Valentin A, Karnik R, Anzböck W, Slany J
II. Medizinische Abteilung und Zentralröntgeninstitut der Krankenanstalt Rudolfstiftung, Wien.
Dtsch Med Wochenschr. 1991 Oct 11;116(41):1553-6. doi: 10.1055/s-2008-1063788.
A previously healthy man, aged 33 years, suddenly developed a hemiparesis and right facial paresis, as well as anisocoria and motor aphasia, preceded by recurrent attacks of dizziness. On admission he was somnolent. A flow murmur was heard over both carotid arteries; the blood pressure was 160/80 mm Hg. Cerebral computed tomography demonstrated multiple hypodense areas in the area supplied by several cerebral arteries, and marked cerebral oedema. Angiography of the aortic arch and the supra-aortic branches showed an occlusion of the left common carotid artery and a stenosis of the brachiocephalic trunk. The cerebral oedema, caused by ischaemia, did not respond to treatment. The patient died on the fourth hospital day from brainstem "strangulation". At autopsy syphilitic mesaortitis with characteristic lymphoplasmacellular endangiitis of the vasa vasorum of the aortic arch was demonstrated as the cause of the "aortic arch syndrome". Serology confirmed the diagnosis of an untreated tertiary syphilis. (VDRL titre 1:256; TPHA reactive; IgM-SPHA titre 1:64). Although a very rare cause, a late stage of syphilis should be considered in the differential diagnosis of cerebrovascular lesions in youngish patients.
一名33岁的既往健康男性，在反复出现头晕发作后，突然出现偏瘫、右侧面瘫，以及瞳孔不等大和运动性失语。入院时他嗜睡。双侧颈动脉可闻及血流杂音；血压为160/80 mmHg。脑部计算机断层扫描显示多条脑动脉供血区域有多个低密度区，并有明显的脑水肿。主动脉弓和主动脉上部分支血管造影显示左颈总动脉闭塞，头臂干狭窄。由缺血引起的脑水肿对治疗无反应。患者于住院第4天死于脑干“绞窄”。尸检显示梅毒性中层主动脉炎伴主动脉弓血管滋养管特征性淋巴细胞浆细胞性血管内膜炎，为“主动脉弓综合征”的病因。血清学检查确诊为未经治疗的三期梅毒（性病研究实验室试验滴度1:256；梅毒螺旋体血凝试验呈阳性；IgM-梅毒螺旋体被动血凝试验滴度1:64）。尽管是非常罕见的病因，但在较年轻患者脑血管病变的鉴别诊断中应考虑梅毒晚期。
