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Differential susceptibility to neurofibrillary pathology among patients with Down syndrome
Wegiel J, Wisniewski HM, Dziewiatkowski J, Popovitch ER, Tarnawski M.. Dementia (Basel, Switzerland), 1996-05-01
Topic: Brain proteins, microglia and lab models
Individual differences in the development of neurofibrillary changes were examined in eight cortical regions in the brains of 43 subjects with Down syndrome (DS; age range, 15-69 years) using sections stained with monoclonal antibodies (mAb) tau-1 and 3-39. Neurofibrillary pathology was found in 4 cases below 36 years of age and in all 20 cases above that age. In the 24 positive cases, numerical density of pretangles stained with tau-1 and 3-39, respectively, was 6.1/mm2 and 0/mm2; early tangles, 5.0/mm2 and 5.3/mm2; mature tangles, 4.0/mm2 and 5.0/mm2 (p < 0.01); and end-stage tangles, 0.04/mm2 and 2.5/mm2 (p < 0.001). Numerical density of pretangles stained with mAb tau-1 and tangles and plaques stained with mAb 3-39 correlates weakly with age (r = 0.43; p< 0.02), and together with the wide range of numerical densities suggested heterogeneity of the population examined. Cluster analysis based on two variables - i.e., numerical density of pretangles stained with mAb tau-1 and neurofibrillary tangles (NFTs) and plaques stained with mAB 3-39, distinguished three groups of subjects with severe, moderate and weak changes. The severely affected group of 5 subject (21%) had an average 54.6/mm2 of neurons and 13.9/mm/ plaques with neurofibrillary changes, whereas the moderately affected group (6 subjects; 25%) showed a significantly lower numerical density of neurons and plaques with neurofibrillary changes (25.7/mm2 and 8.1/mm2, respectively) as compared with the most affected group. Most of the subjects (13; 54%) belong to the third group with only 2.2/mm2 of neurons and 1.4/mm2 plaques with neurofibrillary pathology. Comparison of these three groups of Down syndrome subjects representing high, moderate, and low susceptibility to neurofibrillary changes with the general population suggests that the risk of Alzheimer disease is similar but the onset of pathological changes is earlier in DS.
Key findings
- The severely affected group of 5 subject (21%) had an average 54.6/mm2 of neurons and 13.9/mm/ plaques with neurofibrillary changes, whereas the moderately affected group (6 subjects; 25%) showed a significantly lower numerical density of neurons and plaques with neurofibrillary changes (25.7/mm2 and 8.1/mm2, respectively) as compared with the most affected group.
- Comparison of these three groups of Down syndrome subjects representing high, moderate, and low susceptibility to neurofibrillary changes with the general population suggests that the risk of Alzheimer disease is similar but the onset of pathological changes is earlier in DS.
Why it matters
Among 43 people with Down syndrome, neurofibrillary pathology was found in all 20 cases above 36 years of age, with wide variation in severity between individuals. The authors suggest this indicates differing susceptibility to Alzheimer-type changes, but no treatment, cure, or prevention was tested.
Study details
- Participants43
- Evidence strengthLow
How this study scored 36/100
Published in a peer-reviewed journal. Scored zero for study design, effect reported and randomised / blinded because the record does not state it. We have no data on journal's citation record, so that was left out: it earned 35 of the 96 points we could judge it on, which is 36 out of 100.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Dementia (Basel, Switzerland)) |
| Journal's citation record | — / 4 | No journal record from OpenAlex — left out of this score |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 1996 — older than 20 years, still counted |
| Sample size | 1 / 4 | 43 participants |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 3 / 3 | Studied in people (participant count stated) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 3 / 7 | Cited 29 times (OpenAlex, 2026-10-10) |
| Citations per year | 0 / 5 | 0.9 citations a year over 32 year(s) since 1996 |
| Impact for its field and year | 2 / 6 | Cited 2.9× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 2 / 6 | Cited by 6 other studies in this library |
| Still being cited | 0 / 4 | Cited 0 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 2 / 4 | Last-listed author Michal J Tarnawski: h-index 23 (OpenAlex, whole career) |
| Institution's track record | 0 / 3 | Gdańsk Medical University: institution h-index 365 (OpenAlex) |
| Multi-centre collaboration | 1 / 3 | OpenAlex lists 2 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 8740627) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Points | 35 / 96 | Scored on 19 of 20 parts. We have no data for the rest, so they are left out: 35 of the 96 points we could judge it on is 36 out of 100. |
| Total | 36 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
May need a subscription OpenAlex found no free copy of the full text (checked 6 Feb 1020), so the publisher may charge for it. The PubMed abstract is free.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. Check the citation against the original before you use it.
APA 7th
Wegiel, J., Wisniewski, H. M., Dziewiatkowski, J., Popovitch, E. R., & Tarnawski, M. (1996). Differential susceptibility to neurofibrillary pathology among patients with Down syndrome. Dementia (Basel, Switzerland). https://doi.org/10.1159/000106868
Vancouver
Wegiel J, Wisniewski HM, Dziewiatkowski J, Popovitch ER, Tarnawski M. Differential susceptibility to neurofibrillary pathology among patients with Down syndrome. Dementia (Basel, Switzerland). 1996. doi:10.1159/000106868. PMID: 8740627.
BibTeX
@article{wegiel1996-8740627,
author = {Wegiel, J. and Wisniewski, H. M. and Dziewiatkowski, J. and Popovitch, E. R. and Tarnawski, M.},
title = {{Differential susceptibility to neurofibrillary pathology among patients with Down syndrome}},
journal = {Dementia (Basel, Switzerland)},
year = {1996},
doi = {10.1159/000106868},
pmid = {8740627},
url = {https://doi.org/10.1159/000106868}
}
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