Clinical and nutritional assessment of patients with Huntington's disease. Spanish multicenter study of the european group for Huntington's disease

Abstract

Huntington's disease (HD) is a neurodegenerative disease with motor impairment, psychiatric and cognitive symptoms, global prevalence is 2.7 to 5.0 per 100 000 population, with onset in the fourth decade of life and survival of 15–20 years. From an etiological point of view, nutritional disturbances in HD may have a multifactorial origin. Objectives: To describe and analyze the association between clinical and nutritional characteristics in patients with HD. We accomplish a Spanish multicenter, cross-sectional study of the European Register of HD. HD patients were included with genetic confirmation. Demographic and clinical information were collected using the unified scale EH (UHDRS). Anthropometric and nutritional survey data were included too. Total Energy Expenditure (TEE) was calculated using the Harris-Benedict equation. We present data from the first 22 patients included: 63.6% women, mean age 57 ± 15.0 years, of which, in relation to the stage of the disease, 33.3% with stage I, 9.5% in II, 38.1% in III and IV 19.1%. According to the WHO classification of Body Mass Index (BMI), 9.1% are underweight, 54.6% normal, 18.2% overweight and 18.2% obese. Dysphagia is present in 36.4%. BMI correlated inversely with the number of CAG repeats HTT-15 gene (correlation coefficient -0.521, p = 0.006). The average dietary intake was 2289 ± 608 kcal/day, with an average energy balance of -551 kcal/day (range -1348 to +743 kcal/day), and 25% average energy balance was -1189 ± 41 kcal/day. Energy distribution of macronutrients was as follows: 38.9% lipids, 18.4% proteins and carbohydrates 41.2%. Data presented are preliminary, because the study is ongoing. EH patients would require greater caloric intake than healthy individuals to maintain proper energy balance and BMI, also, taking into account the balanced distribution of macronutrients. Improving nutritional status could potentially change the course of the disease to prevent secondary medical complications (recurrent infections, fractures, etc.) associated to poor nutritional status, enhancing health and avoiding medical and social costs.