10例淀粉样脑血管病相关脑叶出血的临床病理分析

Clinical and pathological study on 10 cases of cerebral lobe hemorrhage related with cerebral amyloid angiopathy

目的总结10例尸检证实的淀粉样脑血管病相关脑叶出血病例的临床资料与病理特点,提高对本病的认识与诊断水平。方法回顾性分析沈阳军区总医院从1983年7月-2005年7月收治的10例淀粉样脑血管病相关脑叶出血患者,总结其临床资料及病理学特点。结果 10例患者中单发脑叶出血2例,多灶性脑叶出血8例,均破入蛛网膜下腔。病理检查2例见微小动脉瘤,4例见"双桶"样改变,5例见多发微小动脉丛,4例见闭塞性洋葱皮样内膜改变,7例见血管壁类纤维素坏死。8例见不同程度的神经原纤维缠结,5例见老年斑。结论淀粉样脑血管病相关脑叶出血多分布在顶叶、颞叶、枕叶,常为多发性、复发性脑叶出血,易破入蛛网膜下腔。淀粉样脑血管病相关微血管病变可能是脑叶出血的重要病理基础。

Objective To summarize the clinical data and pathological features of 10 cases of cerebral lobar hemorrhage related with cerebral amyloid angiopathy（CAA） diagnosed pathologically, thereby to improve the knowledge and diagnosis of the disease. Methods The clinical data of 10 cases of cerebral lobar hemorrhage related with CAA, collected in the General Hospital of Shenyang Command from 1983 up to now, were retrospectively analyzed, and the clinical and neuropathological features of these cases were summarized. Results Of the 10 patients, 2 suffered from single lobar hemorrhage and 8 multiple lobar hemorrhage, all of them were confirmed pathologically to have ruptured into the subarachnoid space. Pathological examination revealed microaneurysm in 2 cases, ＂double barrel＂ change in 4 cases, multiple arteriolar clusters in 5 cases, obliterative onion-liked intima change in 4 cases, and fibrinoid necrosis of vessel wall in 7 cases. In addition, neurofibrillary tangles were found in 8 cases, and senile plaque was observed in 5 cases. Conclusions Cerebral lobar hemorrhage related with CAA is mainly located in the parietal, temporal and occipital lobes, readily breaking into the subarachnoid space, and it is often multiple and recurrent. The CAA associated microvasculopathy was found frequently in the autopsy sample of CAA related cerebral lobar hemorrhage, and it may contribute to the pathogenesis of cerebral hemorrhage.