Abstract
Lifestyle could influence the course of hereditary ataxias, but representative data are missing. The objective of this study was to characterize lifestyle in spinocerebellar ataxia type 3 (SCA3) and investigate possible associations with disease parameters. In a prospective cohort study, data on smoking, alcohol consumption, physical activity, physiotherapy, and body mass index (BMI) were collected from 243 patients with SCA3 and 119 controls and tested for associations with age of onset, disease severity, and progression. Compared with controls, patients with SCA3 were less active and consumed less alcohol. Less physical activity and alcohol abstinence were associated with more severe disease, but not with progression rates or age of onset. Smoking, BMI, or physiotherapy did not correlate with disease parameters. Differences in lifestyle factors of patients with SCA3 and controls as well as associations of lifestyle factors with disease severity are likely driven by the influence of symptoms on behavior. No association between lifestyle and disease progression was detected. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
Highlights
Bonferroni correction was applied with number of hypotheses (m)= 19 to correct for multiple testing in the primary analysis of the association between lifestyle factors and measures of disease severity (SARA score, ADL score, progression rate, age of onset)
We found that higher alcohol consumption was significantly associated with less severe disease, which does not mean that alcohol consumption prevents severe stages of spinocerebellar ataxia type 3 (SCA3) but may be interpreted as a hint that patients with more severe ataxia refrain from alcohol, as the ethyltoxic aggravation of the movement disorder is no longer tolerable
The significant association between higher levels of physical activity and less severe disease can be interpreted in a similar way, which is that patients with more severe ataxia may not be able to engage in much physical activity
Summary
Bonferroni correction was applied with number of hypotheses (m)= 19 to correct for multiple testing in the primary analysis of the association between lifestyle factors (alcohol, smoking, BMI, physical activity, physiotherapy) and measures of disease severity (SARA score, ADL score, progression rate, age of onset). We analyzed if these lifestyle factors were associated with disease severity as assessed by the SARA score, rAOO, annual SARA progression rate, and ADL score (Fig. 1, Supplemental Table S1).
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