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舞蹈病神经病理变化的形态计量学研究。

Morphometric studies of the neuropathological changes in choreatic diseases.

作者信息

Lange H, Thörner G, Hopf A, Schröder K F

出版信息

J Neurol Sci. 1976 Aug;28(4):401-25. doi: 10.1016/0022-510x(76)90114-3.

DOI:10.1016/0022-510x(76)90114-3
PMID:133209
Abstract

The striatum, pallidum and subthalamic nucleus were studied by combined morphometric methods in serial sections of 13 brains of normal adults and of 15 patients with choreatic diseases. In addition the volume of the hemispheres and of the cortex were measured. All data obtained were corrected by the shrinkage factor to represent fresh brain values. In Huntington's chorea the pallidum was more severely affected than is commonly appreciated. The average volume reduction was of the same degree (lateral-57%, medial-50%) as that of the striatum (-56%). The absolute number of nerve cells of the pallidum decreased in both segments by about 40%. The reduction of the volume and of the number of nerve cells was not reduced in the three subcortical nuclei studied. For the first time it has been shown that there is no increase in the absolute number of glial cells in the striatum. The increased numerical density of glial cells is caused by shrinkage. The loss of nerve cells of the pallidum and subthalamic nucleus is caused mainly by a primary process. Huntington's chorea is a multifocal process. Morphometric data do not suggest that subchorea is a variant of Huntington's chorea. Chorea minor is regarded as a multifocal process with varying affliction of the striatum, pallidum and subthalamic nucleus. An increase in the number of glial cells and, as a rule, a moderate loss of nerve cells were found in this disease.

摘要

采用联合形态测量方法,对13例正常成年人及15例舞蹈病患者大脑的连续切片中的纹状体、苍白球和丘脑底核进行了研究。此外,还测量了大脑半球和皮质的体积。所有获得的数据均通过收缩因子进行校正,以代表新鲜脑值。在亨廷顿舞蹈病中,苍白球受影响的程度比通常认为的更为严重。其平均体积减少程度(外侧 - 57%,内侧 - 50%)与纹状体(-56%)相同。苍白球两个节段的神经细胞绝对数量均减少了约40%。在所研究的三个皮质下核中,体积和神经细胞数量的减少并未减轻。首次发现纹状体中神经胶质细胞的绝对数量没有增加。神经胶质细胞数量密度的增加是由萎缩引起的。苍白球和丘脑底核神经细胞的丢失主要是由一个原发性过程导致的。亨廷顿舞蹈病是一个多灶性过程。形态测量数据并不表明亚舞蹈病是亨廷顿舞蹈病的一种变体。小舞蹈病被认为是一个多灶性过程,纹状体、苍白球和丘脑底核受累程度各异。在这种疾病中发现神经胶质细胞数量增加,且通常有适度的神经细胞丢失。

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