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唐氏综合征中的神经原纤维缠结、颗粒空泡变性及神经元丢失:与阿尔茨海默病痴呆的定量比较

Neurofibrillary tangles, granulovacuolar degeneration, and neuron loss in Down Syndrome: quantitative comparison with Alzheimer dementia.

作者信息

Ball M J, Nuttall K

出版信息

Ann Neurol. 1980 May;7(5):462-5. doi: 10.1002/ana.410070512.

DOI:10.1002/ana.410070512
PMID:6446875
Abstract

The degree of neurofibrillary tangle formation, granulovascuolar degeneration, and nerve cell loss was quantified in serial sections of the hippocampal formation from the brains of 5 adults dying with Down syndrome (monogolism). These morphometric results were compared with values obtained from a series of mentally normal elderly patients reported previously. Granulovacuolar change appeared to be related as much to patient age as to the existence of monogolism. By contrast, the number of Alzheimer neurofibrillary tangles and loss of pyramidal neurons from the hippocampus exceeded levels found in considerably older subjects and fell into the same range of severity as was noted in 8 patients with Alzheimer dementia. The neuron population in the hippocampus of Down syndrome patients may be only half the expected normal number for their decade. These quantitative similarities stimulate the search for a common pathogenetic mechanism underlying cerebral changes in monogolism and senile dementia of the Alzheimer type.

摘要

对5名死于唐氏综合征(蒙古症)的成年患者大脑海马结构的连续切片进行神经原纤维缠结形成、颗粒空泡变性和神经细胞丢失程度的定量分析。将这些形态学测量结果与先前报道的一系列精神正常的老年患者的值进行比较。颗粒空泡变化似乎与患者年龄以及蒙古症的存在都密切相关。相比之下,唐氏综合征患者海马中阿尔茨海默神经原纤维缠结的数量以及锥体神经元的丢失超过了年龄大得多的受试者中发现的水平,严重程度与8例阿尔茨海默病痴呆患者中观察到的范围相同。唐氏综合征患者海马中的神经元数量可能仅为其年龄段预期正常数量的一半。这些定量上的相似性促使人们寻找蒙古症和阿尔茨海默型老年性痴呆脑部变化潜在的共同致病机制。

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