de la Monte S M, Hedley-Whyte E T
Charles S. Kubik Laboratory of Neuropathology, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston 02114.
J Neuropathol Exp Neurol. 1990 Sep;49(5):509-20. doi: 10.1097/00005072-199009000-00006.
Morphometric analysis was used to measure cross-sectional areas of cerebral structures in middle-aged patients with Down's syndrome (N = 5) for comparison with data obtained from individuals with senile dementia of the Alzheimer type (N = 16) and neuropathologically normal controls (N = 14). Down's syndrome was distinguished from Alzheimer's disease by the 19% lower mean brain weight which was associated with more pronounced reductions in the areas of both cortex and white matter. However, the differences were most striking in the anterior frontal and anterior temporal regions where the effects of arrested neurodevelopment are grossly evident. In addition, in Down's syndrome the amygdala was significantly smaller than in Alzheimer's disease. In both Down's syndrome and Alzheimer's disease, shrinkage of the cortical ribbon was associated with abundant neuritic plaques and neurofibrillary tangles, while white matter atrophy was associated with histopathological evidence of axonal degeneration. These findings suggest that in Down's syndrome the reduction in volume in the posterior portion of the cerebrum relative to controls is largely due to acquired lesions of Alzheimer's disease, whereas anteriorly and within certain subcortical nuclei, the effects of both Alzheimer's disease and arrested neurodevelopment are manifested. Moreover, the finding of white matter lesions in Down's syndrome corroborates the notion that white matter degeneration is a fundamental component of the Alzheimer's disease process.
采用形态测量分析方法，对5例中年唐氏综合征患者的脑结构横截面积进行测量，以便与16例阿尔茨海默型老年性痴呆患者及14例神经病理学正常对照者的数据进行比较。唐氏综合征与阿尔茨海默病的区别在于，前者平均脑重量低19%，且皮质和白质区域的减少更为明显。然而，差异在额前叶和颞前叶区域最为显著，在这些区域，神经发育停滞的影响非常明显。此外，唐氏综合征患者的杏仁核明显小于阿尔茨海默病患者。在唐氏综合征和阿尔茨海默病中，皮质带的萎缩均与大量神经炎性斑块和神经原纤维缠结有关，而白质萎缩与轴突变性的组织病理学证据有关。这些发现表明，在唐氏综合征中，相对于对照组，大脑后部体积的减少主要是由于阿尔茨海默病的后天性病变所致，而在额叶前部和某些皮质下核内，阿尔茨海默病和神经发育停滞的影响均有体现。此外，唐氏综合征中白质病变的发现证实了白质变性是阿尔茨海默病进程基本组成部分的观点。
