Pavlakis S G, Bello J, Prohovnik I, Sutton M, Ince C, Mohr J P, Piomelli S, Hilal S, De Vivo D C
Department of Neurology, College of Physicians and Surgeons of Columbia University, New York, NY.
Ann Neurol. 1988 Feb;23(2):125-30. doi: 10.1002/ana.410230204.
Brain infarction is a well-known but poorly understood complication of sickle cell disease. Seventy-three sickle cell disease patients underwent neurological examinations and high-field, heavily T2-weighted axial cranial magnetic resonance image (MRI) scanning. Eighteen of the 73 had a history of stroke, defined as an acute, focal neurological sign lasting longer than 1 hour; in the event of a convulsive onset, an MRI abnormality as a correlate was necessary. Sixteen of the 18 stroke patients demonstrated focal MRI abnormalities consistent with arterial borderzone infarctions. Fifty-five of the 73 patients had no history of stroke. Six of the 55 (11%) had focal MRI abnormalities suggesting previous subclinical stroke. Five of these lesions were in borderzone regions. The distinguishing feature in 21 of the 22 patients with MRI abnormalities was the predilection for lesions in the high cortical convexity, in the general regions of arterial borderzones between the major cerebral arteries and adjacent deep white matter. The pattern of MRI lesions suggests two pathogenetic mechanisms: (1) proximal large-vessel disease with inadequate cerebral perfusion (distal field insufficiency syndrome) and (2) distal small-vessel disease (sludging syndrome).
脑梗死是镰状细胞病一种广为人知但了解甚少的并发症。73例镰状细胞病患者接受了神经学检查及高场强、重T2加权头颅轴位磁共振成像（MRI）扫描。73例患者中有18例有卒中病史，卒中定义为急性局灶性神经体征持续超过1小时；若起病时有惊厥发作，则需要MRI异常作为相关依据。18例卒中患者中有16例MRI显示局灶性异常，符合动脉边缘带梗死。73例患者中有55例无卒中病史。55例中的6例（11%）有局灶性MRI异常，提示既往有亚临床卒中。其中5处病变位于边缘带区域。22例有MRI异常的患者中，21例的显著特征是病变好发于大脑皮质高凸面，即主要脑动脉与相邻深部白质之间动脉边缘带的大致区域。MRI病变模式提示两种发病机制：（1）近端大血管病变伴脑灌注不足（远隔区域供血不足综合征）和（2）远端小血管病变（血液淤滞综合征）。
