Glial involvement in diffuse Lewy body disease

Glial involvement in diffuse Lewy body disease
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DOI:
10.1007/s00401-002-0622-9
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发表时间:
2003-02
影响因子:
12.7
通讯作者:
S. Terada;H. Ishizu;O. Yokota;K. Tsuchiya;H. Nakashima;T. Ishihara;Daisuke Fujita;K. Uéda;K. Ikeda;S. Kuroda
S. Terada;H. Ishizu;O. Yokota;K. Tsuchiya;H. Nakashima;T. Ishihara;Daisuke Fujita;K. Uéda;K. Ikeda;S. Kuroda
中科院分区:
医学1区
文献类型:
--
作者:
S. Terada;H. Ishizu;O. Yokota;K. Tsuchiya;H. Nakashima;T. Ishihara;Daisuke Fujita;K. Uéda;K. Ikeda;S. Kuroda

文献摘要

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弥漫性路易体病(DLBD)的特征在于在皮质、皮质下和脑干结构的神经元和神经突中存在路易体(LB)。最近发现α-突触核蛋白(α-synuclein,αS)是LB的核心成分。在DLBD中,αS在神经元和胶质细胞中的异常积累已有报道,但对DLBD中胶质细胞病变的研究有限。我们详细研究了8例DLBD患者的胶质病变的组成和分布,并报告了胶质病变的发病机制。α S阳性的神经元胞质包涵体(NI)、神经纤维丝(NT)和卷曲体(CB)显示相似的免疫染色特征。未经预处理,所有抗αS抗体均可检出NI、NT和CB。星形胶质细胞(SLA)的免疫组化谱与NI、NT和CB的免疫组化谱有很大不同。一些SLA在未经预处理的情况下被针对αS的非A β组分部分的抗体染色,但甲酸预处理显著增强SLA的免疫反应性。8例DLBD患者脑内均发现SLA和CB。脑干内SLA少见,但在100倍放大率下,大多数病例的颞叶皮层每个视野内有数百个SLA,而CB则在大脑皮层和脑干内弥漫性分布,与NI相似。提示SLA的发病机制不同于NI和CB。
Diffuse Lewy body disease (DLBD) is characterized by the presence of Lewy bodies (LB) in the neurons and neurites of cortical, subcortical, and brain stem structures. Recently, α-synuclein (αS) has been found to be a central constituent of LB. In DLBD, abnormal accumulation of αS has been reported in both neurons and glia, but studies on glial lesions in DLBD have been limited. We examined in detail the constituents and distribution of glial lesions in eight patients with DLBD and report the pathogenesis of the glial lesions. αS-positive neuronal cytoplasmic inclusions (NI), neuropil threads (NT), and coiled bodies (CB) showed similar immunostaining profiles. Without pretreatment, NI, NT, and CB were detected by all antibodies against αS. The immunostaining profile of star-like astrocytes (SLA) was quite different from those of NI, NT, and CB. A few SLA were stained by an antibody against the non-Aβ component portion of αS without pretreatment, but formic acid pretreatment dramatically enhanced SLA immunoreactivity. SLA and CB were found in all eight brains with DLBD. SLA were scarce in the brain stem, but there were hundreds of SLA per visual field at ×100 magnification in the temporal cortex of most cases, while CB were found diffusely in both the cerebral cortex and brain stem, similar to NI. This suggests that the pathogenesis of SLA is different from those of NI and CB.