Association of Dementia With Mortality Among Adults With Down Syndrome Older Than 35 Years
Hithersay R, Startin CM, Hamburg S, Mok KY, Hardy J, Fisher EMC, Tybulewicz VLJ, Nizetic D et al.. JAMA neurology, 2019-02-01
Topic: Risk, prevention and care in population studies
Plain-language summary
Importance This work quantifies the fatal burden of dementia associated with Alzheimer disease in individuals with Down syndrome (DS). Objective To explore the association of dementia associated with Alzheimer disease with mortality and examine factors associated with dementia in adults with DS. Results Of the 211 participants, 96 were women (45.5%) and 66 (31.3%) had a clinical dementia diagnosis. For those with dementia, APOE ε4 carriers had a 7-fold increased risk of death (hazard ratio [HR], 6.91; 95% CI, 1.756-27.195). For those without dementia, epilepsy with onset after age 36 years was associated with mortality (HR, 9.66; 95% CI, 1.59-58.56). APOE ε4 carriers (HR, 4.91; 95% CI, 2.53-9.56), adults with early-onset epilepsy (HR, 3.61; 95% CI, 1.12-11.60), multiple health comorbidities (HR, 1.956; 95% CI, 1.087-3.519), and those living with family (HR, 2.14; 95% CI, 1.08-4.20) received significantly earlier dementia diagnoses. Conclusions and relevance Dementia was associated with mortality in 70% of older adults with DS. APOE ε4 carriers and/or people with multiple comorbid health conditions were at increased risk of dementia and death, highlighting the need for good health care. For those who died without a dementia diagnosis, late-onset epilepsy was the only significant factor associated with death, raising questions about potentially undiagnosed dementia cases in this group.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
APA 7th
Hithersay, R., Startin, C. M., Hamburg, S., Mok, K. Y., Hardy, J., Fisher, E. M. C., et al. (2019). Association of Dementia With Mortality Among Adults With Down Syndrome Older Than 35 Years. JAMA neurology. https://doi.org/10.1001/jamaneurol.2018.3616
Vancouver
Hithersay R, Startin CM, Hamburg S, Mok KY, Hardy J, Fisher EMC, et al. Association of Dementia With Mortality Among Adults With Down Syndrome Older Than 35 Years. JAMA neurology. 2019. doi:10.1001/jamaneurol.2018.3616. PMID: 30452522.
BibTeX
@article{hithersay2019-30452522,
author = {Hithersay, R. and Startin, C. M. and Hamburg, S. and Mok, K. Y. and Hardy, J. and Fisher, E. M. C. and Tybulewicz, V. L. J. and Nizetic, D. and others},
title = {{Association of Dementia With Mortality Among Adults With Down Syndrome Older Than 35 Years}},
journal = {JAMA neurology},
year = {2019},
doi = {10.1001/jamaneurol.2018.3616},
pmid = {30452522},
url = {https://doi.org/10.1001/jamaneurol.2018.3616}
}
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. The record's author list ends in “et al.”, so not every author is named. Check the citation against the original before you use it.
Key findings
- Importance This work quantifies the fatal burden of dementia associated with Alzheimer disease in individuals with Down syndrome (DS).
- Objective To explore the association of dementia associated with Alzheimer disease with mortality and examine factors associated with dementia in adults with DS.
- Results Of the 211 participants, 96 were women (45.5%) and 66 (31.3%) had a clinical dementia diagnosis.
- For those with dementia, APOE ε4 carriers had a 7-fold increased risk of death (hazard ratio [HR], 6.91; 95% CI, 1.756-27.195).
- For those without dementia, epilepsy with onset after age 36 years was associated with mortality (HR, 9.66; 95% CI, 1.59-58.56).
Why it matters
In 211 adults with Down syndrome over 35, 66 (31.3%) had a dementia diagnosis, and the authors report that "dementia was associated with mortality in 70% of older adults with DS." APOE ε4 carriers were diagnosed with dementia significantly earlier (HR 4.91), as were people with multiple other health conditions (HR 1.956). Among those with dementia, ε4 carriers had a higher risk of death, though the estimate is imprecise (HR 6.91, 95% CI 1.756–27.195). These are associations from one group of 211 people, and the authors' takeaway is "the need for good health care", not a treatment.
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Study details
- Study designlongitudinal study
- Participants300
- Effect size45.5%; 31.3%
- Evidence strengthModerate
How this study scored 70/100
Published in a leading specialty journal, and it is a longitudinal study following the same people over time. Scored zero for randomized / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 9 / 10 | Tier 2 — leading specialty journal (JAMA neurology) |
| Journal's citation record | 2 / 4 | JAMA Neurology: journal h-index 235 (OpenAlex, 2026-10-29) |
| Study design | 7 / 12 | A longitudinal study following the same people over time |
| Strength of evidence | 3 / 5 | Rated “moderate” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 2 / 5 | 2019 — within 10 year(s) of the newest study here |
| Sample size | 2 / 4 | 300 participants |
| Effect reported | 2 / 3 | Effect reported as a number, without an interval or test |
| Studied in people | 3 / 3 | Studied in people (participant count stated) |
| Randomized / blinded | 0 / 2 | No randomization or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 5 / 7 | Cited 174 times (OpenAlex, 2026-10-10) |
| Citations per year | 3 / 5 | 19.3 citations a year over 9 year(s) since 2019 |
| Impact for its field and year | 4 / 6 | Cited 16.0× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 4 / 6 | Cited by 31 other studies in this library |
| Still being cited | 3 / 4 | Cited 59 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 3 / 4 | Last-listed author André Strydom: h-index 54 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | University College London: institution h-index 2,029 (OpenAlex) |
| Multicenter collaboration | 2 / 3 | OpenAlex lists 9 institution(s) in 3 countries |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 30452522) |
| Free to read | 2 / 2 | Free to read |
| Total | 70 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
- DOI: 10.1001/jamaneurol.2018.3616
- PubMed: 30452522
- Original source →
- Free full text: JAMA Neurology →
Full text: free to read — OpenAlex lists this copy as free to read: the link above (checked 6 Feb 1020).
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