Crutch Sebastian J
Dementia Research Centre, Department of Neurodegeneration, UCL Institute of Neurology, University College London, UK.
J Neuropsychol. 2014 Sep;8(2):157-70. doi: 10.1111/jnp.12011. Epub 2013 Mar 4.
Posterior cortical atrophy (PCA) is a syndrome defined by focal neurodegeneration of the parietal, occipital, and occipito-temporal cortices and associated with progressive dysfunction of visual processing, praxis, numeracy and reading. The condition is most commonly caused by (and viewed as an atypical presentation of) Alzheimer's disease, although can also be caused by other degenerative diseases. The current paper examines the relationship of PCA to other degenerative syndromes, and considers what comparisons of these syndromes and disease phenotypes can tell us about underlying disease mechanisms. The focus then turns to neuropsychological investigations of the cognitive basis of symptoms which, although unusual in the broader context of a dementia clinic, are particularly characteristic of the PCA syndrome, before exploring implications for clinical management and patient and carer support.
后部皮质萎缩(PCA)是一种由顶叶、枕叶和枕颞叶皮质的局灶性神经变性所定义的综合征,与视觉处理、运用、计算和阅读的进行性功能障碍相关。该病症最常见的病因是(并被视为)阿尔茨海默病的非典型表现,不过也可能由其他退行性疾病引起。本文探讨了PCA与其他退行性综合征的关系,并思考这些综合征和疾病表型的比较能让我们了解到哪些潜在的疾病机制。接着重点转向对症状认知基础的神经心理学研究,这些症状在痴呆症诊所的更广泛背景下虽不常见,但却是PCA综合征的特别特征,之后探讨了其对临床管理以及患者和护理人员支持的影响。