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Validation of the Boston Criteria Version 2.0 for Cerebral Amyloid Angiopathy in Patients Presenting With
Margaret H Downes1, Roshini Kalagara1, Christina P Rossitto1
1Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, NY.
Insights
The updated Boston criteria v2.0 improve diagnosis of cerebral amyloid angiopathy (CAA) by increasing sensitivity for probable CAA. This version effectively uses MRI biomarkers, including nonhemorrhagic markers like perivascular spaces, to enhance diagnostic accuracy.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Cerebral amyloid angiopathy (CAA) is a primary cause of lobar intracerebral hemorrhage (ICH) in older adults, leading to significant disability and recurrent events.
- The Boston criteria, particularly version 2.0 (v2.0), have been updated with novel MRI biomarkers to enhance the diagnostic accuracy of CAA.
Purpose of the Study:
- To validate the diagnostic performance of the Boston criteria v2.0 against version 1.5 (v1.5).
- To compare the accuracy of v2.0 in diagnosing CAA in patients with spontaneous ICH who underwent surgical evacuation and brain biopsy.
Main Methods:
- A retrospective single-center cohort study included 186 patients with spontaneous ICH who underwent surgical evacuation, brain biopsy, and preoperative MRI.
- MRI markers assessed included hemorrhagic lesions (ICH, CMBs, cSS) and nonhemorrhagic markers (CSO-PVS, WMHs).
- Pathologic confirmation of CAA was based on modified Vonsattel grading; diagnostic performance was evaluated using sensitivity, specificity, and predictive values.
Main Results:
- Boston criteria v2.0 showed higher sensitivity for probable CAA (75% vs. 57%) with maintained specificity (96% vs. 99%) compared to v1.5.
- Cortical siderosis (cSS) and lobar cerebral microbleeds (CMBs) were significantly associated with CAA.
- Severe visible perivascular spaces in the centrum semiovale (CSO-PVS) were strongly associated with CAA, while multispot white matter hyperintensities (WMHs) were not.
Conclusions:
- The Boston criteria v2.0 improve diagnostic sensitivity for probable CAA without sacrificing specificity, attributed to the inclusion of nonhemorrhagic markers like CSO-PVS.
- Findings suggest v2.0 offers a more robust diagnostic framework for CAA.
- Limitations include retrospective design and modest sample size.
Background And Objectives:
Cerebral amyloid angiopathy (CAA) is a leading cause of lobar intracerebral hemorrhage (ICH) in older individuals, associated with significant morbidity and recurrence. The updated Boston criteria version 2.0 (v2.0) incorporate new MRI biomarkers to improve diagnostic accuracy. This study aimed to validate the diagnostic performance of v2.0 compared with version 1.5 (v1.5) in patients with spontaneous ICH undergoing surgical evacuation and brain biopsy.
Methods:
This retrospective single-center cohort study was conducted at the Mount Sinai Health System from 2015 to 2021. Patients with spontaneous ICH who underwent surgical evacuation with brain biopsy and preoperative MRI were included. MRI markers assessed included lobar hemorrhagic lesions (ICH, cerebral microbleeds [CMBs], cortical siderosis [cSS]) and nonhemorrhagic markers (severe visible perivascular spaces in the centrum semiovale [CSO-PVS] and multispot white matter hyperintensities [WMHs]). Pathologic confirmation of CAA was based on modified Vonsattel grading, which evaluates β-amyloid deposition in vessel walls. Diagnostic performance of v2.0 was compared with v1.5 using sensitivity, specificity, and predictive values. Logistic regression models calculated odds ratios (ORs) and 95% CIs for associations between MRI biomarkers and pathologically confirmed CAA.
Results:
Among 186 patients (median age: 63 years; 38% female), 24% had confirmed CAA. The Boston criteria v2.0 demonstrated higher sensitivity for probable CAA (0.75 vs 0.57) while maintaining specificity (0.96 vs 0.99). For possible CAA, sensitivity improved modestly (0.82 vs 0.77) with comparable specificity (0.84 vs 0.87). Among hemorrhagic markers, cSS (OR 4.14, 95% CI 1.35-13.00, p = 0.013) and lobar CMBs (OR 3.03, 95% CI 1.31-7.10, p = 0.009) were significantly associated with CAA. Among nonhemorrhagic markers, CSO-PVS was strongly associated (OR 5.49, 95% CI 2.37-13.06, p < 0.001) while multispot WMHs were not (OR 1.10, 95% CI 0.45-2.56, p = 0.834).
Discussion:
The Boston criteria v2.0 enhance sensitivity for diagnosing probable CAA without compromising specificity, largely due to the inclusion of nonhemorrhagic markers such as CSO-PVS. Limitations include the retrospective design, the absence of formal inter-rater reliability measures, and the modest sample size. These findings underscore the potential of v2.0 to improve the diagnostic framework for CAA.
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