Weight loss is common among patients with Huntington's disease (HD), althou
gh the mechanisms contributing to this phenomenon are not known. We measure
d 24-hour sedentary energy expenditure (24-hour EE) and sleeping metabolic
rate (SMR) in a human respiratory chamber in 17 patients with mild to moder
ate HD and 17 control subjects matched for age, sex, and body mass index. T
otal energy expenditure was measured during 7 days in free-living condition
s, using the doubly labeled water technique, Body weight, fat mass, and fat
-free mass (measured by dual-energy x-ray absorptiometry) were similar in p
atients with HD and control subjects. Twenty-four-hour EE was 14% higher in
HD patients than controls in absolute terms (2,038 +/- 98 vs 1,784 +/- 68
kca1/24 hours) and after adjustment for age, sex, fat mass, and fat-free ma
ss (1,998 +/- 45 vs 1,824 +/- 45 kcal/24 hours). In contrast, SMR and total
energy expenditure were similar in patients and controls both in absolute
terms (1,314 +/- 38 vs 1,316 +/- 42 and 2,402 +/- 102 vs 2,373 +/- 98 kcal/
24 hours, respectively) and after adjustment. Spontaneous physical activity
measured by radar in the chamber and the ratio of 24-hour EE to SMR were s
ignificantly higher in HD patients than controls (11.4 +/- 1.4 vs 6.1 +/- 0
.6% and 1.54 +/- 0.05 vs 1.36 +/- 0.03, respectively). In the group as a wh
ole, 24-hour EE/SMR correlated with spontaneous physical activity. Among HD
patients, both 24-hour EE/SMR and spontaneous physical activity correlated
with the severity of chorea, but SMR and total energy expenditure did not.
There were no differences in reported energy intake during 7 days in patie
nts with HD compared with controls. The results of this study indicate that
sedentary energy expenditure is higher in patients with I-ID than in contr
ols in proportion to the severity of the movement disorder. Total free-livi
ng energy expenditure is not higher, however, because patients with HD appe
ar to engage in less voluntary physical activity.