后部皮质萎缩的新分类与诊断共识解读
Interpretation of the new consensus on classification and diagnosis of posterior cortical atrophy
后部皮质萎缩(posterior cortical atrophy,PCA)是一种以神经退行性变为特征的临床综合征,其核心症状为视觉障碍和顶枕叶皮质萎缩相关的认知功能衰退,神经影像学表现为后部皮质脑萎缩、血流灌注减少及代谢减低。PCA最常见的病理表现是后部皮质的淀粉样斑块沉积和神经原纤维缠结,分子生物学标志物为脑脊液β-淀粉样蛋白1-42水平减低及总tau蛋白和(或)磷酸化tau蛋白水平增加,故认为PCA是阿尔茨海默病(Alzheimer′s disease,AD)的一种非典型形式。然而部分PCA患者的临床影像学特征可能同时满足其他神经系统退行性疾病的诊断,并且与AD相关生物学标志物呈现阴性,提示PCA临床表现及病理生物机制存在较大的异质性,也促使PCA工作组就PCA分类及诊断建立了新的共识,以促进AD、非典型AD及相关综合征的研究。文中就最新提出的PCA分类及诊断标准做一简要介绍和解读。
更多Posterior cortical atrophy (PCA) is a kind of neurodegenerative dementia. The core feature of the PCA includes progressive decline in visual processing and other posterior parietal-occipital cortex-related cognitive functions. Recently, neuroimaging features of PCA from magnetic resonance imaging/single photon emission computed tomography/ 18F-deoxyglucose positron emission tomography-computer tomography studies represent the typical characteristics of cortical atrophy, hypoperfusion, and hypometabolism in the posterior parietal-occipital cortex. The most common neuropathological changes of PCA are amyloid plaques deposition and neurofibrillary tangles in posterior cortex, while the molecular biomarkers are decreased amyloid β-protein 1-42 together with increased T-tau and/or P-tau in cerebral spinal fluid. From this point, PCA is also considered as an atypical form of Alzheimer′s disease (AD). However, individuals fulfilling the criteria for the core clinico-radiological PCA syndrome, can also fulfill the core clinical criteria for any other neurodegenerative syndrome, and represent negative AD-related pathophysiological biomarkers. Heterogeneity within the PCA syndrome and pathophysiological biomarkers prompt the PCA working group to establish a new consensus on PCA classification and diagnostic criteria, which is proposed for use in a number of different research contexts and the research of AD, atypical AD and related syndromes. This paper gives a brief introduction and interpretation of the newly proposed classification and diagnostic criteria of PCA.
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