Best John, Chapleau Marianne, Rabinovici Gil D
Memory and Aging Center, Department of Neurology, University of California San Francisco, San Francisco, CA, USA.
Departments of Neurology, Radiology & Biomedical Imaging, Weill Institute for Neurosciences, University of California San Francisco, San Francisco, CA, USA.
Expert Rev Neurother. 2023 Mar;23(3):227-236. doi: 10.1080/14737175.2023.2190885. Epub 2023 Mar 15.
Posterior Cortical Atrophy (PCA) is a neurodegenerative disorder characterized by impairment of higher-order visual processing in the setting of progressive atrophy of the parietal and occipital lobes. The underlying pathology is variable but most commonly Alzheimer's disease. The majority of individuals develop symptoms before 65 years of age; however, delayed diagnosis is common due to misattribution of symptoms to ocular rather than cortical pathology.
The purpose of this review is to provide readers with an in-depth analysis of Posterior Cortical Atrophy syndrome, including clinical, imaging, pathological, and genetic features, management, and treatments.
Most patients present initially with a relatively pure visuoperceptual-visuospatial syndrome, though other cognitive domains become affected over time. Structural neuroimaging demonstrates parieto-occipital or temporo-occipital predominant atrophy. Cerebrospinal fluid Alzheimer's disease biomarkers, or amyloid/tau PET imaging can help evaluate for underlying Alzheimer's disease, which is the most common underlying neuropathology. The cornerstone of management is focused on nonpharmacologic measures. Early etiologic diagnosis is important with the arrival of disease-modifying therapies, especially for Alzheimer's disease.
后皮质萎缩（PCA）是一种神经退行性疾病，其特征是在顶叶和枕叶进行性萎缩的情况下高阶视觉处理受损。潜在病理情况多样，但最常见的是阿尔茨海默病。大多数患者在65岁之前出现症状；然而，由于症状被误诊为眼部而非皮质病变，延迟诊断很常见。
本综述的目的是为读者提供对后皮质萎缩综合征的深入分析，包括临床、影像学、病理和遗传特征、管理及治疗。
大多数患者最初表现为相对单纯的视觉感知 - 视觉空间综合征，不过随着时间推移其他认知领域也会受到影响。结构神经影像学显示以顶枕叶或颞枕叶为主的萎缩。脑脊液阿尔茨海默病生物标志物或淀粉样蛋白/ tau正电子发射断层显像（PET）成像有助于评估潜在的阿尔茨海默病，这是最常见的潜在神经病理学情况。管理的基石侧重于非药物措施。随着疾病修饰疗法的出现，早期病因诊断很重要，尤其是对于阿尔茨海默病。
