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Cerebral Amyloid Angiopathy With Progressive Cortical Superficial Siderosis and Convexity Subarachnoid Hemorrhage in an Apolipoprotein E (APOE) ε2/ε2 Homozygous Patient: A Case Report and Literature Review
Masahiro Hayashi, Mayumi Ikeda, Katsuji Kobayashi. Cureus, 2025-06-29
Topic: Brain proteins, microglia and lab models
It is associated with intracerebral hemorrhage (ICH), cognitive decline, and gait disturbance. Homozygosity for APOE ε2 is rare in the general population and has been associated with an elevated risk of hemorrhage in CAA, making this case particularly noteworthy. Imaging revealed recurrent cSAH and progressive cSS, along with simultaneous ICH and cSAH in opposite hemispheres. This case highlights the aggressive course of CAA in a patient with APOE ε2/ε2 homozygote, where repeated hemorrhagic events and iron accumulation may result in both vascular injury and parenchymal dysfunction.
Key findings
- It is associated with intracerebral hemorrhage (ICH), cognitive decline, and gait disturbance.
- Homozygosity for APOE ε2 is rare in the general population and has been associated with an elevated risk of hemorrhage in CAA, making this case particularly noteworthy.
- Imaging revealed recurrent cSAH and progressive cSS, along with simultaneous ICH and cSAH in opposite hemispheres.
- This case highlights the aggressive course of CAA in a patient with APOE ε2/ε2 homozygote, where repeated hemorrhagic events and iron accumulation may result in both vascular injury and parenchymal dysfunction.
Why it matters
This case report describes cerebral amyloid angiopathy (CAA) in a patient carrying two copies of APOE ε2, a rare genotype linked to a higher risk of bleeding in CAA. Imaging showed recurrent bleeding on the brain surface, progressive cortical superficial siderosis, and a bleed within the brain and a surface bleed on opposite sides at the same time. The authors say it highlights how aggressive CAA can be in ε2/ε2 carriers, with repeated bleeding and iron build-up possibly injuring both blood vessels and brain tissue.
Study details
- Evidence strengthLow
How this study scored 31/100
Published in a peer-reviewed journal. Scored zero for study design, sample size, effect reported, studied in people and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Cureus) |
| Journal's citation record | 1 / 4 | Cureus: journal h-index 131 (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 | 4 / 5 | 2025 — within 3 year(s) of the newest study here |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 0 / 3 | Not stated whether this was studied in people — scores zero (not stated in the record — scored 0) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 1 / 7 | Cited 1 times (OpenAlex, 2026-10-10) |
| Citations per year | 0 / 5 | 0.3 citations a year over 3 year(s) since 2025 |
| 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 | 0 / 4 | Cited 1 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 1 / 4 | Last-listed author Katsuji Kobayashi: h-index 16 (OpenAlex, whole career) |
| Institution's track record | 0 / 3 | National Hospital Organization Hokuriku Hospital: institution h-index 40 (OpenAlex) |
| Multi-centre collaboration | 1 / 3 | OpenAlex lists 2 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 40589441) |
| Free to read | 2 / 2 | Free to read |
| Total | 31 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
Full text: free to read — OpenAlex lists this copy as free to read: the link above (checked 6 Feb 1020).
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
Hayashi, M., Ikeda, M., & Kobayashi, K. (2025). Cerebral Amyloid Angiopathy With Progressive Cortical Superficial Siderosis and Convexity Subarachnoid Hemorrhage in an Apolipoprotein E (APOE) ε2/ε2 Homozygous Patient: A Case Report and Literature Review. Cureus. https://doi.org/10.7759/cureus.87007
Vancouver
Hayashi M, Ikeda M, Kobayashi K. Cerebral Amyloid Angiopathy With Progressive Cortical Superficial Siderosis and Convexity Subarachnoid Hemorrhage in an Apolipoprotein E (APOE) ε2/ε2 Homozygous Patient: A Case Report and Literature Review. Cureus. 2025. doi:10.7759/cureus.87007. PMID: 40589441.
BibTeX
@article{hayashi2025-40589441,
author = {Hayashi, Masahiro and Ikeda, Mayumi and Kobayashi, Katsuji},
title = {{Cerebral Amyloid Angiopathy With Progressive Cortical Superficial Siderosis and Convexity Subarachnoid Hemorrhage in an Apolipoprotein E (APOE) ε2/ε2 Homozygous Patient: A Case Report and Literature Review}},
journal = {Cureus},
year = {2025},
doi = {10.7759/cureus.87007},
pmid = {40589441},
url = {https://doi.org/10.7759/cureus.87007}
}
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