Department of Neuroscience, University Kentucky, Lexington, KY, 40536, USA.
Department of Pathology and Laboratory Medicine, David Geffen SOM at UCLA and Ronald Reagan UCLA Medical Center, Los Angeles, CA, 90095-1732, USA.
Acta Neuropathol. 2021 Jan;141(1):1-24. doi: 10.1007/s00401-020-02235-6. Epub 2020 Oct 24.
Brain arteriolosclerosis (B-ASC), characterized by pathologic arteriolar wall thickening, is a common finding at autopsy in aged persons and is associated with cognitive impairment. Hypertension and diabetes are widely recognized as risk factors for B-ASC. Recent research indicates other and more complex risk factors and pathogenetic mechanisms. Here, we describe aspects of the unique architecture of brain arterioles, histomorphologic features of B-ASC, relevant neuroimaging findings, epidemiology and association with aging, established genetic risk factors, and the co-occurrence of B-ASC with other neuropathologic conditions such as Alzheimer's disease and limbic-predominant age-related TDP-43 encephalopathy (LATE). There may also be complex physiologic interactions between metabolic syndrome (e.g., hypertension and inflammation) and brain arteriolar pathology. Although there is no universally applied diagnostic methodology, several classification schemes and neuroimaging techniques are used to diagnose and categorize cerebral small vessel disease pathologies that include B-ASC, microinfarcts, microbleeds, lacunar infarcts, and cerebral amyloid angiopathy (CAA). In clinical-pathologic studies that factored in comorbid diseases, B-ASC was independently associated with impairments of global cognition, episodic memory, working memory, and perceptual speed, and has been linked to autonomic dysfunction and motor symptoms including parkinsonism. We conclude by discussing critical knowledge gaps related to B-ASC and suggest that there are probably subcategories of B-ASC that differ in pathogenesis. Observed in over 80% of autopsied individuals beyond 80 years of age, B-ASC is a complex and under-studied contributor to neurologic disability.
脑小动脉病(B-ASC)的特征是小动脉壁病理性增厚,在老年尸检中很常见,并与认知障碍有关。高血压和糖尿病被广泛认为是 B-ASC 的危险因素。最近的研究表明,还有其他更复杂的危险因素和发病机制。在这里,我们描述了脑小动脉独特的结构方面、B-ASC 的组织形态学特征、相关的神经影像学发现、与衰老的流行病学和相关性、已确定的遗传风险因素,以及 B-ASC 与其他神经病理学状况(如阿尔茨海默病和边缘为主的年龄相关性 TDP-43 脑病(LATE))的共同发生。代谢综合征(如高血压和炎症)与脑小动脉病理之间可能存在复杂的生理相互作用。尽管没有普遍应用的诊断方法学,但有几种分类方案和神经影像学技术用于诊断和分类包括 B-ASC、微梗死、微出血、腔隙性梗死和脑淀粉样血管病(CAA)在内的脑小血管疾病病理学。在考虑合并症的临床病理研究中,B-ASC 与总体认知、情景记忆、工作记忆和知觉速度受损独立相关,并与自主神经功能障碍和运动症状(包括帕金森病)相关。最后,我们讨论了与 B-ASC 相关的关键知识空白,并提出 B-ASC 可能存在不同发病机制的亚类。B-ASC 在超过 80 岁的尸检个体中超过 80%观察到,是导致神经功能障碍的复杂而研究不足的因素。