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[少突胶质细胞包涵体,多系统萎缩的一个标志物]

[Oligodendroglial inclusions, a marker of multisystemic atrophies].

作者信息

Costa C, Duyckaerts C, Cervera P, Hauw J J

机构信息

Service de Neurologie, Hosp. Sta Maria, Lisbonne.

出版信息

Rev Neurol (Paris). 1992;148(4):274-80.

PMID:1332174
Abstract

Nine cases of multiple system atrophy and 1 case of autosomal dominant olivopontocerebellar atrophy (neuropathological diagnosis) were retrospectively examined for the presence of oligodendroglial inclusions. Clinical diagnosis in the first 9 cases had been: olivopontocerebellar atrophy (3 cases), atypical Parkinson's disease (2 cases), Shy-Drager syndrome (2 cases) and multiple system atrophy (1 case); one of the patients could not be included in any of the above mentioned groups. The oligodendroglial inclusions were argyrophilic and located in the cytoplasm around the nucleus. They were revealed by Bodian's method in all cases of multiple system atrophy. They were not found in the case of autosomal dominant olivopontocerebellar atrophy. They were labelled by anti-ubiquitin antibodies, and were negative with anti-tau antibodies. At electron microscopy, they consisted of rectilinear profiles coated with a fuzzy material (diameter: 20-33 nm); this aspect was compatible with microtubules. Oligodendroglial inclusions were prominent in regions selectively vulnerable in multiple system atrophy (tegmentum pontis, putamen, inferior olives, substantia nigra and cerebellar white matter), even in those areas where neuronal loss or fascicular atrophy were minimal or absent. They were also observed in regions considered to be spared in multiple system atrophy, such as the motor cortex and the corpus callosum. Argyrophilic oligodendroglial inclusions are an early and specific marker of multiple system atrophy. It is suggested that autosomal dominant olivopontocerebellar atrophy lacking oligodendroglial inclusions does not belong to multiple system atrophy.

摘要

对9例多系统萎缩患者和1例常染色体显性遗传性橄榄体脑桥小脑萎缩患者(经神经病理学诊断)进行回顾性研究,以检查是否存在少突胶质细胞包涵体。前9例患者的临床诊断分别为:橄榄体脑桥小脑萎缩(3例)、非典型帕金森病(2例)、夏伊-德雷格综合征(2例)和多系统萎缩(1例);其中1例患者不属于上述任何一组。少突胶质细胞包涵体嗜银,位于细胞核周围的细胞质中。在所有多系统萎缩病例中,通过博迪安氏法均发现了这些包涵体。在常染色体显性遗传性橄榄体脑桥小脑萎缩病例中未发现。它们被抗泛素抗体标记,而抗tau抗体检测为阴性。在电子显微镜下,它们由包被着模糊物质的直线状结构组成(直径:20 - 33纳米);这种形态与微管相符。少突胶质细胞包涵体在多系统萎缩的选择性易损区域(脑桥被盖、壳核、下橄榄核、黑质和小脑白质)很突出,即使在神经元丢失或束状萎缩极少或不存在的区域也是如此。在多系统萎缩被认为 spared 的区域,如运动皮层和胼胝体中也观察到了它们。嗜银性少突胶质细胞包涵体是多系统萎缩的一种早期特异性标志物。提示缺乏少突胶质细胞包涵体的常染色体显性遗传性橄榄体脑桥小脑萎缩不属于多系统萎缩。 注:原文中“spared”疑似拼写错误,推测应为“spared”,结合语境这里可能是想说“未受影响的、 spared 的区域”,但由于不确定,所以保留原文翻译。你可根据实际情况修正。

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