From:  Cerebral amyloid angiopathy: updates on pathophysiology, diagnosis, and management

 Key studies in CAA.

AuthorsStudy objectiveSetting/ParticipantsResultsNotes
Banerjee et al. [60] (2020)To assess CSF biomarkers in patients with CAACAA (n = 10), AD (n = 20), controls (n = 10)Reduced CSF Aβ40 and Aβ42 levels in CAA compared to AD and controls; tau levels were intermediateSupports the role of CSF biomarkers in distinguishing CAA from AD
Charidimou et al. [30] (2022)To validate Boston criteria v2.0 for diagnosing CAA using MRI-neuropathology correlationMulticenter retrospective study; n = 159 in the deviation cohort, n = 59 in the temporal validation cohort, n = 123 in the geographical validation cohortBoston criteria v2.0 improved diagnostic sensitivity (74%) and maintained specificity (95%)Established updated, reliable criteria for in vivo CAA diagnosis
Charidimou et al. [77] (2018)Meta-analysis of CSF biomarkers in CAAPooled data from multiple studies (n = 3 studies: n = 59 CAA cases, n = 94 healthy controls, n = 158 AD cases)Decreased Aβ40 and Aβ42 in CAA; variable tau levelsConfirms CSF profile differs from AD; suggests heterogeneity
Charidimou et al. [41] (2017)Evaluate the detection and clinical implications of cortical superficial siderosis (cSS) in CAAMixed cohort with CAA and related conditionscSS is strongly associated with the risk of future ICH and TFNEscSS is a key imaging marker in the Boston criteria
Kargiotis et al. [49] (2018)Review of cerebral microbleeds (CMBs): causes, imaging, and clinical relevanceLiterature reviewLobar CMBs are prominent in CAA and detectable via SWI/T2* MRIUseful for differentiating CAA from hypertensive arteriopathy
Muir et al. [44] (2024)Compare plasma biomarker quantification methods for AD and CAA applicationAnalytical comparison of biomarker assaysCombining biomarkers (Aβ ratio, p-tau, neuroinflammation, demographic/clinical variables) has the potential to improve the differential diagnosis of AD and CAASeveral methodological issues were identified: suggest using neuropathological gold standard, control for disease stage, essay standardization; sex differences and longitudinal changes
Paniagua Bravo et al. [51] (2014)Compare MRI techniques for WMH detectionHealthy and clinical populations (n = 40 randomly selected patients; n = 10 controls)3D-FLAIR is superior in detecting WMHs compared to other sequencesInforms optimal MRI protocols for identifying WMHs in CAA
Seifert et al. [78] (2025)Overview of diagnosis and management of CAA-related inflammation (CAA-ri)Review articleCAA-ri is an immune-mediated variant with distinct imaging and steroid-responsive featuresHighlights need to differentiate CAA-ri from sporadic CAA
Sin et al. [62] (2025)Comprehensive review of plasma biomarkers for CAA in the context of ARIAReview, including prospective biomarker studiesPlasma GFAP, Aβ42, and t-tau may predict CAA and ARIA riskSuggests risk prediction with the potential plasma biomarkers for CAA in future studies
Smith et al. [39] (2021)Characterize TFNEs in CAA and their clinical outcomesObservational study of CAA patients with TFNEsTFNEs are predictive of subsequent ICH and cSSTFNEs should raise suspicion for underlying CAA
Theodorou et al. [37] (2023)Examine the link between cSS, TFNEs, and lobar hemorrhage in CAACase series of CAA patientsTFNEs and cSS often precede symptomatic lobar hemorrhageReinforces the importance of early MRI detection of cSS in risk stratification

CAA: cerebral amyloid angiopathy; CSF: cerebrospinal fluid; AD: Alzheimer’s disease; Aβ: amyloid β; MRI: magnetic resonance imaging; ICH: intracerebral hemorrhage; TFNEs: transient focal neurological episodes; SWI: susceptibility-weighted imaging; p-tau: phosphorylated tau; WMH: white matter hyperintensity; FLAIR: fluid-attenuated inversion recovery; ARIA: amyloid-related imaging abnormalities; GFAP: glial fibrillary acidic protein; t-tau: total tau.