国家神经系统疾病临床医学研究中心数据管理与共享系统

Data Management And Sharing System of China National Clinical Research Center for Neurological Diseases

《国家中心数据管理与共享系统操作说明书 —— 研究者部分》

CADASIL: two new cases with intracerebral hemorrhage.

第一作者：chen zhang,wei li

通讯作者：zaiqiang zhang

CADASIL ICH perihematomal edema

基本信息

2017-03-07

已发表文章

发表杂志：Ann Clin Transl Neurol

发表类型: Brief Reports

影响因子(IF): 4.64

PMID: 28382307 Pubmed原文链接 全文下载

主要研究背景及目的

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited cerebral small-vessel disease (SVD) caused by a mutation in the Notch3 gene that leads to the accumulation of granular osmiophilic material (GOM) in arteries and the disintegration of vascular walls. Neuroimaging abnormalities such as cerebral white matter lesions (WMLs), lacunes, and microbleeds in the CADASIL patients are related to this cerebral small-vessel histopathology.1 While CADASIL is considered a primarily ischemic form of vascular dementia, spontaneous intracerebral hemorrhage (ICH) has recently been reported in association with CADASIL,2-6 suggesting that the structural fragility of arterial walls may lead to ICH attacks. However, there are limited data describing the effects that the underlying structural changes related to CADASIL have on the occurrence of ICH and subsequent outcomes. In this study, we report on two unrelated CADASIL patients presenting with recurrent cerebral lobar hemorrhage and thalamic hemorrhage to determine the relation-ship between imaging manifestations of CADASIL and the occurrence and dynamic evolution of ICH. We also briefly review the literature on this issue.

方法

MRI Blood measure using an ambulatory blood pressure monitor

结论

This report demonstrates temporal characteristics of ICH in association with CADASIL, which suggests that ICH in patients with CADASIL is significantly different than primary cerebral hemorrhage, and specific management strategies for ICH patients with CADASIL are recommended.

主要结果

In this study, we report two cases of CADASIL presenting with cerebral hemorrhages. These cases suggest that a CADASIL vasculopathy by itself mainly results in ICH, as indicated by slight vascular risk factors and prominent neuroimaging abnormalities, suggesting that CADASIL should be considered a risk factor for ICH. Interestingly, decreased perihematomal edema was noted in ICH patients with CADASIL in this study.