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首页> 外文期刊>Journal of Cerebral Blood Flow and Metabolism: Official Journal of the International Society of Cerebral Blood Flow and Metabolism >Vascular permeability in cerebral cavernous malformations
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Vascular permeability in cerebral cavernous malformations

机译：脑海绵状畸形的血管通透性

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摘要

Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case-controlled observational study investigated whether the brains of human subjects with familial CCM show vascular hyperpermeability by dynamic contrast-enhanced quantitative perfusion magnetic resonance imaging, in comparison with CCM cases without familial disease, and whether lesional or brain vascular permeability correlates with CCM disease activity. Permeability in white matter far (WMF) from lesions was significantly greater in familial than in sporadic cases, but was similar in CCM lesions. Permeability in WMF increased with age in sporadic patients, but not in familial cases. Patients with more aggressive familial CCM disease had greater WMF permeability compared to those with milder disease phenotype, but similar lesion permeability. Subjects receiving statin medications for routine cardiovascular indications had a trend of lower WMF, but not lesion, permeability. This is the first demonstration of brain vascular hyperpermeability in humans with an autosomal dominant disease, as predicted mechanistically. Brain permeability, more than lesion permeability, may serve as a biomarker of CCM disease activity, and help calibrate potential drug therapy.
机译：家族性脑海绵状畸形（CCM）的患者对CCM1，CCM2或CCM3基因的单倍不足。相应的CCM蛋白的丢失会增加RhoA激酶介导的体外和小鼠脑内内皮通透性。一项前瞻性病例对照观察性研究调查了患有家族性CCM的人类受试者的大脑是否通过动态对比增强定量灌注磁共振成像与没有家族性疾病的CCM病例相比，显示血管通透性高，以及病变或脑血管通透性是否与CCM相关疾病活动。与散发性病例相比，家族性白质病（WMF）远高于病灶，但CCM病灶的通透性相似。 WMF的通透性随散发患者的年龄而增加，但对于家族性病例则没有。与较轻的疾病表型相比，家族性CCM疾病侵袭性较高的患者具有更高的WMF渗透性，但病灶渗透性相似。接受他汀类药物作为常规心血管适应症的受试者的WMF呈降低趋势，但病变的通透性却没有。如机制预测的那样，这是常染色体显性疾病人类脑血管通透性的首次证明。脑部通透性比病灶通透性更高，可以作为CCM疾病活动的生物标志物，并有助于校准潜在的药物治疗。

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