Abstract:

Posterior cotical atrophy (PCA) is a kind of progressive dementia with main clinical manifestations of visual dysfunction as the starting symptom and associated with progressive cognitive disorder. The histopathology and imaging of PCA show visual dysfunction, neuritic plaques (NPs) and neurofibrillary tangles (NFTs), which are the same as those in Alzheimer's disease (AD). Researches also showed that mutation of presenilin?1 (PS?1) gene and apolipoprotein E (ApoE) genotype may participate in the occurrence and development of PCA. Parieto ? occipital lobe atrophy, hypoperfusion and/or glucose metabolism reduction in the right posterior cerebrum can be seen in PCA. Cholinesterase inhibitors (ChEIs) may improve the symptoms and postpone the progression of illness. No unified diagnostic criteria will reduce the comparability between different studies. Reasonable usage of the diagnostic criteria of PCA will be helpful in classifying and differentiating this disease.

Key words: Cerebral cortex, Atrophy, Review

摘要：

后部皮质萎缩是以视觉障碍伴进行性认知功能障碍为主要表现的进展性痴呆。其首发症状、组织病理学和影像学分别表现为视觉障碍、与阿尔茨海默病相同的神经炎性斑（亦称老年斑）和神经原纤维缠结形成（亦有研究发现PS-1 基因和ApoEε4 等位基因突变可能参与后部皮质萎缩的发病过程），以及以大脑后部右侧为主的顶枕叶萎缩、低灌注和（或）葡萄糖代谢降低。胆碱酯酶抑制剂可以改善临床症状并延缓病情。无统一诊断标准使得这些研究之间的可比度大为降低。合理应用诊断标准将有助于疾病分型和鉴别诊断。

关键词: 大脑皮质, 萎缩, 综述