Dementia Associated With Mortality in Adults With Down Syndrome

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Investigators observed that APOE ε4 carriers and/or individuals with multiple comorbid health conditions were at increased risk for dementia and death.
Investigators observed that APOE ε4 carriers and/or individuals with multiple comorbid health conditions were at increased risk for dementia and death.

Dementia is associated with mortality in older adults with Down syndrome (DS), according to research findings published in JAMA Neurology. Researchers examined the association between Alzheimer disease-related dementia and mortality in adults with DS, quantifying the fatal burden.

A prospective longitudinal study in a community setting in England enrolled 211 participants with DS 36 and older. Researchers collected data on dementia status, age, sex, APOE genotype, level of intellectual disability, health variables, and living situation. They analyzed crude mortality rates, time to death, and time to dementia diagnosis with proportional hazards of predictors.

Of the total participants (n=211), 45% were women and 31.3% had a clinical diagnosis of dementia. In 70% of older adults with DS, dementia was associated with mortality. During the study period, 27 participants died. The mortality rates in participants with dementia were 5 times higher than in participants without dementia. Individuals who were APOE ε4 carriers or who had comorbid health conditions had an increased risk for and earlier onset of dementia and death. Participants who died without a dementia diagnosis tended to have late-onset epilepsy, which may point to undiagnosed dementia.

The study was limited by a small sample size and data collection via informant report, potentially introducing bias.

The researchers stated that “Nearly all older adults with Down syndrome now have dementia when they die, making this a vital population for researching disease progression, modifying factors, and potential treatments.”

There is an urgent need for continued research and treatments focused on delaying or preventing dementia in this population. This research may have an impact on clinical care by identifying factors that increase the risk for dementia and mortality in patients with DS.

Reference

Hithersay R, Startin CM, Hamburg S, et al. Association of dementia with mortality among adults with down syndrome older than 35 years [published online November 19, 2018]. JAMA Neurol. doi:10.1001/jamaneurol.2018.3616

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