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[一例先天性蛋白C缺乏所致多发性脑动脉血栓形成]

[A case of multiple cerebral arterial thrombosis due to congenital protein C deficiency].

作者信息

Ohtani R, Kazui S, Naritomi H, Kinugawa H, Sawada T

出版信息

Rinsho Shinkeigaku. 1997 Aug;37(8):717-20.

PMID:9404153
Abstract

We report a 49-year-old man who had right hemiparesis and motor aphasia. A computed tomography revealed hypodense areas in the left frontal subcortex. A cerebral angiography demonstrated occlusion of the left distal internal carotid artery and both anterior cerebral arteries, as well as stenosis of the left internal carotid artery at the cervical portion. The second angiogram obtained a month later showed no changes. The diagnosis of atherothrombotic cerebral infarction was established on the basis of clinical profile and angiographic findings. Protein C activity and antigen levels were reduced to approximately one half of the normal level in the patient and his brother. The patient had no other risk factors for stroke. Protein C deficiency has been considered one of the risk factors for thrombotic diseases. Venous thrombosis is the most common clinical manifestation, whereas arterial thrombosis is relatively rare. It is generally believed that arterial ischemic stroke associated with protein C deficiency occurs with embolic mechanism, and atherothrombotic infarction is extremely rare. This is the first report suggesting the possibility that protein C deficiency can cause cerebral thrombosis.

摘要

我们报告了一名49岁男性,他患有右侧偏瘫和运动性失语。计算机断层扫描显示左侧额叶皮质下有低密度区。脑血管造影显示左侧颈内动脉远端及双侧大脑前动脉闭塞,以及左侧颈内动脉颈部狭窄。一个月后进行的第二次血管造影显示无变化。根据临床特征和血管造影结果确诊为动脉粥样硬化性脑梗死。患者及其兄弟的蛋白C活性和抗原水平降至正常水平的约一半。该患者没有其他中风危险因素。蛋白C缺乏被认为是血栓形成性疾病的危险因素之一。静脉血栓形成是最常见的临床表现,而动脉血栓形成相对少见。一般认为,与蛋白C缺乏相关的动脉缺血性中风是由栓塞机制引起的,动脉粥样硬化性梗死极为罕见。这是第一份提示蛋白C缺乏可能导致脑血栓形成的报告。

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