Department of Ophthalmology, University of Lausanne, Jules Gonin Eye Hospital, Neuro-Ophthalmology Unit, Avenue de France 15, 1004, Lausanne, Switzerland,
Curr Neurol Neurosci Rep. 2013 Dec;13(12):406. doi: 10.1007/s11910-013-0406-8.
Posterior cortical atrophy (PCA) is a group of neurodegenerative dementing disorders characterized by initial predominant visual complaints followed by progressive decline in cognitive functions. The visuospatial and visuoperceptual defects arise from the dysfunction of, respectively, the dorsal (occipito-parietal) and the ventral (occipito-temporal) streams. Clinical symptoms, results of neuropsychological examination, and findings of posterior cerebral atrophy and/or posterior hypoperfusion/hypometabolism contribute to the diagnosis. However, owing to the insidious onset of PCA and the non-specificity of initial symptoms, the diagnosis is often delayed. Specific etiologies include Alzheimer's disease, dementia with Lewy bodies, subcortical gliosis, corticobasal degeneration, and prion-associated diseases. Alzheimer's disease accounts for at least 80 % of PCA cases. Recent research has concentrated on better defining the clinical presentation of PCA, improving neuroimaging analysis, testing new neuroimaging techniques, and developing biological measurements. Selected recent papers on PCA are reviewed in this article.
后部皮质萎缩症（PCA）是一组神经退行性痴呆疾病，其特征为最初以明显的视觉障碍为表现，随后认知功能逐渐下降。视空间和视知觉缺陷分别由背侧（枕顶-顶颞）和腹侧（枕颞）流的功能障碍引起。临床症状、神经心理学检查结果、后颅窝萎缩和/或后循环低灌注/低代谢的发现有助于诊断。但是，由于 PCA 的隐匿性起病和初始症状的非特异性，诊断常常被延误。特定病因包括阿尔茨海默病、路易体痴呆、皮质下胶质增生、皮质基底节变性和朊病毒相关疾病。阿尔茨海默病至少占 PCA 病例的 80%。最近的研究集中于更好地定义 PCA 的临床表现，改进神经影像学分析，测试新的神经影像学技术，以及开发生物标志物。本文综述了 PCA 的一些近期研究论文。
