Background: Little is known about the impact of nutrition status on Huntington's disease (HD) severity. Objective: To analyze the association of nutritional factors with HD severity. Methods: Observational, cross-sectional, national multicenter study. Participants were selected from a Spanish cohort of patients who participate in the European Huntington Disease Network (EHDN). The frequency of food consumption, caloric and nutrients intake in patients with HD were assessed using validated questionnaires for the Spanish population, and calculated using Alimentación and Salud, version 2.0, and the Spanish Dietary Recommended Intakes were used as the gold standard. Disease severity was assessed using the Unified HD Rating Scale (UHDRS) and Total Functional Capacity (TFC). Logistic regression models were performed using the TFC as the dependent variable, and the macro-micronutrients as the independent variables, adjusted for age, gender, education, physical activity, and intake of supplements. Results: Two hundred and twenty four patients with HD were included (59% women), mean age of 47.41 ± 14.26 years, median TFC 9 (3-13), median UHDRS motor score of 33.5 (3.75-56), 75.8% with normal-high caloric intake, 55.4% normal BMI, and 54.4% with medium-high adherence to the Mediterranean diet. Compared to patients with mild-moderate HD, patients with severe HD had higher caloric intake (p = 0.02), and similar BMI (p = 0.33). Advanced HD was associated with higher intake of water-soluble vitamins (OR = 2.08; 95% CI = 1.12-3.85, p = 0.02), and minerals (OR = 1.86; 95% CI = 1.09-3.19, p = 0.02). Conclusions and relevance: An adequate dietary intake prevents against weight loss in patients with advanced HD, but it is not associated with better functional state.

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