The association between lifestyle factors and mortality in Huntington's disease.

Background: Despite the wealth of evidence suggesting a protective role of lifestyle factors on Huntington's disease (HD) onset and progression, their association with mortality has not been extensively studied. The aim of this study was to examine whether lifestyle factors such as caffeine and alcohol consumption, smoking, physical activity level, and Mediterranean diet (MeDi) adherence are associated with mortality among a Spanish cohort of patients with HD with a 9-year surveillance period.

Methods: This national study was performed using a nested, observational, longitudinal design. We included subjects diagnosed with HD who participated in the European Huntington's disease network and Enrol-HD studies. Date of death and baseline lifestyle factor information, demographics, disease severity assessed by the Unified Huntington's Disease Rating Scale (UHDRS), Problem Behaviours Assessment, total functional capacity (TFC) scores, and comorbidities were collected. Adjusted Cox proportional hazards models were conducted to determine the association of lifestyle factors with mortality.

Results: We included 87 patients (52 females) with a mean age of 48.62±14.43 years and CAG repeats of 43.76±5.92. Sixteen deaths were recorded. After correcting for multiple comparisons, deceased patients had higher UHDRS scores at baseline and lower caffeine consumption than live patients. In multivariate Cox regression models, after adjusting for age, CAG repeats, and TFC, mortality risk was associated with lower caffeine consumption (HR 0.13, 95% CI 0.04, 0.45).

Conclusions: This national-based study suggests that increased caffeine consumption is associated with decreased HD mortality. Our results may help guide clinicians in counselling lifestyle practices for decreasing mortality in HD.