Abstract: Objective To summarize the clinical and imaging features of patients with posterior cortical atrophy (PCA). Methods and Results Five patients with PCA admitted in West China Hospital, Sichuan University from April to June 2021 were included. All of them showed spatial perception disorder, simultaneous agnosia, miscalculation, agraphia, structural apraxia, environmental agnosia. Apraxia of dressing and agnosia of left and right were found in 3 cases. One case had facial agnosia, visual ataxia and object perception disorder. Cerebrospinal fluid (CSF) biomarker determination:amyloid β-protein 42 (Aβ₄₂) decreased in 3 cases, the ratio of Aβ₄₂/amyloid β-protein 40 (Aβ₄₀) decreased significantly in 2 cases, the content of total tau protein (t-tau) increased in all 5 cases, phosphorylated tau 181 protein (p-tau181) increased in 3 cases. Neuropsychological tests showed that:1) Five patients all had different degrees of cognitive dysfunction and impairment of activities of daily living, as well as impairment of visuospatial ability, visual memory and attention. 2) Three cases had anxiety and depression, language and executive function were impaired in 3 cases, and verbal memory was impaired in 2 cases. Head MRI examination all indicated different degrees of parieto-occipital-temporal cortical atrophy. Hypometabolism in posterior regions involving parietal and temporal cortex was observed in one case with ¹⁸F-FDG PET. Three cases completed the visual field examination, including 5 cases of binocular vision loss, one case of complete visual field defect, 2 cases of partial visual field defect. Except one case lost to follow-up, other 4 cases were followed up for 20-22 months. Two cases of cognitive function, visuospatical ability decline, of which one case could not take care of themselves most of the life. Cognitive function and visuospatial ability improved in one case. There was no significant change in cognitive function and visuospatial ability in one case. Conclusions PCA should be considered in patients with relatively early-onset of agnosia, visual field defect and visuospatial impairment. Comprehensive neuropsychological tests related to visual function, brain MRI, ¹⁸F-FDG PET and CSF biomarkers would be helpful for accurate diagnosis.

Key words: Cerebral cortex, Atrophy, Cognition disorders, Magnetic resonance imaging, Positron-emission Tomography

摘要： 目的 总结后部皮质萎缩的临床及影像学特征。方法与结果 纳入2021年4-6月四川大学华西医院收治的5例后部皮质萎缩患者，均表现为空间感知觉障碍、同时性失认、失算、失写、结构性失用、环境失认，以及各有3例存在穿衣失用、左右失认，有1例存在面孔失认、视觉性共济失调、物体感知觉障碍；脑脊液生物学标志物测定，3例β-淀粉样蛋白42（Aβ₄₂）降低，2例Aβ₄₂/β-淀粉样蛋白40（Aβ₄₀）比值显著降低，5例总t-tau蛋白水平均升高，3例磷酸化tau蛋白181水平升高；神经心理学测验，5例均存在不同程度认知功能障碍和日常生活活动能力障碍，以及视空间能力、视觉记忆、注意力减退，3例伴焦虑或抑郁情绪，3例语言功能和执行功能下降，2例言语记忆减退；头部MRI检查，5例均存在不同程度顶枕颞皮质萎缩，1例¹⁸F-FDG PET显示颞顶皮质葡萄糖代谢明显降低。5例双眼视力下降，1例全视野缺损、2例部分视野缺损。除1例失访外，余4例平均随访21个月，2例患者认知功能、视空间能力减退，其中1例生活大部分无法自理； 1例认知功能、视空间能力改善； 1例认知功能、视空间能力未见明显变化。结论 对于发病年龄较早，以失认、视野缺损、视空间能力障碍等为核心症状的患者，可以完善视觉相关神经心理学测验、头部MRI和¹⁸F-FDG PET检查、脑脊液生物学标志物测定等，以辅助诊断后部皮质萎缩。

关键词: 大脑皮质, 萎缩, 认知障碍, 磁共振成像, 正电子发射断层显像术