I09 Energy Balance In Huntington's Disease

Introduction: Little is known about the energy needs in Huntington's disease (HD). The aims of this study are to analyze and compare the total energy expenditure (TEE) and energy balance (EB) in a representative sample of HD patients with healthy controls. Methods: This is an observational, case-control single-center study. Food caloric energy intake (EI) and TEE were considered for estimating EB. A dietary recall questionnaire was used to assess the EI. TEE was computed as the sum of resting energy expenditure (REE), measured by indirect calorimetry and physical activity (PA) monitored by an actigraph. Results: A total of 22 patients were included (36% men, mean age 50.3 ± 15.6 years, motor Unified Huntington's Disease Scale 27.9 ± 23.7, total functional capacity 11.0 (7.0-13.0), EI 38.6 ± 10.0 kcal/kg, PA 5.3 (3.0-7.4) kcal/kg, REE 30.9 ± 6.4 kcal/kg, TEE 2,023.4 (1,592.0-2,226.5) kcal/day) and 18 controls (50% men, mean age 47.4 ± 13.8 years, EI 38.6 ± 10.3 kcal/kg, PA 8.4 (5.0-13.8) kcal/kg, REE 30.8 ± 6.6 kcal/kg, TEE 2,281.0 (2,057.3-2,855.3) kcal/day). TEE was significantly lower in patients compared to controls (p = 0.03). PA was lower in patients compared to controls (p = 0.02). Conclusions: Although patients with HD appeared to have lower energy expenditure, mainly due to decreased voluntary PA, they were still able to maintain their energy needs with an adequate food intake.

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Circulating and cerebrospinal fluid ghrelin and leptin: potential role in altered body weight in Huntington's disease

Acta Endocrinologica ◽

10.1530/eje.0.1510451 ◽

2004 ◽

pp. 451-455 ◽

Cited By ~ 66

Author(s):

V Popovic ◽

M Svetel ◽

M Djurovic ◽

S Petrovic ◽

M Doknic ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Energy Balance ◽

Huntington's Disease ◽

Huntington’S Disease ◽

Healthy Subjects ◽

Rating Scale ◽

Neurological Impairment ◽

Negative Energy ◽

Normal Weight ◽

Energy Output

OBJECTIVE: In addition to neurological impairment, weight loss is a prominent characteristic of Huntington's disease (HD). Neuropathologically, the disease affects the caudate nucleus and the cerebral cortex, and also the hypothalamus. The recently discovered orexigenic hormone of gastric origin, ghrelin and the adipocyte hormone leptin, are two peripherally produced hormones exerting opposite effects on specific populations of hypothalamic neurons that play a key role in regulating energy intake and energy output. The aim of this study was to investigate the possible involvement of cerebrospinal fluid (CSF) and circulating ghrelin and leptin in the regulation of energy balance in patients with HD. METHODS: Twenty healthy normal-weight subjects undergoing orthopedic surgery, and fifteen patients with genetically verified HD, were enrolled in this study. The unified Huntington's disease rating scale (UHDRS) was used to assess clinical course of the disease. Blood samples for hormonal measurements were obtained by venipuncture and in-parallel CSF samples for leptin/ghrelin determination were obtained by lumbar puncture. RESULTS: Patients with HD had increased concentrations of ghrelin in plasma compared with healthy subjects (4523.7+/-563.9 vs 2781.1+/-306.2 pg/ml, P<0.01). On the other hand, patients with HD had decreased concentrations of leptin in plasma compared with healthy subjects (4.8+/-1.6 vs 10.9+/-2.4 ng/ml, P<0.01). The concentrations of CSF ghrelin and CSF leptin were equivalent to values in healthy subjects. No correlation was found between disease duration--and other clinical features of HD--and plasma or CSF leptin/ghrelin levels. In patients with HD, baseline levels of GH, IGF-I, insulin and glucose did not differ from those in healthy subjects. CONCLUSION: High circulating ghrelin and low leptin levels in patients with HD suggest a state of negative energy balance. Early nutritional support of patients with HD is advocated since patients with HD and higher body mass index at presentation have slower progression of the disease.

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