Merkel K H, Ginsberg P L, Parker J C, Post M J
Stroke. 1978 Jan-Feb;9(1):45-52. doi: 10.1161/01.str.9.1.45.
An opportunity to study cerebrovascular changes in sickle cell anemia (SCA) presented itself when a black child with this disorder died of bihemispheric strokes. Angiography demonstrated severe occlusive vascular disease involving primarily the circle of Willis and major bifurcations of both internal carotid arteries. Collateral circulation to the distal branches of the internal carotid arteries occurred through transdural anastomoses from the external carotid system and via the leptomeningeal route. Perfusion of the basal ganglia was accomplished by vessels arising from the proximal internal carotid arteries. These changes resembled those of Moyamoya disease. Autopsy showed old and recent cerebral infarcts. Two vascular processes were responsible for the arterial occlusions: (1) exuberant intimal hyperplasia, and (2) old and recent thrombi with partial recanalization. The former has been described only once before in SCA. Small vessels in the basal ganglia were exceptionally numerous and dilated. We conclude that intimal hyperplasia within large cerebral arteries may be responsible for infarction and small vessel prliferation in basal ganglia in patients with SCA.
一名患有镰状细胞贫血（SCA）的黑人儿童死于双侧半球中风，这为研究该病的脑血管变化提供了契机。血管造影显示，严重的闭塞性血管疾病主要累及 Willis 环和双侧颈内动脉的主要分支。颈内动脉远端分支的侧支循环通过来自颈外动脉系统的经硬膜吻合和软脑膜途径形成。基底节的灌注由颈内动脉近端发出的血管完成。这些变化类似于烟雾病。尸检显示有陈旧性和近期脑梗死。有两个血管过程导致了动脉闭塞：（1）内膜增生过度，（2）陈旧性和近期血栓伴部分再通。前者在 SCA 中此前仅被描述过一次。基底节的小血管异常丰富且扩张。我们得出结论，大脑大动脉内的内膜增生可能是 SCA 患者基底节梗死和小血管增殖的原因。
