Panicker Jalesh N, Nagaraja D, Chickabasaviah Yasha T
Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore - 560 029, India.
Ann Indian Acad Neurol. 2010 Jul;13(3):216-20. doi: 10.4103/0972-2327.70879.
Cerebral amyloid angiopathy (CAA) is an important cause for intracerebral hemorrhage (ICH), yet often goes undiagnosed in the absence of histological examination of the blood vessels in the clot. In this study, we report three patients who presented with ICH. Two patients had no risk factors for bleed, whereas one patient had systemic hypertension. Tissue for analysis was obtained during hematoma evacuation in two patients and necropsy in the third. Histopathology in all three patients revealed severe degree of amyloid angiopathy with extensive amyloid deposits in the vessel walls, which was diagnostic of CAA. Both medium- and small-sized leptomeningeal and cortical vessels were affected. The vascular amyloid deposits stained with Congo red and displayed characteristic birefringence under polarizing light. In addition, vessels also showed fibrinoid necrosis and vascular endothelial proliferation. Immunohistochemistry demonstrated beta-amyloid peptide in all three cases-the protein most commonly involved in sporadic CAA. Senile plaques with amyloid cores were present in all areas, whereas neurofibrillary tangles were restricted to the medial temporal region in the autopsied case. CAA is an important cause for intracerebral bleed and may be a contributory factor even when other risk factors for ICH are present. Areas of hemorrhage tend to correlate with severity of CAA changes.
脑淀粉样血管病（CAA）是脑出血（ICH）的一个重要病因，但在没有对血凝块中的血管进行组织学检查的情况下，该病常常无法得到诊断。在本研究中，我们报告了3例脑出血患者。2例患者无出血危险因素，而1例患者患有系统性高血压。2例患者在血肿清除过程中获取了用于分析的组织，第3例患者进行了尸检。所有3例患者的组织病理学检查均显示严重程度的淀粉样血管病，血管壁有广泛的淀粉样沉积物，这可诊断为CAA。软脑膜和皮质的中、小血管均受累。血管淀粉样沉积物经刚果红染色，在偏振光下呈现特征性双折射。此外，血管还显示纤维蛋白样坏死和血管内皮增生。免疫组织化学在所有3例病例中均显示β-淀粉样肽——散发性CAA中最常涉及的蛋白质。所有区域均存在有淀粉样核心的老年斑，而在尸检病例中神经原纤维缠结局限于内侧颞叶区域。CAA是脑出血的一个重要病因，即使存在其他脑出血危险因素，它也可能是一个促成因素。出血区域往往与CAA变化的严重程度相关。
