Objectives: Certain heart conditions and diseases are common in Down syndrome (DS; trisomy 21), but their role in early onset dementia that is prevalent in older adults with DS has not been evaluated. To address this knowledge gap, we conducted a study of risk factors for low neurocognitive/behavioral scores obtained with a published dementia test battery (DTB). Participants were adults with DS living in New York (N = 29; average age 46 years). We asked three questions. 1. Does having any type of heart disease affect the association between DTB scores and chronological age? 2. Does thyroid status affect the association between heart disease and DTB scores? 3. Are the E4 or E2 alleles of apolipoprotein E (APOE) associated with DTB scores or with heart disease?
Method: The study was retrospective, pilot, and exploratory. It involved analysis of information in a database previously established for the study of aging in DS. Participants had moderate intellectual disability on average. Information for each person included: gender, age, a single DTB score obtained by combining results from individual subscales of the DTB, the presence or absence of heart disease, thyroid status (treated hypothyroidism or normal), and APOE genotype. Trends were visualized by inspection of graphs and contingency tables. Statistical methods used to evaluate associations included Pearson correlation analysis, Fisher's exact tests (2-tailed), and odds ratio analysis. P values were interpreted at the 95% confidence level without Bonferroni correction. P values >.05<.1 were considered trends.
Results: The negative correlation between DTB scores and age was significant in those with heart disease but not in those without. Heart disease was significantly associated with DTB scores >1 SD below the sample mean; there was a strong association between heart disease and low DTB scores in those with treated hypothyroidism but not in those with normal thyroid status. The APOE genotype was weakly associated with heart disease (E4, predisposing; E2, protective) in males.
Conclusions: On the basis of the potentially important findings from the present study, large prospective studies are warranted to confirm and extend the observations. In these, particular heart conditions or diseases and other medical comorbidities in individuals should be documented.
Keywords: Alzheimer’s disease; Down syndrome; apolipoprotein E; cognitive function; dementia; dementia test battery; heart disease; hypoperfusion; hypothyroidism.