Neary D, Snowden J S, Mann D M
Department of Neurology, Royal Infirmary, Manchester, UK.
J Neurol Neurosurg Psychiatry. 1993 Oct;56(10):1122-5. doi: 10.1136/jnnp.56.10.1122.
Two brothers presented with slowly progressive aphasia. One brother, who became behaviourally disturbed only at the end of his illness, was found at necropsy to have predominant left frontotemporal atrophy. The other brother developed severe behavioural disturbances shortly after the onset of language impairment. His brain revealed bilateral frontotemporal atrophy. In both there was non-Alzheimer's disease pathology with the histological features of loss of large cortical nerve cells, spongiform change and mild gliosis. The differential anatomical atrophy supports the view that clinical manifestations of lobar atrophy are dictated by the topographical distribution of a common underlying pathology, linking the syndromes of progressive aphasia to dementia of frontal lobe type (DFT) and DFT with motor neuron disease.
两兄弟均出现缓慢进展性失语。其中一个兄弟仅在疾病末期出现行为紊乱，尸检发现主要为左侧额颞叶萎缩。另一个兄弟在语言障碍发作后不久就出现了严重的行为紊乱。他的大脑显示双侧额颞叶萎缩。两人均存在非阿尔茨海默病病理改变，组织学特征为大脑皮质大神经细胞丢失、海绵状改变和轻度胶质细胞增生。不同的解剖学萎缩支持这样一种观点，即叶萎缩的临床表现由共同潜在病理改变的地形分布决定，将进行性失语综合征与额叶型痴呆（DFT）以及DFT合并运动神经元病联系起来。
