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[一例脑炎后帕金森综合征尸检病例:与进行性核上性麻痹患者神经原纤维缠结的对比研究]

[An autopsy case of postencephalitic parkinsonism: investigation on neurofibrillary tangles in comparison with those in progressive supranuclear palsy].

作者信息

Mori H, Yoshimura M, Mizutani T, Kuzuhara S, Yamanouchi H, Shimada H, Tomonaga M

机构信息

Department of Clinical Pathology, Tokyo Metropolitan Institute of Gerontology, Japan.

出版信息

No To Shinkei. 1990 Jun;42(6):553-9.

PMID:2169789
Abstract

We report an autopsy case of postencephalitic parkinsonism (PEP). The distribution, histochemical, and immunohistochemical characteristics of neurofibrillary tangles (NFTs) in the case were studied and compared with NFTs in progressive supranuclear palsy (PSP). The patient was a 78-year-old woman who had suffered from "sleeping sickness" at age 27 and parkinsonism developed 13 years later. NFTs were found in the brainstem, basal ganglia, subthalamus, hypothalamus, thalamus and hippocampus in addition to marked degeneration of the substantia nigra. In the brainstem, NFTs were found not only in the amine-containing neurons but also in the pedunculopontine nucleus, which was recently reported to have cholinergic neurons. NFTs were immunostained by antibodies against tau and ubiquitin, both of which have been identified as antigenic components of NFTs in Alzheimer's disease and also those in PSP. Ultrastracturally, NFTs were composed of paired helical filaments. In serial sections with stained with Congo red and Bodian stains, all NFTs visible with Bodian stain in the brainstem of the case seemed to have Congo red birefringence which is produced by beta-sheet conformation of protein. In contrast, in three cases of PSP, less than half of NFTs seemed to have birefringence. These findings suggest that the protein components of NFTs in PEP and PSP are common to each other, but the conformation of the protein of some NFTs in PSP may be different from those in PPE.

摘要

我们报告了一例脑炎后帕金森综合征(PEP)的尸检病例。研究了该病例中神经原纤维缠结(NFTs)的分布、组织化学和免疫组织化学特征,并与进行性核上性麻痹(PSP)中的NFTs进行了比较。患者为一名78岁女性,27岁时曾患“昏睡病”,13年后出现帕金森综合征。除黑质明显变性外,在脑干、基底神经节、下丘脑、丘脑和海马中均发现了NFTs。在脑干中,不仅在含胺神经元中发现了NFTs,而且在最近报道含有胆碱能神经元的脚桥核中也发现了NFTs。NFTs用针对tau和泛素的抗体进行免疫染色,这两种抗体已被确定为阿尔茨海默病以及PSP中NFTs的抗原成分。超微结构上,NFTs由双螺旋丝组成。在用刚果红和博迪安染色的连续切片中,该病例脑干中所有能用博迪安染色看到的NFTs似乎都有刚果红双折射,这是由蛋白质的β折叠构象产生的。相比之下,在3例PSP病例中,不到一半的NFTs似乎有双折射。这些发现表明,PEP和PSP中NFTs的蛋白质成分彼此相同,但PSP中一些NFTs的蛋白质构象可能与PEP中的不同。

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