Knopman D S
Department of Neurology, University of Minnesota Hospitals, Minneapolis 55455.
Dementia. 1993 May-Aug;4(3-4):132-6. doi: 10.1159/000107354.
The group of progressive dementing illnesses that lack distinctive histologic features includes at least four variants: a cortical type, a thalamostriate type, a motor neuronopathy type and a leukogliotic type. While the clinical presentation of some cases is that of anterograde amnesia, progressive aphasia and dysexecutive syndrome are the most common initial symptom complexes. A large number of reported cases are familial, although no abnormal gene has been identified. Pathologically, these illnesses are defined by cortical, hippocampal, striatal, medial thalamic, nigral and motor nuclei cell dropout and astrogliosis. In some cases, white matter gliosis is striking. Identification of specific histological or molecular markers of at least some of these conditions will greatly advance our understanding of these specific conditions as well as dementing illnesses in general.
皮质型、丘脑纹状体型、运动神经元病型和白质胶质增生型。虽然有些病例的临床表现为顺行性遗忘，但进行性失语和执行功能障碍综合征是最常见的初始症状组合。尽管尚未鉴定出异常基因，但大量报告病例为家族性。病理上，这些疾病的定义是皮质、海马、纹状体、丘脑内侧、黑质和运动核细胞脱失以及星形胶质细胞增生。在某些情况下，白质胶质增生很明显。确定至少其中一些病症的特定组织学或分子标志物将极大地推进我们对这些特定病症以及一般痴呆疾病的理解。
