Department of Neurosurgery, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan.
Department of Pathology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan.
Neuropathology. 2022 Jun;42(3):226-231. doi: 10.1111/neup.12801. Epub 2022 Mar 9.
We report an autopsy case of repeated recurrent intracerebral hemorrhage (ICH) diagnosed as having cerebral amyloid angiopathy-related inflammation (CAA-ri) in a 65-year-old woman. She had no history of hypertension or other risk factors for stroke. She had a history of lobar ICH in the right parietal lobe 30 months prior. Follow-up magnetic resonance imaging (MRI) performed 29 months after the initial ICH revealed superficial siderosis in the left frontal lobe. She initially presented with severe headache and dysarthria. An initial computed tomography (CT) revealed a subarachnoid hemorrhage on the left frontal lobe. One hour later, an epileptic seizure occurred, and another CT performed at the time revealed lobar ICH in the frontal lobe. Emergency surgical hematoma evacuation was performed. The cortical artery was removed from the surgical specimen. Hematoxylin and eosin (HE) staining revealed lymphocytic perivascular cuffs, and immunohistochemical staining revealed abundant amyloid-β (Aβ) deposits. The patient was diagnosed as having CAA-ri. On the next day, and 19, 46, 55, 78, 79, and 85 days after admission, ICH recurred. We did not administer immunosuppressive drugs, such as high-dose corticosteroids or cyclophosphamide, because of the patient's condition. Following the last ICH, the patient died on the 90th day after symptom onset. An autopsy was performed. Histologically, abundant Aβ deposits were observed within the leptomeningeal and cortical arteries. Further dense-cored amyloid plaques were observed in the cortical samples. Our findings that ICH occurred initially three times in the left hemispheres and later five times in the right hemispheres were considered immune-mediated effects on vascular function. Further, superficial hemosiderosis in the left frontal lobe occurred before the first three ICH events, and hyperintensity of the white matter on MRI was identified in the right parietal lobe, a typical finding of CAA-ri. We hypothesize that the inflammatory process in CAA-ri may be related to recurrent ICH.
我们报告了一例 65 岁女性反复发生的脑内出血（ICH）的尸检病例，该患者被诊断为伴有脑淀粉样血管病相关炎症（CAA-ri）。她没有高血压或其他中风危险因素的病史。30 个月前，她的右侧顶叶发生过一次脑叶 ICH。初次 ICH 后 29 个月的随访磁共振成像（MRI）显示左侧额叶有浅表铁沉积。她最初表现为严重头痛和构音障碍。初始计算机断层扫描（CT）显示左侧额叶蛛网膜下腔出血。1 小时后，发生癫痫发作，当时进行的另一项 CT 显示额叶脑叶 ICH。紧急手术血肿清除术。从手术标本中取出皮质动脉。苏木精和伊红（HE）染色显示淋巴细胞血管周围套袖，免疫组织化学染色显示大量淀粉样β（Aβ）沉积。患者被诊断为 CAA-ri。入院后第 2、19、46、55、78、79 和 85 天，ICH 再次发生。由于患者的病情，我们没有给予免疫抑制药物，如大剂量皮质类固醇或环磷酰胺。在最后一次 ICH 后，患者在症状发作后第 90 天死亡。进行了尸检。组织学上，在软脑膜和皮质动脉内观察到大量 Aβ 沉积。在皮质样本中还观察到更密集的核心淀粉样斑块。我们发现 ICH 最初三次发生在左侧半球，后来五次发生在右侧半球，这被认为是对血管功能的免疫介导效应。此外，在第一次三次 ICH 事件之前，左侧额叶出现了浅表铁沉积，MRI 显示右侧顶叶的白质出现了高信号，这是 CAA-ri 的典型表现。我们假设 CAA-ri 中的炎症过程可能与复发性 ICH 有关。
