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一名后皮质萎缩患者的异常阅读错误模式

Unusual Pattern of Reading Errors in a Patient with Posterior Cortical Atrophy.

作者信息

Pavisic Ivanna M, Yong Keir X X, Primativo Silvia, Crutch Sebastian J, Suarez Gonzalez Aida

机构信息

Dementia Research Centre, Department of Neurodegenerative Diseases, Queen Square Institute of Neurology, University College London (UCL), London, United Kingdom.

Department of Human Science, LUMSA University, Rome, Italy.

出版信息

Case Rep Neurol. 2019 May 8;11(2):157-166. doi: 10.1159/000500081. eCollection 2019 May-Aug.

Abstract

Posterior cortical atrophy (PCA) is a degenerative condition characterized by a progressive deterioration of visual processing. Dyslexia constitutes an early and frequent visual symptom of the disease and previous comprehensive investigations in series of individuals have extensively documented a characteristic abundance of visual errors as the most prevalent error category in this population. Here we describe the profile of a patient with PCA, C.P., who presents an unusual prevalence of phonological, instead of purely visual, errors in his reading, in the context of an otherwise classic PCA phenotype. In keeping with the well-known PCA profile, C.P. exhibited deficits at the pre-lexical level with elements of crowding and defective early visual processing impairments but additionally showed an unusually prominent disruption of phonological processing. We also argue that our patient may have a refractory access type deficit in reading given that accuracy doubled with the introduction of a five-second response-stimulus interval. To our knowledge, no previous case of a refractory deficit affecting word reading has been reported in PCA. Our examination builds on previous knowledge about reading behaviour in PCA and describes a singular example of the rich phenotypic heterogeneity within the syndrome.

摘要

后部皮质萎缩(PCA)是一种退行性疾病,其特征是视觉处理能力逐渐衰退。阅读障碍是该疾病早期常见的视觉症状,此前对一系列个体的全面研究广泛记录了在这一人群中,视觉错误大量存在是最普遍的错误类型。在此,我们描述了一位PCA患者C.P.的情况,在典型PCA表型的背景下,他在阅读中出现了不寻常的语音错误而非单纯的视觉错误。与众所周知的PCA特征相符,C.P.在词汇前水平表现出缺陷,存在拥挤效应以及早期视觉处理缺陷,但同时语音处理也出现了异常显著的紊乱。我们还认为,鉴于引入五秒的反应 - 刺激间隔后准确率翻倍,我们的患者在阅读中可能存在难治性加工类型缺陷。据我们所知,此前PCA中尚未有影响单词阅读的难治性缺陷病例的报道。我们的检查基于先前对PCA阅读行为的了解,并描述了该综合征丰富表型异质性的一个独特例子。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70bc/6738147/1268b3bc7729/crn-0011-0157-g01.jpg

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