The language profile of posterior cortical atrophy
The language profile of posterior cortical atrophy
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DOI：
10.1136/jnnp-2012-303309
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发表时间：
2013-04-01
影响因子：
11
通讯作者：
Rohrer, Jonathan D.
中科院分区：
文献类型：
--
作者：
Crutch, Sebastian J.;Lehmann, Manja;Rohrer, Jonathan D.
背景后部皮质萎缩(PCA)通常被认为是一种视觉综合征，其主要特征是视知觉和视空间技能的进行性损害。然而，患者通常描述早期的单词检索困难。本文首次系统分析了主成分分析中的语言功能。方法15例PCa患者、7例对数/语音失语(LPA)患者和18例年龄匹配的健康受试者完成了一系列详细的语言测试，评估听觉输入加工、重复和工作记忆、词汇和语法理解、单字提取和流利性以及自发说话。结果与健康对照组相比，PCA患者在所有检查领域都有语言障碍，但以异常、音素流畅性降低和语速减慢最为突出。在听觉输入加工、重复和数字广度的测试中，PCA的表现与LPA患者最为相似，但在理解和自发言语任务上，PCA的表现相对更好。结论本研究表明，PCA的特征除了视力、识字和计算能力的退化外，还表现为进行性口语语言障碍，并伴有突出的单词提取困难。Pca和LPA的语言特征重叠，这两种疾病都是由阿尔茨海默病最常见的原因，这进一步强调了这种疾病的典型和非典型表现之间的表型连续体的概念。明确阿尔茨海默病表型之间的界限对于阿尔茨海默病的诊断、临床试验招募和研究驱动阿尔茨海默病表型异质性的生物学因素具有重要意义。需要康复策略来改善PCA的语音缺陷。
Background Posterior cortical atrophy (PCA) is typically considered to be a visual syndrome, primarily characterised by progressive impairment of visuoperceptual and visuospatial skills. However, patients commonly describe early difficulties with word retrieval. This paper details the first systematic analysis of linguistic function in PCA. Characterising and quantifying the aphasia associated with PCA is important for clarifying diagnostic and selection criteria for clinical and research studies.Methods 15 patients with PCA, seven patients with logopenic/phonological aphasia (LPA) and 18 age matched healthy participants completed a detailed battery of linguistic tests evaluating auditory input processing, repetition and working memory, lexical and grammatical comprehension, single word retrieval and fluency, and spontaneous speech.Results Relative to healthy controls, PCA patients exhibited language impairments across all of the domains examined, but with anomia, reduced phonemic fluency and slowed speech rate the most prominent deficits. PCA performance most closely resembled that of LPA patients on tests of auditory input processing, repetition and digit span, but was relatively stronger on tasks of comprehension and spontaneous speech.Conclusions The study demonstrates that in addition to the well reported degradation of vision, literacy and numeracy, PCA is characterised by progressive oral language dysfunction with prominent word retrieval difficulties. Overlap in the linguistic profiles of PCA and LPA, which are both most commonly caused by Alzheimer's disease, further emphasises the notion of a phenotypic continuum between typical and atypical manifestations of the disease. Clarifying the boundaries between Alzheimer's disease phenotypes has important implications for diagnosis, clinical trial recruitment and investigations into biological factors driving phenotypic heterogeneity in Alzheimer's disease. Rehabilitation strategies to ameliorate the phonological deficit in PCA are required.