| Domain | Item | Key details/quantitative data | Evidence type | Primary source (with DOI/URL when available) |
|---|---|---|---|---|
| Identifiers/synonyms | Cerebral amyloid angiopathy (CAA) | Age-related cerebral small-vessel disease characterized by amyloid-β deposition in cortical and leptomeningeal vessel walls; major cause of lobar ICH and contributor to cognitive impairment/dementia. Common synonyms: cerebral β-amyloid angiopathy, congophilic angiopathy, amyloid angiopathy of the CNS (pqac-00000001, pqac-00000008) | Human clinicopathologic review; autopsy-validated diagnostic review | Cordonnier et al., 2025, doi:10.1177/17474930251365861, https://doi.org/10.1177/17474930251365861; Zotin et al., 2024, doi:10.1212/WNL.0000000000207940, https://doi.org/10.1212/WNL.0000000000207940 |
| Diagnostics | Boston criteria v2.0 | Probable CAA can be diagnosed with age ≥50 years plus appropriate clinical presentation and MRI showing either ≥2 strictly lobar hemorrhagic lesions (ICH, cerebral microbleeds, cSS/cSAH foci) or 1 strictly lobar hemorrhagic lesion plus 1 white-matter feature (severe CSO-PVS or WMH-MS), with absence of deep hemorrhagic lesions (pqac-00000001, pqac-00000002, pqac-00000007) | Human clinical/imaging criteria; guideline/review | Cordonnier et al., 2025, https://doi.org/10.1177/17474930251365861; Weidauer & Hattingen, 2025, doi:10.3390/biomedicines13030603, https://doi.org/10.3390/biomedicines13030603 |
| Diagnostics | Boston criteria v2.0 performance | Community autopsy-validated sample: sensitivity 38.8%, specificity 83.5% for probable CAA; Boston v1.0/v1.5 sensitivity 26.5%, specificity 90.6%/89.4%. v2.0 added ~12.3% sensitivity at cost of ~5.9% specificity decrease (pqac-00000008, pqac-00000042) | Human autopsy-validated diagnostic accuracy study | Zotin et al., Neurology 2024, doi:10.1212/WNL.0000000000207940, https://doi.org/10.1212/WNL.0000000000207940 |
| Diagnostics/imaging markers | Non-hemorrhagic MRI markers in v2.0 | Severe CSO-PVS defined as >20 visible perivascular spaces in the centrum semiovale on one slice in one hemisphere; WMH-MS defined as ≥10 small round/ovoid subcortical T2-FLAIR hyperintense lesions across the whole brain (pqac-00000008, pqac-00000042) | Human imaging methods from diagnostic study | Zotin et al., 2024, https://doi.org/10.1212/WNL.0000000000207940 |
| Genetics/risk | APOE ε4 | Major common genetic susceptibility factor; associated with CAA onset and recurrent CAA bleeding/hemorrhagic disease burden (pqac-00000003, pqac-00000005, pqac-00000006) | Human genetic association; review | Hu et al., 2025, doi:10.1080/07853890.2024.2445194, https://doi.org/10.1080/07853890.2024.2445194; Weidauer & Hattingen, 2025, https://doi.org/10.3390/biomedicines13030603 |
| Genetics/risk | APOE ε2 | Associated with CAA and vessel-fragility/hemorrhagic phenotype; highlighted alongside ε4 as important in CAA biology and risk stratification (pqac-00000015, pqac-00000016) | Human genetic association; review | Weidauer & Hattingen, 2025, https://doi.org/10.3390/biomedicines13030603; Banerjee et al., Brain 2023, doi:10.1093/brain/awad193, https://doi.org/10.1093/brain/awad193 |
| Monogenic early-onset CAA | APP, PSEN1, PSEN2 | Early-onset CAA may result from APP missense mutations/copy-number variants (including APP duplication), PSEN1, and PSEN2 mutations; presentations may be hemorrhagic, cognitive, or mixed. Dutch-type hereditary CAA is caused by APP p.E693Q and is considered a “pure” CAA form (pqac-00000009, pqac-00000011, pqac-00000016) | Human genetic review; pathophysiologic review | Banerjee et al., Brain 2023, https://doi.org/10.1093/brain/awad193; Koemans et al., Lancet Neurol 2023, doi:10.1016/S1474-4422(23)00114-X, https://doi.org/10.1016/S1474-4422(23)00114-X |
| Monogenic early-onset CAA | Other genes | Other monogenic early-onset/non-Aβ CAA genes include ITM2B, CST3, GSN, PRNP, and TTR; guideline review also lists cystatin-C, transthyretin, and BRI2 among non-Aβ monogenic causes (pqac-00000011, pqac-00000012, pqac-00000013) | Human genetic reviews/guideline | Banerjee et al., 2023, https://doi.org/10.1093/brain/awad193; Cordonnier et al., 2025, https://doi.org/10.1177/17474930251365861 |
