Mehler M F
Department of Neurology, Albert Einstein College of Medicine, Bronx, New York.
Cortex. 1988 Dec;24(4):545-54. doi: 10.1016/s0010-9452(88)80048-0.
The neurolinguistic and cognitive profiles of a patient with mixed transcortical aphasia and non-familial dysphasic dementia associated with progressive, left perisylvian involution are presented. This clinicopathological entity has recently been shown to be an example of a novel class of intrinsic, focal cortical degenerations with sparing of the basal forebrain. The characteristics of the aphasia were unusual. There were occasional literal and verbal paraphasic errors, but no completion phenomenon, embellishment or significant echolalia. Evidence of a generalized lexical disruption was found on detailed analysis of residual abilities in reading, writing and spelling. A newly described form of non-lexical repetition was present, resulting in the patient's inability to correct syntactical and semantic errors. Affective prosodic repetition was intact. A combined paraphasic word-production and semantic anomia was found. These observations combined with evidence of evolving region-specific cognitive impairments suggest that the pathological process resulted in a relative disconnection of integral frontal and parieto-occipital areas from adjacent perisylvian language zones.
本文报告了一例患有混合性经皮质失语症和与进行性左侧外侧裂周围脑萎缩相关的非家族性失语性痴呆患者的神经语言学和认知特征。最近研究表明，这种临床病理实体是一类新型的原发性局灶性皮质变性的例子，不累及基底前脑。该失语症的特征不同寻常。偶尔会出现字面和言语性错语，但没有完成现象、修饰或明显的模仿言语。通过对阅读、写作和拼写剩余能力的详细分析，发现了广泛词汇破坏的证据。存在一种新描述的非词汇性重复形式，导致患者无法纠正句法和语义错误。情感韵律重复功能完好。发现了错语性单词生成和语义性命名障碍并存的情况。这些观察结果与不断发展的区域特异性认知障碍的证据相结合，表明病理过程导致额叶和顶枕叶整体区域与相邻的外侧裂周围语言区域相对分离。
