Subcortical neurodegeneration in chorea: Similarities and differences between chorea-acanthocytosis and Huntington's disease
Subcortical neurodegeneration in chorea: Similarities and differences between chorea-acanthocytosis and Huntington's disease
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舞蹈病的皮层下神经变性：舞蹈病棘红细胞增多症和亨廷顿病之间的异同
DOI：
10.1016/j.parkreldis.2018.01.009
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发表时间：
2018-04-01
影响因子：
4.1
通讯作者：
Danek, Adrian
中科院分区：
文献类型：
--
作者：
Liu, Jia;Heinsen, Helmut;Danek, Adrian
简介：舞蹈棘红细胞增多症（ChAc）和亨廷顿氏病（HD）是神经退行性疾病，尽管它们具有不同的遗传病因，但它们具有共同的临床和神经病理学特征。方法：为了比较这些神经病理学特征，分析了来自三名患有ChAc的受试者的连续的花青苷染色的脑切片，并与我们先前对八名HD病例的研究进行了比较，此外还分析了来自两名男性对照的三个半球。星形胶质细胞增生是更大的ChAc纹状体，相比，在HD中发现的，与纹状体神经胶质细胞总数和每纹状体神经元的神经胶质细胞的数量显着增加。纹状体星形胶质细胞最有可能来源于ChAc中的纹状体室管膜下层，其显示出大量增殖。丘脑中央核-束旁复合体与纹状体相连，并且在HD中比ChAc.Conclusion受到更严重的影响：HD和ChAc中观察到的选择性脆弱性和胶质增生的不同模式挑战了这两种疾病的发病机制的简单观点，具有相当相似的临床体征。星形胶质细胞在ChAc和HD中发挥的特殊作用显然需要更详细地阐明。(C)2018爱思唯尔有限公司版权所有
Introduction: Chorea-acanthocytosis (ChAc) and Huntington's disease (HD) are neurodegenerative conditions that share clinical and neuropathological features, despite their distinct genetic etiologies.Methods: In order to compare these neuropathologies, serial gallocyanin-stained brain sections from three subjects with ChAc were analyzed and compared with our previous studies of eight HD cases, in addition to three hemispheres from two male controls.Results: Astrogliosis was much greater in the ChAc striatum, as compared to that found in HD, with dramatic increase in total striatal glia numbers and the number of glia per striatal neuron. Striatal astrocytes are most likely derived from the striatal subependymal layer in ChAc, which showed massive proliferation. The thalamic centromedian-parafascicular complex is reciprocally connected to the striatum and is more heavily affected in HD than in ChAc.Conclusion: The distinct patterns of selective vulnerability and gliosis observed in HD and ChAc challenge simplistic views on the pathogenesis of these two diseases with rather similar clinical signs. The particular roles played by astroglia in ChAc and in HD clearly need to be elucidated in more detail. (C) 2018 Elsevier Ltd. All rights reserved.