| Iatrogenic transmission | Documented exposures | Reported sources include cadaveric dura mater grafts/Lyodura, cadaveric pituitary-derived human growth hormone (c-hGH), neurosurgical instrumentation, and embolized lyophilized dura; early-onset iatrogenic CAA increasingly recognized (pqac-00000010, pqac-00000012, pqac-00000014, pqac-00000017) | Human case reports/series; review | Koemans et al., 2023, https://doi.org/10.1016/S1474-4422(23)00114-X; Muller, 2023, doi:10.3389/fnins.2023.1185267, https://doi.org/10.3389/fnins.2023.1185267; Banerjee et al., Nat Med 2024, doi:10.1038/s41591-023-02729-2, https://doi.org/10.1038/s41591-023-02729-2 |
| Iatrogenic transmission | Latency | Published iatrogenic CAA cases linked to Aβ exposure show mean latency 34 years (range 25–46 years); c-hGH-related iatrogenic AD/Aβ amyloidosis cases had latency from exposure of 3–4 decades with symptom onset ages 38–55 years (pqac-00000010, pqac-00000018, pqac-00000022) | Human case-series/review; human cohort/biomarker study | Koemans et al., 2023, https://doi.org/10.1016/S1474-4422(23)00114-X; Banerjee et al., 2024, https://doi.org/10.1038/s41591-023-02729-2; Zhao et al., JAMA 2023, doi:10.1001/jama.2023.14445, https://doi.org/10.1001/jama.2023.14445 |
| Epidemiology | Age-related prevalence | Autopsy prevalence rises with age: 5–9% at ages 60–69, 43–58% at >90 years; among people >80 years, prevalence is 20–40% in cognitively normal individuals and 50–60% with cognitive impairment; histopathologic CAA is present in ~90% of AD cases (pqac-00000002, pqac-00000015) | Human autopsy/epidemiologic review | Weidauer & Hattingen, 2025, https://doi.org/10.3390/biomedicines13030603 |
| Epidemiology/outcomes | Recurrent hemorrhage burden | CAA-related lobar ICH recurrence estimated at ~7.4% per year in pathophysiologic framework review; Dutch-type hereditary CAA has annual recurrence >20% after first ICH, mean first lobar ICH at ~54 years (pqac-00000009) | Human cohort/review | Koemans et al., 2023, https://doi.org/10.1016/S1474-4422(23)00114-X |
| Prognostic imaging markers | Cortical superficial siderosis (cSS) | Disseminated/multifocal cSS is among the strongest predictors of future ICH; recurrent stereotyped TFNEs are linked to cSS/cSAH; presence/extent of cSS used for hemorrhage risk stratification (pqac-00000001, pqac-00000006, pqac-00000012) | Human imaging cohorts; guideline/review | Cordonnier et al., 2025, https://doi.org/10.1177/17474930251365861; Weidauer & Hattingen, 2025, https://doi.org/10.3390/biomedicines13030603 |
| Prognostic imaging markers | Strictly lobar microbleeds | Higher number of strictly cortical/lobar microbleeds improves diagnostic specificity and predicts recurrent ICH risk; true-positive Boston v2.0 cases had higher strictly cortical lobar microbleed burden than false positives (p=0.004) (pqac-00000006, pqac-00000008) | Human autopsy-validated diagnostic study; review | Zotin et al., 2024, https://doi.org/10.1212/WNL.0000000000207940; Weidauer & Hattingen, 2025, https://doi.org/10.3390/biomedicines13030603 |
| Prognostic imaging markers | CSO-PVS | Severe centrum semiovale enlarged perivascular spaces are a non-hemorrhagic MRI marker incorporated into Boston v2.0 and associated with CAA burden/hemorrhage risk stratification (pqac-00000001, pqac-00000042) | Human imaging criteria/guideline | Cordonnier et al., 2025, https://doi.org/10.1177/17474930251365861; Zotin et al., 2024, https://doi.org/10.1212/WNL.0000000000207940 |
| Prognostic imaging markers | WMH-MS | Multispot white matter hyperintensity pattern (WMH-MS) is the second non-hemorrhagic Boston v2.0 MRI feature; reflects small-vessel/white-matter injury and increases diagnostic sensitivity (pqac-00000001, pqac-00000042) | Human imaging criteria/guideline | Cordonnier et al., 2025, https://doi.org/10.1177/17474930251365861; Zotin et al., 2024, https://doi.org/10.1212/WNL.0000000000207940 |


*Table: Compact evidence table summarizing cerebral amyloid angiopathy identifiers, diagnostic criteria and performance, major genetic and iatrogenic causes, epidemiology, and key prognostic imaging markers. It is designed to support rapid knowledge-base population with quantitative details and source-linked evidence.*