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Alzheimer's Disease in Down Syndrome
Elizabeth Head1, David Powell2, Brian T Gold3
1Department of Molecular and Biomedical Pharmacology, University of Kentucky Lexington, KY, 40356 ; Sanders-Brown Center on Aging, University of Kentucky Lexington, KY, 40356.
European Journal of Neurodegenerative Disease
|October 7, 2014
Summary
Adults with Down syndrome (DS) face higher risks of cognitive decline and Alzheimer disease (AD). Frontal impairments and white matter changes may precede dementia in DS, alongside other health conditions.
Area of Science:
- Neurology
- Gerontology
- Genetics
Background:
- Aging adults with Down syndrome (DS) exhibit an elevated risk for cognitive decline, dementia, and Alzheimer disease (AD).
- Dementia prevalence in DS individuals aged 40-59 years is substantial, with reported ranges of 5.7-55% and 4-55%, respectively.
- A hallmark of aging in DS is the consistent, progressive accumulation of AD neuropathology, with nearly all individuals showing AD brain pathologies by age 40.
Purpose of the Study:
- To explore the hypothesis that frontal impairments are a critical factor in cognitive function decline in aging adults with DS.
- To investigate the association between frontal impairments, white matter (WM) abnormalities, and AD neuropathology in DS.
- To review other clinical comorbidities that may contribute to dementia onset in DS.
Main Methods:
- Review of existing literature on cognitive decline, dementia, and AD in Down syndrome.
- Analysis of the relationship between neuropathological findings (senile plaques, neurofibrillary tangles) and clinical presentation of dementia.
- Examination of the role of frontal lobe function and white matter integrity in cognitive changes.
Main Results:
- A dissociation exists between the early onset of AD neuropathology (by age 40) and the clinical manifestation of dementia in DS.
- Frontal impairments are hypothesized to be a significant factor affecting cognitive function in aging DS individuals.
- White matter abnormalities and AD neuropathology are associated with frontal impairments.
Conclusions:
- Frontal impairments and associated white matter changes may serve as early indicators for dementia conversion in Down syndrome.
- Clinical comorbidities in DS may also play a role in the development of dementia.
- Further research is needed to understand the complex interplay of factors leading to dementia in aging individuals with DS.
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