Posterior cortical atrophy (PCA) describes a rare heterogenous neurodegenerative syndrome with early visuospatial and visuoperceptual deficits due to atrophy of parieto-occipital brain regions. Here, we describe the case of a 62-year-old woman showing severe cognitive impairments as well as hemianopsia and all core symptoms of Bálint's syndrome. Years ago, the patient had complained about a "tunnel view" and concentration problems. The diagnostic results point to a case of PCA with underlying Alzheimer pathology. The disease course until diagnosis lasted for 7 years, reflecting the diagnostic difficulties with this still largely unknown syndrome. The unfamiliar symptom presentation including fluctuations in cognitive performance, affective symptoms, cerebrospinal fluid (CSF) biomarkers, which were at first inconspicuous, and a former suspected diagnosis of dissociative pseudodementia, altogether brought considerable uncertainty to the involved health-care professionals. We conclude that cases of "atypical dementia" presenting with visual symptoms, even if appearing unspecific at first, are suspect of PCA. This case report provides an ostensive overview of PCA, including imaging data, CSF-findings, original drawings and handwriting samples from the patient.

中文翻译：

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62岁女性后皮质萎缩的疑难诊断。

后皮质萎缩症（PCA）描述了一种罕见的异质性神经退行性综合症，由于顶枕大脑区域的萎缩而导致早期的视觉空间和视觉上感觉不足。在这里，我们描述了一个62岁女性的案例，该女性表现出严重的认知障碍以及偏瘫和巴林氏综合症的所有核心症状。几年前，患者抱怨“隧道视野”和注意力不集中。诊断结果指向患有潜在阿尔茨海默氏病的PCA病例。直至诊断的疾病过程持续了7年，反映出这种仍然未知的综合症的诊断困难。陌生的症状表现包括认知表现，情感症状，脑脊液（CSF）生物标志物的波动，这些变化最初并不明显，以及以前怀疑可诊断为解离性假性痴呆，这给相关的医疗专业人员带来了很大的不确定性。我们得出的结论是，即使最初乍看起来没有特异性，但出现视觉症状的“非典型痴呆”病例仍可疑为PCA。该病例报告从表面上概述了PCA，包括成像数据，CSF发现，原始图和来自患者的手写样本。