SEMANTIC DEMENTIA - PROGRESSIVE FLUENT APHASIA WITH TEMPORAL-LOBE ATROPHY
SEMANTIC DEMENTIA - PROGRESSIVE FLUENT APHASIA WITH TEMPORAL-LOBE ATROPHY
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DOI：
10.1093/brain/115.6.1783
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发表时间：
1992-12-01
期刊：
影响因子：
14.5
通讯作者：
FUNNELL, E
中科院分区：
文献类型：
--
作者：
HODGES, JR;PATTERSON, K;FUNNELL, E
我们报告了5例刻板的临床综合征，其特征是流利的言语障碍与严重的命名障碍，减少词汇和突出的障碍，单个词的理解，进展到一个阶段，几乎完全溶解的语义成分的语言。从受限的语义类别（例如动物、车辆等）生成范例的能力显著降低是一个持续的早期特征。语义记忆测试表明，他们对一系列生活和人造物品的知识完全贫乏。相比之下，语音和语法的口语在很大程度上被保留下来，因为是复杂的句法命令的理解。阅读显示出表面阅读障碍的模式。自传体和日常（情节）记忆相对保留。非语言记忆、知觉和视觉空间能力也得到了惊人的保留。在某些情况下，行为和性格的变化可能随之而来;一名患者发展的Kluver-Bucy综合征的功能。放射学检查显示所有5例患者均出现明显的局灶性颞叶萎缩，单次正电子发射断层扫描和正电子发射断层扫描（1例）的功能成像显示所有5例患者均存在颞叶优势。在较早的文献中，此类病例将被归入皮克病的标题下。其他人已被列入一系列进行性失语症。我们提出语义性痴呆这个术语，首先由Snowden et al.（1989）提出，来指定这种临床综合征。
We report five patients with a stereotyped clinical syndrome characterized by fluent dysphasia with severe anomia, reduced vocabulary and prominent impairment of single-word comprehension, progressing to a stage of virtually complete dissolution of the semantic components of language. A marked reduction in the ability to generate exemplars from restricted semantic categories (e.g. animals, vehicles, etc.) was a consistent and early feature. Tests of semantic memory demonstrated a radically impoverished knowledge about a range of living and man-made items. In contrast, phonology and grammar of spoken language were largely preserved, as was comprehension of complex syntactic commands. Reading showed a pattern of surface dyslexia. Autobiographical and day-to-day (episodic) memory were relatively retained. Non-verbal memory, perceptual and visuospatial abilities were also strikingly preserved. In some cases, behavioural and personality changes may supervene; one patient developed features of the Kluver-Bucy Syndrome. Radiological investigations have shown marked focal temporal atrophy in all five patients, and functional imaging by single positron emission tomography and positron emission tomography (one case) have implicated the dominant temporal lobe in all five. In the older literature, such cases would have been subsumed under the rubric of Pick's disease. Others have been included in series with progressive aphasia. We propose the term semantic dementia, first coined by Snowden et al. (1989), to designate this clinical syndrome.