The Impact of Cerebral Amyloid Angiopathy in Various Neurodegenerative Dementia Syndromes: A Neuropathological Study
The Impact of Cerebral Amyloid Angiopathy in Various Neurodegenerative Dementia Syndromes: A Neuropathological Study
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DOI：
10.1155/2019/7247325
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发表时间：
2019-01-01
影响因子：
1.5
通讯作者：
De Reuck, Jacques
中科院分区：
文献类型：
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作者：
De Reuck, Jacques
目的。脑淀粉样血管病(CAA)的波士顿标准必须通过尸检来确认。本研究旨在探讨各种神经退行性痴呆性疾病中CAA严重程度的发生率及其对脑血管的影响。材料和方法。208名患者接受了尸检。其中阿尔茨海默病(AD)92例，额颞叶变性(FTLD)46例，进行性核上性瘫痪(PSP)24例，路易体痴呆(LBD)21例，皮质基底膜变性(CBD)5例，对照组20例。除大体检查外，还在乳头体水平取大脑半球的整个冠状切面，对小的脑血管病变进行半定量显微镜评估。结果。CAA存在于2/3%的AD患者脑组织中，其中一半为重度3级，仅后者脑血管病变较多。45%的LBD患者大脑中存在CAA。皮质微梗塞仅在CAA 3级组中更为常见。在LBD中，41%的CAA分级为0级，83%为1-2级，100%为3级。在PSP中，仅有21%的CAA分级为1-2级。FTLD、CBD和正常对照组均未观察到CAA病理改变。结论。目前的研究表明，CAA最常与AD有关，但只有严重的CAA才表现出更多的脑血管病变。LBD是与CAA相关的第二种最常见的疾病，相关AD特征的发生率与CAA日益严重之间存在明显的相关性。在PSP中，只有21%的人表现出轻微的CAA特征。PSP、tau-FTLD和CBD是Pick复杂疾病的一部分，众所周知，与AD和LBD大脑相比，这些疾病具有良好的血管轮廓，可以解释其脑血管病变发生率较低的原因。
Purpose. The Boston criteria for cerebral amyloid angiopathy (CAA) have to be confirmed by postmortem examination. The present study investigates the incidence and the cerebrovascular impact of the severity of CAA in various neurodegenerative dementia diseases. Material and Methods. 208 patients underwent an autopsy. They consisted of 92 brains with Alzheimer's disease (AD), 46 with frontotemporal lobar degeneration (FTLD), 24 with progressive supranuclear palsy (PSP), 21 with Lewy body dementia (LBD), 5 with corticobasal degeneration (CBD), and 20 controls. In addition to the macroscopic examination, a whole coronal section of a cerebral hemisphere, at the level of the mamillary body, was taken for semiquantitative microscopic evaluation of the small cerebrovascular lesions. Results. CAA is present in 2/3% of the AD brains of which half of them have a severe form, grade 3. Only the latter displays more cerebrovascular lesions. CAA is present in 45% of the LBD brains. Cortical microinfarcts are only more frequent in the CAA grade 3 group. In LBD additional AD pathology is present in 41% of the CAA grade 0, 83% in grade 1-2, and 100% in grade 3. In PSP only 21% had CAA grade 1-2. In FTLD, CBD, and normal controls no CAA pathology is observed. Conclusions. The present study shows that CAA is most frequently associated to AD but that only the severe form displays more cerebrovascular lesions. LBD is the second most frequent disease associated to CAA with a clear correlation between the incidence of the associated AD features and the increasing severity of the CAA. In PSP only 21% display mild CAA features. PSP, tau-FTLD, and CBD are part of the Pick complex diseases, who are known to have a favourable vascular profile which can explain their low incidence of cerebrovascular lesions, in contrast to AD and LBD brains.