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后部皮质萎缩:一种罕见的痴呆形式,有β淀粉样蛋白积累的活体证据。

Posterior cortical atrophy: a rare form of dementia with in vivo evidence of amyloid-beta accumulation.

作者信息

Tenovuo Olli, Kemppainen Nina, Aalto Sargo, Någren Kjell, Rinne Juha O

机构信息

Department of Neurology, University of Turku, Turku, Finland.

出版信息

J Alzheimers Dis. 2008 Nov;15(3):351-5. doi: 10.3233/jad-2008-15301.

DOI:10.3233/jad-2008-15301
PMID:18997288
Abstract

Posterior cortical atrophy (PCA) is a rare form of degenerative dementia, which is characterized by progressive atrophy of occipital and parietal cortical areas. It usually manifests as increasing difficulties of visuoperceptive abilities. Later on, memory and other cognitive functions are involved. Various pathologies have been associated with clinical PCA presentation, but most of the patients with autopsy have had Alzheimer-type pathology. Thus, PCA has been considered to be a rare form of Alzheimer-type dementia with unusual pathological distribution. Here we describe a patient who had a typical clinical course for this syndrome and who showed a positive accumulation of amyloid-beta in posterior areas studied with positron emission tomography.

摘要

后部皮质萎缩(PCA)是一种罕见的退行性痴呆形式,其特征是枕叶和顶叶皮质区域进行性萎缩。它通常表现为视觉感知能力日益困难。随后,记忆和其他认知功能也会受到影响。多种病理情况与临床PCA表现相关,但大多数尸检患者患有阿尔茨海默病类型的病理改变。因此,PCA被认为是阿尔茨海默病类型痴呆的一种罕见形式,具有不寻常的病理分布。在此,我们描述一名具有该综合征典型临床病程的患者,其在正电子发射断层扫描研究的后部区域显示β淀粉样蛋白呈阳性积聚。

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