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11 年内后部皮质萎缩的纵向进展:病变拓扑与临床缺陷之间的关系。

Longitudinal progression of posterior cortical atrophy over 11 years: Relationship between lesion topology and clinical deficits.

机构信息

a Department of Psychiatry and Psychotherapy, Medical Faculty, Heinrich Heine University , Düsseldorf , Germany.

b Institute of Anatomy, Faculty of Medicine, University of Belgrade , Belgrade , Serbia.

出版信息

J Clin Exp Neuropsychol. 2019 Oct;41(8):875-880. doi: 10.1080/13803395.2019.1638345. Epub 2019 Jul 19.

DOI:10.1080/13803395.2019.1638345
PMID:31322045
Abstract

Posterior cortical atrophy (PCA) is a rare form of dementia primarily characterized by slowly progressing deterioration of visual processing corresponding to atrophy in the posterior parietal and occipital cortices with less prominent memory loss than are usually seen in other forms of dementia such as Alzheimer's Disease (AD). In the present case report, we describe longitudinal data over a period of 11 years regarding clinical and neuropsychological impairments and their relation to the location and extent of cortical changes related to higher order visual processing in a patient with posterior cortical atrophy. In our patient, visual processing deficits concerning space, motion and object perception emerged at the age of 50 and continued to worsen. By the age of 58, while the perception of contrast, color and figure-ground separation appeared undisturbed the patient exhibited pronounced dorsal- and ventral-related visual deficits, which continued to worsen with age. The patient's MRI scans over the course of the disease revealed increasing circumscribed and bilateral atrophy of the parietal and occipital cortices, with a right-sided predominance. The specific localization of cortical atrophy, the slow progression characterized by visual processing deficits and relatively preserved memory were the main criteria for the diagnosis of posterior cortical atrophy. The case report also highlights the importance of an early extensive neurological and neuropsychological evaluation of visual deficits that occur without the presence of ophthalmological disease.

摘要

后部皮质萎缩症(PCA)是一种罕见的痴呆症形式,主要表现为视觉处理逐渐恶化,与后部顶叶和枕叶皮质萎缩相对应,与阿尔茨海默病(AD)等其他形式的痴呆症相比,记忆丧失不那么明显。在本病例报告中,我们描述了一名后部皮质萎缩症患者的纵向数据,这些数据涉及临床和神经心理学损伤及其与与高级视觉处理相关的皮质变化的位置和程度的关系。在我们的患者中,与空间、运动和物体感知相关的视觉处理缺陷在 50 岁时出现,并持续恶化。到 58 岁时,尽管对比、颜色和图形-背景分离的感知没有受到干扰,但患者表现出明显的背侧和腹侧相关的视觉缺陷,这些缺陷随着年龄的增长而继续恶化。该患者在疾病过程中的 MRI 扫描显示出顶叶和枕叶皮质的局限性和双侧进行性萎缩,右侧更为明显。皮质萎缩的特定定位、以视觉处理缺陷为特征的缓慢进展以及相对保留的记忆是后部皮质萎缩症诊断的主要标准。该病例报告还强调了对没有眼科疾病的视觉缺陷进行早期广泛的神经学和神经心理学评估的重要性。

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引用本文的文献

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Posterior Cortical Atrophy: Characteristics From a Clinical Data Registry.后部皮质萎缩:来自临床数据登记处的特征
Front Neurol. 2020 Jun 3;11:358. doi: 10.3389/fneur.2020.00358. eCollection 2020.