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Atrophy patterns in cerebral amyloid angiopathy with and without cortical superficial siderosis
Kim J, Na HK, Shin JH, Kim HJ, Seo SW, Seong JK, Na DL.. Neurology, 2018-04-20
Topic: Amyloid, tau and blood-test diagnosis
Objective To investigate differential atrophy patterns based on the presence of cortical superficial siderosis (cSS) and the role of cSS in predicting amyloid positivity in memory clinic patients fulfilling the diagnostic criteria for probable cerebral amyloid angiopathy (CAA). Methods We retrospectively collected data from 44 cognitively impaired patients with probable CAA who underwent 3-dimensional, T1-weighted MRIs (cSS+, n = 27; cSS-, n = 17). Amyloid-positive patients with Alzheimer disease (AD) (n = 56) and amyloid-negative cognitively normal participants (n = 34) were recruited as controls. Among the patients with CAA who underwent amyloid-PET scans (75.0%), we investigated whether amyloid-negative cases were unevenly distributed based on cSS presentation. APOE genotypes, Mini-Mental State Examination scores, and cortical atrophy pattern along with hippocampal volume were compared across groups. Results Ten patients with probable CAA presented amyloid negativity and all of them belonged to the cSS- group (58.8%). Compared to the cSS- group, the cSS+ group presented higher APOE ε4 frequency, worse memory dysfunction, and lower hippocampal volume. Compared with cognitively normal participants, the cSS+ group exhibited atrophy in the precuneus, posterior cingulate, parietotemporal, superior frontal, and medial temporal areas, a pattern similar to AD-specific atrophy. The cSS- group exhibited atrophy in the parietotemporal, superior frontal, and precentral regions. Conclusion Our findings imply that the current version of the Boston criteria may not be sufficient enough to remove non-CAA cases from a cognitively impaired population, especially in the absence of cSS. Patients with probable CAA presenting cSS appear to reflect a CAA phenotype that shares pathologic hallmarks with AD, providing insight into the CAA-to-AD continuum.
Key findings
- Results Ten patients with probable CAA presented amyloid negativity and all of them belonged to the cSS- group (58.8%).
- Compared to the cSS- group, the cSS+ group presented higher APOE ε4 frequency, worse memory dysfunction, and lower hippocampal volume.
- Compared with cognitively normal participants, the cSS+ group exhibited atrophy in the precuneus, posterior cingulate, parietotemporal, superior frontal, and medial temporal areas, a pattern similar to AD-specific atrophy.
- Conclusion Our findings imply that the current version of the Boston criteria may not be sufficient enough to remove non-CAA cases from a cognitively impaired population, especially in the absence of cSS.
Why it matters
Among patients with probable cerebral amyloid angiopathy, those with cortical superficial siderosis had more Alzheimer-like brain atrophy and lower hippocampal volume, and all amyloid-negative cases were in the group without siderosis. This is about imaging patterns and diagnosis, not about treating, curing, or preventing Alzheimer's disease.
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Study details
- Participants27
- Effect size75.0%; 58.8%
- Evidence strengthLow
How this study scored 46/100
Published in a leading specialty journal. Scored zero for study design and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 9 / 10 | Tier 2 — leading specialty journal (Neurology) |
| Journal's citation record | 4 / 4 | Neurology: journal h-index 525 (OpenAlex, 2026-10-29) |
| 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 | 2 / 5 | 2018 — within 10 year(s) of the newest study here |
| Sample size | 1 / 4 | 27 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) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 3 / 7 | Cited 16 times (OpenAlex, 2026-10-10) |
| Citations per year | 1 / 5 | 1.6 citations a year over 10 year(s) since 2018 |
| Impact for its field and year | 1 / 6 | Cited 0.6× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 0 / 6 | No other study in this library cites it |
| Still being cited | 1 / 4 | Cited 8 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 3 / 4 | Last-listed author Duk Lyul Na: h-index 67 (OpenAlex, whole career) |
| Institution's track record | 1 / 3 | Sungkyunkwan University: institution h-index 788 (OpenAlex) |
| Multi-centre collaboration | 2 / 3 | OpenAlex lists 6 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 29678935) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 46 / 100 | — |
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Read the original
May need a subscription Unpaywall, Europe PMC and OpenAlex found no free copy of the full text (checked 10 Oct 2026), 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. The record names 7 authors; APA and Vancouver show the first six, BibTeX and the downloads carry all of them. Check the citation against the original before you use it.
APA 7th
Kim, J., Na, H. K., Shin, J. H., Kim, H. J., Seo, S. W., Seong, J. K., et al. (2018). Atrophy patterns in cerebral amyloid angiopathy with and without cortical superficial siderosis. Neurology. https://doi.org/10.1212/wnl.0000000000005524
Vancouver
Kim J, Na HK, Shin JH, Kim HJ, Seo SW, Seong JK, et al. Atrophy patterns in cerebral amyloid angiopathy with and without cortical superficial siderosis. Neurology. 2018. doi:10.1212/wnl.0000000000005524. PMID: 29678935.
BibTeX
@article{kim2018-29678935,
author = {Kim, J. and Na, H. K. and Shin, J. H. and Kim, H. J. and Seo, S. W. and Seong, J. K. and Na, D. L.},
title = {{Atrophy patterns in cerebral amyloid angiopathy with and without cortical superficial siderosis}},
journal = {Neurology},
year = {2018},
doi = {10.1212/wnl.0000000000005524},
pmid = {29678935},
url = {https://doi.org/10.1212/wnl.0000000000005524}
}
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