Delgado D Carolina, Donoso S Archibaldo
Hospital Clínico de la Universidad de Chile. Santiago de Chile.
Rev Med Chil. 2009 Nov;137(11):1482-7. Epub 2010 Jan 13.
Posterior cortical atrophy (PCA) is a neurodegenerative syndrome, usually due to Alzheimer's disease. The first symptoms are progressive impairment of visuo spatial (Balint's and Gertsmann's syndromes) or visuo perceptive (visual agnosia, alexia) function. Episodic memory and executive function are spared until later stages. We report two males aged 51 and 55years and three females aged 50, 54 and 56 years, with posterior cortical atrophy. Ophthalmologic study was normal in all. Presenting signs and symptoms were visual ataxia, simultagnosia, agraphia, acalculia, spatial disorientation and unilateral neglect (Balint's and Gerstmann's syndromes). Apperceptive visual agnosia, aphasia, apraxia and alexia were also observed. One female had cortical blindness. Structural images were inconclusive, but PET scan and SPECT disclosed functional impairments in occipitotemporal or occipitoparietal areas.
后部皮质萎缩(PCA)是一种神经退行性综合征,通常由阿尔茨海默病引起。最初症状是视空间功能(巴林特综合征和格斯特曼综合征)或视感知功能(视觉失认、失读症)进行性受损。情景记忆和执行功能在疾病后期才会受到影响。我们报告了两名年龄分别为51岁和55岁的男性以及三名年龄分别为50岁、54岁和56岁的女性患有后部皮质萎缩。所有患者的眼科检查均正常。主要症状和体征包括视觉共济失调、同时失认、失写症、失算症、空间定向障碍和单侧忽视(巴林特综合征和格斯特曼综合征)。还观察到统觉性视觉失认、失语症、失用症和失读症。一名女性患有皮质盲。结构影像检查结果不明确,但PET扫描和SPECT显示枕颞或枕顶叶区域存在功能障碍。