脑小血管病--影像识别讲解材料.pptVIP

脑小血管病-----影像识别;脑小血管病病理分型;1.小动脉硬化; Features of VP subtypes;神经影像学特征表现是脑白质弥漫性疏松性病变，皮质不受累。 CT表现为脑室周围、半卵圆中心白质的低密度。 MRI表现为侧脑室周围对称性弥漫性白质高信号。 ;二、糖尿病;;2.脑淀粉样血管病;2.脑淀粉样血管病;Flair像显示非特异性表现：脑容积中度弥漫性减少，伴脑沟增宽、脑室扩大，伴深部及周围白质散在的不规则高信号病灶。T2成像显示淀粉样血管病的特征：皮质及近皮质的幕上脑实质大量微出血病灶，基底节区未受累。;3.遗传性小血管病 ;一、CADASIL;前颞叶、外囊、额叶上部旁正中区受累是CADASIL高度特异性表现。;二、常染色体显性遗传性视网膜血管病伴有白质脑病（ADRVCL）;Cerebroretinal vasculopathy (CRV) is an adult-onset autosomal dominant disorder involving the microvessels of the brain and retina due to frameshift mutations in the gene TREX1. Patients present with vision loss, seizures, hemiparesis, apraxia, dysarthria, or memory loss. There is progression to blindness, a neurovegetative state, and death within 5 to10 years. MRI reveals a tumor-like lesion in approximately half of patients, resembling a primary brain malignancy, while in the other half there are multiple small white matter lesions which may be misdiagnosed as demyelinating disease. The purpose of this report is to heighten awareness of this disease and to note near resolution of the tumor-like lesion.;Sequential axial MRI showing an ovoid T2-hyperintense (A) and gadolinium-enhancing (B) lesion (2.1 ×1 cm), abutting the frontal horn ofthe right lateral ventricle,brighton both diffusion weighte dimaging(DWI) and apparent diffusion coefficient (ADC) map.;; At 12 months, the lesion approximated its size on initial imaging (1.4 × 1.2 ×0.9 cm) with a persistent rim pattern of enhancement (E, F). ;At 18 months, the lesion further decreased in size (1.1 × 0.9×0.3 cm) with near resolution of the surrounding edema. Fluorescein angiography and indocyanine green with corresponding color photographs of the retina show views of the macula of the right eye (G–I). Periarteriolar narrowing and sheathing, focal leakage, telangiectasias, and cotton wool spots are present.;三、伴钙化和囊变的脑视网膜微血管病（CRMCC或LCC）;临床表现： CRMCC：一种伴有先天性视网膜毛细血管扩张症病理改变产生了异常的血管渗透性，视网膜脱离和失明，早产，贫血，胃肠道出血，骨折和骨生长不良。 LCC:癫痫发作、认知功能下降、以及基底节、小脑和锥体束征. 该综合症影像婴儿和青少年表现较典型，而在成年人表现轻微。 最