Feldmann E, Tornabene J
Department of Clinical Neurosciences, Brown University, Providence, Rhode Island.
Clin Geriatr Med. 1991 Aug;7(3):617-30.
CAA is the infiltration of leptomeningeal and penetrating cortical vessels with amyloid, sparing the subcortical regions and the systemic vasculature. It occurs with increasing frequency after the sixth decade. The major clinical manifestation of CAA is lobar intracerebral hemorrhage, which can be sporadic or hereditary. CAA has also been associated with normal aging, Alzheimer's disease, cerebral infarction, and periventricular demyelination. Biochemical studies have shown that the amyloid deposits in the brains of patients with normal aging, sporadic CAA-associated hemorrhage, hereditary cerebral hemorrhage, and Alzheimer's disease are identical. The exact mechanism by which CAA produces lobar hemorrhages and the role of CAA in the development of dementia are unclear. Biopsy of the involved cerebral cortex and leptomeninges is the only definitive way to diagnose CAA. Acute management of CAA-associated lobar hemorrhage consists of aggressive control of associated hypertension and supportive care. Surgical removal of the hemorrhage has not been shown to improve survival. Antiplatelet and anticoagulant therapy should be avoided in elderly patients with known CAA.
脑淀粉样血管病(CAA)是软脑膜和穿透皮质的血管被淀粉样物质浸润,不累及皮质下区域和全身血管系统。其发病率在60岁以后逐渐增加。CAA的主要临床表现为脑叶脑出血,可为散发性或遗传性。CAA还与正常衰老、阿尔茨海默病、脑梗死和脑室周围脱髓鞘有关。生化研究表明,正常衰老、散发性CAA相关性出血、遗传性脑出血和阿尔茨海默病患者大脑中的淀粉样沉积物是相同的。CAA导致脑叶出血的确切机制以及CAA在痴呆症发展中的作用尚不清楚。对受累大脑皮质和软脑膜进行活检是诊断CAA的唯一确切方法。CAA相关性脑叶出血的急性处理包括积极控制相关高血压和支持治疗。手术清除出血尚未显示能提高生存率。已知患有CAA的老年患者应避免使用抗血小板和抗凝治疗。