7例脑淀粉样血管病合并痴呆的临床特点分析

Clinical characteristics of cerebral amyloid angiopathy complicated by dementia in a series of 7 cases

目的:总结经北京协和医院诊断很可能(probable)脑淀粉样血管病(cerebral amyloid angiopathy,CAA)的临床特点。方法:对诊断符合临床很可能(probable)脑淀粉样血管病患者7例的影像学特点和脑脊液痴呆标记物检查进行回顾分析总结。结果:共纳入7例CAA患者,男5例,女2例,平均年龄为70.6±4.8岁。患者起病方式均为隐袭起病,临床症状包括情景记忆障碍、精神行为症状、神经系统定位体征。MRI检查示脑室扩大、脑白质缺血灶、对称性或多发性脑白质缺血灶,脑叶出血病灶,海马MAT分级Ⅰ级(0例)、Ⅱ级(2例)、Ⅲ级(3例)和Ⅳ级(2例)。2例患者行PET-CT检查,双侧前额、双侧顶叶大部及双侧颞叶皮层代谢下降,其表现与AD不易区别。脑脊液痴呆标志物检查示,与非CAA痴呆疾病对照组患者相比较,CAA合并痴呆患者的脑脊液痴呆标志物Aβ42下降,t-tau及p-tau升高,且差异有显著性。结论:痴呆标志物的检查无法鉴别CAA合并痴呆与阿尔茨海默病。如果阿尔茨海默病患者的影像学检查提示有较多的血管性病灶时,进一步行SWI明确多发微出血,对于疑诊CAA的患者很必要。

Objective To review the clinical features at presentation in detail in outpatients with cerebral amyloid angiopathy(CAA)complicated by dementia.Methods Seven patients were enrolled retrospectively from Department of Neurology of Peking Union Medical College Hospital were enrolled when they meet the Boston criteria.The clinical features and their CSF biomarkers and neuroimaging in detail were reviewed and analyzed.Result There were 5 men and 2 women,and their mean age was 70.6±4.8 years old.The mean duration from memory impairment to presence of Parkinsonism was less than 1 year.Situational memory disorder,behavioral and psychiatric symptoms,and focal neurological signs were the major symptoms.MRI showed that Ventricular enlargement,white matter ischemia,white matter ischemia,and lobar hemorrhage in different patients.Neuroimage examination shows MAT grading of hippocampus Ⅰ(0 case)、Ⅱ(2 case)、Ⅲ(3 case)and Ⅳ(2 case).PET showed the cingulate island sign in three patients.FDG PET showed significant lower FDG uptake in both frontal,parietal,and temporal lobe in two patients.The CSF markers(Aβ42,Aβ40,t-tau,and p-tau)have shown the same change in Alzheimer’s disease(AD)patients.The low Aβ42 content and the high t-tau and p-tau content exist in CAA patients.Conclusion Taken together,these results suggest that dementia biomarkers might not be useful to differentiate from AD.There is needed to perform neuroimage examination on SWI to determine whether microbleeds exist in the patient with AD who have a prodigious cerebrovascular burden.