditure (REE) measured by indirect calorimetry, physical activity (PA) monitored by an actigraph, and dietary induced thermogenesis. The results were adjusted by fat free mass measured by bioelectrical impedance.

Results 22 patients (36% males, mean age 50.2 ± 15.6 years, motor UHDRS 27.9 ± 23.7, TFC 11.0 (13.0–7.0), cognitive UHDRS 167.0 (242.5–100.2), CAG 43.0 ± 6.0, EI 38.5 ± 9.9 kcal/kg, PA 5.3 (7.4–3.0) kcal/kg, REE 30.8 ± 6.3 kcal/kg, TEE 40.5 ± 7.9 kcal/kg), and 18 controls (50% males, mean age 47.4 ± 13.7, EI 38.6 ± 10.3 kcal/kg, PA 8.4 (13.7–4.9) kcal/kg, REE 30.7 ± 6.6 kcal/kg, TEE 44.0 ± 7.8 kcal/kg) were included. EB was similar in both groups (patients -2.0 ± 11.7 kcal/kg; controls -5.3 ± 11.2 kcal/kg; p = 0.36). PA was lower in patients compared to controls (p = 0.01).

Conclusions Although patients with HD appear to have lower energy expenditure mainly due to decreased voluntary physical activity, they are still able to maintain their energy needs with an adequate food intake.