Data Management And Sharing System of China National Clinical Research Center for Neurological Diseases
CADASIL: two new cases with intracerebral hemorrhage.
First author：chen zhang,wei li
Corresponding author：zaiqiang zhang
essential_information
- Person in charge：chen zhang,wei li
- Data Sources： OTHERS
- Number of papers：OTHERS20190117
- Published time：2017-03-07
2017-03-07
Published articles
Journal：Ann Clin Transl Neurol
Publication type: Brief Reports
IF(IF): 4.64
PMID: 28382307 Link To Pubmed Full text Download
Background And Purpose
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.
Method
MRI Blood measure using an ambulatory blood pressure monitor
Conclusion
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.
Main results
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.