From the J. Philip Kistler Stroke Research Center (M.C.Z.Z., N.M., A.C., S.M.G., S.J.V.), Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston; Center for Imaging Sciences and Medical Physics (M.C.Z.Z.), Department of Medical Imaging, Hematology and Clinical Oncology, Ribeirão Preto Medical School, University of São Paulo, Brazil; Rush Alzheimer's Disease Center (J.A.S., K.A.), Rush University Medical Center; and Department of Biomedical Engineering (K.A.), Illinois Institute of Technology, Chicago.
Neurology. 2024 Jan 9;102(1):e207940. doi: 10.1212/WNL.0000000000207940. Epub 2023 Dec 13.
The Boston criteria are a set of clinical and neuroimaging features that enable accurate diagnosis of cerebral amyloid angiopathy (CAA) without invasive methods such as brain biopsies or autopsy. The last updates to the Boston criteria, named version 2.0, were recently released and incorporated new nonhemorrhagic MRI features. These criteria have been validated in symptomatic samples, with improved diagnostic yield. We set out to investigate the accuracy of the Boston criteria v2.0 for the diagnosis of CAA in a community-based sample.
Participants were recruited from longitudinal clinical-pathologic studies of aging conducted at the Rush Alzheimer's Disease Center in Chicago: the Religious Orders Study and the Rush Memory and Aging Project. Deceased participants with in vivo 3T MRI and detailed pathologic data available were included in the analysis. We compared the diagnostic yield of the current and earlier versions of the Boston criteria in our sample. Among those classified as probable CAA according to the Boston criteria v2.0, we investigated the ability of each neuroimaging marker to distinguish between false-positive and true-positive cases.
In total, 134 individuals were included in the study (mean age = 82.4 ± 6.0 years; 69.4% F), and 49 of them were considered pathology-proven definite cases with CAA (mean age = 82.9 ± 6.0 years; 63.3% F). The Boston criteria versions 1.0 and 1.5 yielded similar sensitivity (26.5%, both), specificity (90.6% and 89.4%, respectively), and predictive values (negative: 68.1% and 67.9%; positive: 61.9% and 59.1%, respectively). The recently released Boston criteria v2.0 offered higher sensitivity (38.8%) and slightly lower specificity (83.5%). Among those classified as probable CAA (v2.0), pathology-proven true-positive cases had higher numbers of strictly cortical lobar microbleeds compared with false-positive cases ( = 0.004).
Similar to findings from symptomatic samples, the inclusion of nonhemorrhagic neuroimaging markers in the updated Boston criteria offered a 12.3% gain in sensitivity among community-dwelling individuals, at the expense of a 5.9% drop in specificity. In cases with probable CAA, the cortical location of microbleeds may represent a promising distinguishing feature between true-positive and false-positive cases. Despite its improved performance, the diagnostic sensitivity of the updated criteria in a community-based sample remains limited.
This study provides Class II evidence that the Boston criteria v2.0 accurately distinguishes people with CAA from those without CAA.
波士顿标准是一组临床和神经影像学特征，可在不进行脑活检或尸检等有创方法的情况下准确诊断脑淀粉样血管病（CAA）。最近发布了波士顿标准的最新版本 2.0，纳入了新的非出血性 MRI 特征。这些标准已在有症状的样本中得到验证，提高了诊断效果。我们旨在调查该标准在社区样本中诊断 CAA 的准确性。
参与者从芝加哥拉什阿尔茨海默病中心进行的老龄化纵向临床病理研究中招募：宗教秩序研究和拉什记忆与衰老项目。将有体内 3T MRI 和详细病理数据的已故参与者纳入分析。我们比较了当前和早期版本的波士顿标准在我们样本中的诊断效果。在根据波士顿标准 2.0 被归类为可能的 CAA 的人群中，我们研究了每个神经影像学标志物在区分假阳性和真阳性病例方面的能力。
共有 134 人纳入研究（平均年龄=82.4±6.0 岁；69.4%女性），其中 49 人被认为是具有 CAA 的病理学确诊明确病例（平均年龄=82.9±6.0 岁；63.3%女性）。波士顿标准 1.0 版和 1.5 版的敏感性（分别为 26.5%）、特异性（90.6%和 89.4%）和预测值（阴性：68.1%和 67.9%；阳性：61.9%和 59.1%）相似。最近发布的波士顿标准 2.0 提供了更高的敏感性（38.8%）和略低的特异性（83.5%）。在被归类为可能的 CAA（v2.0）的人群中，与假阳性病例相比，经病理学证实的真阳性病例有更多严格的皮质叶微出血（ = 0.004）。
与有症状样本的发现类似，在更新的波士顿标准中纳入非出血性神经影像学标志物可使社区居民的敏感性提高 12.3%，特异性降低 5.9%。在可能的 CAA 病例中，微出血的皮质位置可能是真阳性和假阳性病例之间的一个有希望的鉴别特征。尽管该标准的性能有所提高，但在社区样本中的诊断敏感性仍然有限。
本研究提供了 II 级证据，表明波士顿标准 2.0 可准确区分 CAA 患者和非 CAA 患者。
