Naganuma Y, Konishi T, Hongou K, Murakami M, Yamatani M, Okada T
Department of Pediatrics, Faculty of Medicine, Toyama Medical and Pharmaceutical University.
No To Hattatsu. 1990 Jan;22(1):71-6.
Cerebrovascular involvement is rare in progeria syndrome (Hutchinson-Gilford syndrome). A patient with progeria syndrome, who developed cerebral infarction, was reported. At 7 years of age, she suffered from right hemiplegia and transient ischemic attacks. X-ray CT showed multiple low density lesions in left frontal and parieto-occipital areas, which were enhanced with a contrast medium. Cerebral angiography demonstrated complete occlusion of left carotid artery and narrowing of vertebral artery. Brain imaging using 123I-N-isopropyl-p-iodoamphetamine (123I-IMP SPECT), which expressed regional cerebral blood flow, showed extensive perfusion defect over the left cerebral hemisphere in early scans, and redistribution phenomena in late scans. The symptoms improved gradually, which correlated well with 123I IMP-SPECT findings. This method will be useful to determine the prognosis as well as to understand changeable hemodynamic pathophysiology. A slowing of back ground on EEG also correlated well with clinical symptoms. It was necessary to examine the possibility of cerebrovascular involvement in progeria.
脑血管受累在早老综合征（哈钦森 - 吉尔福德综合征）中较为罕见。本文报道了一名患有早老综合征且发生脑梗死的患者。该患者7岁时出现右侧偏瘫和短暂性脑缺血发作。X线计算机断层扫描（CT）显示左侧额叶和顶枕叶区域有多个低密度病变，增强造影剂后病变强化。脑血管造影显示左侧颈动脉完全闭塞，椎动脉狭窄。使用123I - N - 异丙基 - p - 碘安非他明（123I - IMP单光子发射计算机断层扫描，SPECT）进行脑成像以显示局部脑血流，早期扫描显示左侧大脑半球广泛灌注缺损，晚期扫描出现再分布现象。症状逐渐改善，这与123I IMP - SPECT检查结果密切相关。该方法对于判断预后以及了解多变的血液动力学病理生理学将很有用。脑电图背景活动减慢也与临床症状密切相关。有必要研究早老综合征患者脑血管受累的可能性。
