后皮质萎缩的纵向进展超过11年：病变拓扑结构和临床缺陷之间的关系。,Journal of Clinical and Experimental Neuropsychology

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后皮质萎缩的纵向进展超过11年：病变拓扑结构和临床缺陷之间的关系。
Journal of Clinical and Experimental Neuropsychology ( IF 1.8 ) Pub Date : 2019-07-19 , DOI: 10.1080/13803395.2019.1638345
Milenko Kujovic ₁ , Aleksandar Malikovic ₂ , Sarah Jochum ₁ , Zsofia Margittai ₁ , Christian Lange-Asschenfeldt ₁ , Tillmann Supprian ₁

Affiliation

后皮质萎缩症（PCA）是痴呆症的一种罕见形式，其主要特征是与顶叶和枕叶后皮质的萎缩相对应的视力处理缓慢进展，其记忆力较其他痴呆症（如阿尔茨海默氏病）通常少见。广告）。在本病例报告中，我们描述了11年间有关临床和神经心理损害及其与皮质后部萎缩患者的高级视觉处理相关的皮质变化的位置和程度的关系的纵向数据。在我们的患者中，与空间，运动和物体感知有关的视觉处理缺陷在50岁时出现，并持续恶化。到58岁时，虽然感觉到对比，颜色和人物与地面的分离似乎不受干扰，患者表现出明显的背侧和腹侧相关视力缺陷，随着年龄的增长，视力缺陷继续恶化。患者在病程中的MRI扫描显示，顶叶和枕叶皮质的边界和双侧萎缩程度增加，占优势。皮质萎缩的特定定位，以视觉加工缺陷为特征的缓慢进展以及相对保留的记忆是诊断后皮质萎缩的主要标准。病例报告还强调了对没有眼科疾病的情况下发生的视觉缺陷进行早期广泛的神经和神经心理学评估的重要性。

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

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.

更新日期：2019-11-01

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