Dataset Information

The association between educational attainment and SCA 3 age of onset and disease course.

ABSTRACT:

Background

The number of trinucleotide CAG repeats is inversely correlated with the age at onset (AAO) of motor symptoms in individuals with Spinocerebellar Ataxia type 3 (SCA 3) and may be responsible for 50%-60% of the variability in AAO. Drawing from a social determinants of health model, we sought to determine if educational attainment further contributes to the AAO and motor symptom progression of SCA 3.

Methods

We performed a retrospective chart review in which twenty individuals met criteria for inclusion and had been seen by an ataxia specialist at our hospital between January 2005 and July 2019. AAO of motor symptoms and Scale for Assessment and Rating of Ataxia (SARA) scores were used as primary outcome measures.

Results

Using a linear regression, we found that having greater CAG repeat length and greater than 16 years of education results in an earlier AAO. The importance of the CAG repeat length on AAO, however, is greater amongst individuals with lower education. Using a linear mixed model evaluating SARA score over time with AAO, we found that less than 16 years of education is associated with faster progression of the disease.

Conclusion

In our group of SCA 3 patients, level of education correlated with both the AAO and SARA scores. Though our findings need to be confirmed with a larger cohort, our study suggests that level of education can have a strong influence on health outcomes in SCA 3 and possibly other groups of patients with ataxia.

SUBMITTER: Iannuzzelli K

PROVIDER: S-EPMC10498785 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature

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Publications

The association between educational attainment and SCA 3 age of onset and disease course.

Iannuzzelli Katherine K Shi Rosa R Carter Reece R Huynh Rachel R Morgan Owen O Kuo Sheng-Han SH Bang Jee J Mills Kelly A KA Baranano Kristin K Zee David S DS Moukheiber Emile E Roda Ricardo R Butala Ankur A Marvel Cherie C Joyce Michelle M Li Ximin X Wang Jiangxia J Rosenthal Liana S LS

Parkinsonism & related disorders 20220310

<h4>Background</h4>The number of trinucleotide CAG repeats is inversely correlated with the age at onset (AAO) of motor symptoms in individuals with Spinocerebellar Ataxia type 3 (SCA 3) and may be responsible for 50%-60% of the variability in AAO. Drawing from a social determinants of health model, we sought to determine if educational attainment further contributes to the AAO and motor symptom progression of SCA 3.<h4>Methods</h4>We performed a retrospective chart review in which twenty indivi ...[more]

PMID: 35635856

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Dataset Information

The association between educational attainment and SCA 3 age of onset and disease course.

Background

Methods

Results

Conclusion

Publications

The association between educational attainment and SCA 3 age of onset and disease course.

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