Huntington Disease (HD) is a genetic neurologic disorder characterized by uncontrollable dance-like physical movements, dystonia, impairment in gait and eye movement, cognitive impairment, and psychiatric symptoms.
Predictions of increased basal metabolic rate as a cause of weight loss in HD are consistent with observations of a lower average BMI in presymptomatic carriers of the HD gene and in persons with very early symptoms of liD than in healthy control subjects. However, these previous studies have not ruled out increased energy expenditure (EE) with HD from voluntary movement, such as fidgeting, which may be related to body mass. Therefore, a group of researchers from New York compared 24-hour EE and energy intake in persons with early midstage HD with those of matched controls to determine how HD affects energy balance.
Energy expenditure was measured in 13 subjects with early-stage HD and in 9 controls via indirect calorimetry in a human respiratory chamber. Body composition was assessed to determine fat-flee mass and percentage body fat for each subject by using air-displacement plethysmography. Energy intake was determined by weighing all food provided and all leftovers from an ad libitum diet. Stage of disease was estimated based on the Unified Huntington's Disease Rating Scale and modified Mini-Mental Status examinations.
The 24-hour EE was 11% higher in the HD subjects than in the controls. This difference was due to a higher waking metabolic rate, which was related to a significantly greater displacement of the center of mass by HD subjects than by controls. Both groups were in positive energy balance and exceeded their energy expenditure. However, under flee-living conditions as measured by repeated 24-hour recalls, intake varied much more in the HD subjects than in the controls. The variability in energy intake also increased with the progression of the disease.
Higher 24-hour EE in persons with early midstage HD is due to increased physical activity, both voluntary and involuntary. However, HD subjects are able to maintain positive energy balance when offered adequate amounts of food in a controlled setting. The variability of energy intake are likely related to the weight loss observed with HD and should be taken into account in nutritional evaluations of persons with HD. Further studies of patients with late-stage HD are warranted.
A. M. Gaba, Kuan Zhang, Karen Marder, et al. Energy Balance in Early-Stage Huntington Disease. Am J Clin Nutr; 81:1335-1341 (June, 2005). [Address reprint requests to A. M. Gaba, Department of Food and Nutrition Services, Milstein Hospital Building 8-211, 177 Fort Washington Avenue, New York, NY 10032. E-mail: firstname.lastname@example.org]
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