Downes Margaret H, Kalagara Roshini, Rossitto Christina P, Vasan Vikram, Vasa Devarshi, Chennareddy Susmita, Lefton Daniel R, Yildiz Sema, Umphlett Melissa, Brockington Carolyn, Kellner Christopher P
Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, NY.
Department of Diagnostic, Molecular and Interventional Radiology, Icahn School of Medicine at Mount Sinai, New York, NY.
Neurology. 2025 Apr 8;104(7):e213460. doi: 10.1212/WNL.0000000000213460. Epub 2025 Mar 3.
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.
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.
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, = 0.013) and lobar CMBs (OR 3.03, 95% CI 1.31-7.10, = 0.009) were significantly associated with CAA. Among nonhemorrhagic markers, CSO-PVS was strongly associated (OR 5.49, 95% CI 2.37-13.06, < 0.001) while multispot WMHs were not (OR 1.10, 95% CI 0.45-2.56, = 0.834).
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.
脑淀粉样血管病（CAA）是老年人群叶内脑出血（ICH）的主要原因，与显著的发病率和复发相关。更新后的波士顿标准2.0版（v2.0）纳入了新的MRI生物标志物以提高诊断准确性。本研究旨在验证v2.0与1.5版（v1.5）相比在接受手术清除血肿和脑活检的自发性ICH患者中的诊断性能。
本回顾性单中心队列研究于2015年至2021年在西奈山医疗系统进行。纳入接受手术清除血肿和脑活检以及术前MRI检查的自发性ICH患者。评估的MRI标志物包括叶内出血性病变（ICH、脑微出血[CMB]、皮质铁沉积[cSS]）和非出血性标志物（半卵圆中心严重可见的血管周围间隙[CSO-PVS]和多发白质高信号[WMH]）。CAA的病理确诊基于改良的冯萨特尔分级，该分级评估血管壁中的β淀粉样蛋白沉积。使用敏感性、特异性和预测值比较v2.0与v1.5的诊断性能。逻辑回归模型计算MRI生物标志物与病理确诊的CAA之间关联的比值比（OR）和95%置信区间（CI）。
在186例患者（中位年龄：63岁；38%为女性）中，24%确诊为CAA。波士顿标准v2.0对可能的CAA显示出更高的敏感性（0.75对0.57），同时保持特异性（0.96对0.99）。对于可能的CAA，敏感性适度提高（0.82对0.77），特异性相当（0.84对0.87）。在出血性标志物中，cSS（OR 4.14，95%CI 1.35 - 13.00，P = 0.013）和叶内CMB（OR 3.03，95%CI 1.31 - 7.10，P = 0.009）与CAA显著相关。在非出血性标志物中，CSO-PVS密切相关（OR 5.49，95%CI 2.37 - 13.06，P < 0.001），而多发WMH则不然（OR 1.10，95%CI 0.45 - 2.56，P = 0.834）。
波士顿标准v2.0在不影响特异性的情况下提高了诊断可能CAA的敏感性，这主要归因于纳入了CSO-PVS等非出血性标志物。局限性包括回顾性设计、缺乏正式的评分者间可靠性测量以及样本量适度。这些发现强调了v2.0在改善CAA诊断框架方面的潜力。
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