Babarczy Kristof, Radics Bence L, Horvath Orsolya, Klivenyi Peter, Szalardy Levente
Department of Neurology, Albert Szent-Györgyi Medical School, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged, Hungary.
Institute of Pathology, Albert Szent-Györgyi Medical School, Albert Szent-Györgyi Clinical Center, University of Szeged, Szeged, Hungary.
Neuropathology. 2025 Aug;45(4):e70013. doi: 10.1111/neup.70013. Epub 2025 Jun 5.
Cerebral amyloid angiopathy (CAA) has been implicated as a risk for developing lobar intracerebral hemorrhage (ICH) after intravenous thrombolysis (IVT) applied for acute ischemic stroke (AIS). However, there is a paucity of cases reported with histopathological CAA diagnosis in this setting, with a single report to imply the role of CAA-related inflammation (CAA-RI). We report clinical, radiological, and neuropathological observations of a 65-year-old woman who presented with acute left-hemispheric symptoms with an initially unrevealing cranial computed tomography (CT) and received IVT for presumed AIS. The course was rapidly complicated by a huge lobar ICH and a fatal outcome. The autopsy revealed severe CAA, unexpectedly with transmural CAA-RI, a.k.a. amyloid-β-related angiitis (ABRA), and histopathological evidence for vascular amyloid-β phagocytosis. Re-evaluation of initial imaging did not reveal signs of asymmetric confluent white matter edema characteristic of CAA-RI, but raised the suspicion of a tiny left central convexity subarachnoid hemorrhage, a substrate of amyloid spells. The genotype of the apolipoprotein E (ApoE) gene (ApoE) was ε3/ε3. Being the second published thrombolysis-associated fatality with ABRA and among the few with definite CAA, the present case confirms CAA/CAA-RI to be a potential hidden risk for IVT-associated ICHs, urging for awareness of CAA-associated pathologies and clinical-radiological hints in an AIS setting. The findings implicate the relevance of vascular Aβ phagocytosis in the pathogenesis, confirm that CAA-RI may present without prominent edema, highlight that CAA/CAA-RI-related focal neurological deficits (including amyloid spells) can be potential AIS mimics within the IVT time window, and urge for rigorous analysis of pre-IVT CT scans for even subtle sulcal hyperdensities suggesting cSAH/amyloid spell in elderly patients, prompting consideration of magnetic resonance imaging.
脑淀粉样血管病（CAA）被认为是急性缺血性卒中（AIS）静脉溶栓（IVT）后发生脑叶脑出血（ICH）的一个风险因素。然而，在这种情况下，经组织病理学诊断为CAA的病例报告很少，仅有一份报告暗示了CAA相关炎症（CAA-RI）的作用。我们报告了一名65岁女性的临床、影像学和神经病理学观察结果，该患者出现急性左半球症状，最初头颅计算机断层扫描（CT）未发现异常，因疑似AIS接受了IVT治疗。病情迅速因巨大的脑叶ICH而复杂化，并导致致命后果。尸检显示严重的CAA，意外地伴有透壁CAA-RI，即淀粉样β相关血管炎（ABRA），以及血管淀粉样β吞噬的组织病理学证据。对初始影像学的重新评估未发现CAA-RI特征性的不对称融合性白质水肿迹象，但引发了对微小的左侧中央凸面蛛网膜下腔出血的怀疑，这是淀粉样发作的一个基础。载脂蛋白E（ApoE）基因的基因型为ε3/ε3。作为第二例发表的与ABRA相关的溶栓致死病例，也是少数几例确诊CAA的病例之一，本病例证实CAA/CAA-RI是IVT相关ICH的一个潜在隐藏风险，促使人们在AIS情况下意识到CAA相关病变以及临床影像学提示。这些发现表明血管Aβ吞噬在发病机制中的相关性，证实CAA-RI可能在没有明显水肿的情况下出现，强调CAA/CAA-RI相关的局灶性神经功能缺损（包括淀粉样发作）可能是IVT时间窗内潜在的AIS模仿者，并促使对老年患者IVT前CT扫描进行严格分析，以寻找提示cSAH/淀粉样发作的即使是细微的脑沟高密度影，从而促使考虑进行磁共振成像检查。
