Language
English
Publication Date
8-1-2024
Journal
The Cerebellum
DOI
10.1007/s12311-023-01655-w
PMID
38217689
PMCID
PMC11269368
PubMedCentral® Posted Date
1-13-2024
PubMedCentral® Full Text Version
Post-print
Abstract
SCA6 patients with the same size CAG repeat allele can vary significantly in age at onset (AAO) and clinical progression. The specific external factors affecting SCA6 have yet to be investigated. We assessed the effect of early life events on AAO, severity, and progression in SCA6 patients using a social determinant of health approach. We performed a survey of biological and social factors in SCA6 patients enrolled in the SCA6 Network at the University of Chicago. AAO of ataxia symptoms and patient-reported outcome measure (PROM) of ataxia were used as primary outcome measures. Least absolute shrinkage and selection operation (LASSO) regressions were used to identify which early life factors are predictive of SCA6 AAO, severity, and progression. Multiple linear regression models were then used to assess the degree to which these determinants influence SCA6 health outcomes. A total of 105 participants with genetically confirmed SCA6 completed the assessments. SCA6 participants with maternal difficulty during pregnancy, active participation in school sports, and/or longer CAG repeats were determined to have earlier AAO. We found a 13.44-year earlier AAO for those with maternal difficulty in pregnancy than those without (p = 0.008) and a 12.31-year earlier AAO for those active in school sports than those who were not (p < 0.001). Higher education attainment was associated with decreased SCA6 severity and slower progression. Early life biological and social factors can have a strong influence on the SCA6 disease course, indicating that non-genetic factors can contribute significantly to SCA6 health outcomes.
Keywords
Humans, Female, Male, Age of Onset, Disease Progression, Adult, Middle Aged, Spinocerebellar Ataxias, Severity of Illness Index, Social Determinants of Health, Young Adult, Cerebellar ataxia, Spinocerebellar ataxia, Age of onset, Epidemiology, Cerebellum
Published Open-Access
yes
Recommended Citation
Chen, Tiffany X; Casey, Hannah L; Lin, Chi-Ying R; et al., "Early-Life Social Determinants of SCA6 Age at Onset, Severity, and Progression" (2024). Faculty, Staff and Students Publications. 7237.
https://digitalcommons.library.tmc.edu/baylor_docs/7237