脑淀粉样血管病_赵元立.ppt

Mortality and Morbidity CAA ICH associated with lower mortality rate (11-32%) and better functional outcome 25-40% have a recurrence, with the highest risk in the first year, associated with a high mortality rate (up to 40%) Cognitive impairment is common 建立规范化的微创外科诊断治疗标准 （新增样本2000例） 规范试验标准 多中心大样本研究 小骨窗手术 大骨瓣减压手术 其它微创手段 病理学检查 高血压动脉硬化性 淀粉样血管病 疗效分析 自然史研究 新增2000例 既往2764例 筛选疾病相关危险因素 建立预警体系 卫生经济学研究 建立关于成本/效益的数学模型 社区干预 淀粉样变脑血管病研究-技术路线 数据库网络平台 现有病例分析 67例确诊为自发性脑出血 开颅手术获取出血灶周围病理标本 刚果红染色和Aβ免疫组化染色 结果 病理学证实8例为淀粉样血管病 占11.9％ 男:女＝48:19 (CAA男:女＝6:2) 40-49岁组15.8% (3/19) 50-59岁组13.0% (3/23) 60-69岁组占9.1%(1/11) 70岁以上组11.1% (1/9) 典型病例 典型复发性、多发脑出血 女性，73岁 主因“突发意识丧失1小时”于2008年4月22日急诊入院 3年前曾因“突发头痛、头晕、呕吐1天”急诊收入我院神经内科 否认高血压病、糖尿病、高血脂、冠心病等病史 无服用抗凝药物史 此次入院时深昏迷，去脑强直状态，四肢肌张力增高，病理征（＋） 急诊行额颞顶大骨瓣开颅血肿清除术 病理诊断为淀粉样脑血管病 CT Scan (1)1/10/2005 8mL right occipital lobe CT Scan (2)5/20/2005 CT Scan (3)4/22/2008 100ml, recurrent bilateral multiple hemorrhage CT Scan (4)4/23/2008 P.O CT Scan (5)5/19/2008 Thank you ! * (A) Only a fine rim of salmon-like staining is found in the vessel wall. There is no change in the vessel architecture or thickening of the wall. Score 1 also note Alzheimer plaques in the vicinity of the vessels (arrows) (Congo red, ×100). (B) The entire wall of the vessel is dominated by Congo-positive material. There are no vessel fractures or fibrinoid necrosis or paravascular blood. Score 2 (Congo red,×100). (C) The vessel architecture (arrow) is destroyed. The wall is Congo positive. Fibrinoid necrosis can be seen (*). Score 3 (Congo red ×100; small section in upper left corner, hematoxylin-eosin.×100). Cerebral Amyloid Angiopathy脑淀粉样血管病 赵元立 北京天坛医院 What is CAA? amyloid deposition aged (=50-60y) arteries of the cortical, subcortical areas M F in incidence Recurrent, Multiple Hemorrhage Prada et al., J. Neurosci., 2007 Background Cerebral amyloid angiopathy (CAA) - deposition of β-amyloid in the media and adventitia of small- and mid-sized arteries ICH