Mehler M F, Horoupian D S, Davies P, Dickson D W
Neurology. 1987 Sep;37(9):1448-53. doi: 10.1212/wnl.37.9.1448.
A nonfamilial syndrome is described in two middle-aged men who presented with progressive aphasia without incipient signs of cognitive impairment. In each case, 2 years elapsed before progressive functional decline or behavioral disabilities supervened. Radiologic studies documented asymmetric left cerebral atrophy that was progressive. The structure of the language disintegration was distinctive and not like that in Alzheimer's disease. Pathologic studies performed at postmortem examination of one patient documented asymmetric cerebral atrophy with nonspecific histopathologic changes. Biochemical studies revealed normal tissue levels of choline acetyltransferase activity, but reduced somatostatin-like immunoreactivity. Since cerebral somatostatin is largely present in intrinsic cortical neurons, while cholinergic innervation is largely derived from the basal forebrain, these findings suggest that nonfamilial dysphasic dementia may be an example of a distinct class of dementia due to intrinsic cortical degeneration, with sparing of the basal forebrain.
两名中年男性被诊断患有非家族性综合征，他们表现为进行性失语，且无早期认知障碍迹象。在每个病例中，2年后才出现进行性功能衰退或行为障碍。影像学研究记录了左侧大脑不对称性萎缩，且呈进行性发展。语言功能衰退的结构具有独特性，与阿尔茨海默病不同。对其中一名患者进行的尸检病理研究记录了不对称性脑萎缩及非特异性组织病理学变化。生化研究显示胆碱乙酰转移酶活性的组织水平正常，但生长抑素样免疫反应性降低。由于大脑生长抑素主要存在于皮质固有神经元中，而胆碱能神经支配主要来自基底前脑，这些发现表明非家族性失语性痴呆可能是一种由于皮质固有神经元变性导致的独特类型痴呆的实例，基底前脑未受影响。
