Background Weight loss is common among patients with Huntington’s disease (HD), although the mechanisms contributing to this phenomenon are not well known.

Objective To analyse the relationship between dietary intake and HD severity.

Methods National, multicenter, observational, cross-sectional study (EHDN). The frequency of food consuming, caloric and nutrients intake at an individual, and population level in pre-symptomatic and symptomatic patients with HD were assessed using validated questionnaires for the Spanish population, and the Spanish Dietary Recommended Intakes (SDRI). In addition, nutritional state was estimated by the body mass index (BMI), disease severity using the Unified HD Rating Scale (UHDRS), psychiatric status (PBA-s), dysphagia (EAT-10), caregiver burden (summacare), comorbidities (CIRS-G), and quality of life (SF-36 questionnaire).

Results Two hundred and twenty four patients with HD were included (59% women) with a mean age of 47.41 ± 14.26, median Total Functional Capacity (TFC) 9 (13–3), and median UHDRS motor score 33.5 (56–3.75). In this population, 55% had moderate-high adherence to the Mediterranean diet, 24% had low caloric intake, 6,7% were underweight, 55.4% had normal weight; 37.9% were overweight-obese, 22% were on vitamins/supplements (VS) and 2% PEG. The use of antidopaminergic was associated with normal BMi (p = 0.02), and patients on VS had lower comorbidities (p = 0.03). Both, high and low functional capacity (TFC score > and < 6, respectively) were mostly associated with normal macro/micronutrients intake.

Conclusions In this study, conducted in the Spanish HD population, patients' dietary intake was mostly associated with normal weight and normal nutritional intake. Longitudinal studies are warranted to evaluate the efficacy of supplementary dietary intakes to prevent HD related comorbidities.