Multiple System Atrophy
Multiple System Atrophy
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DOI：
10.1111/j.1750-3639.1997.tb01034.x
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发表时间：
1997-10
期刊：
影响因子：
6.4
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中科院分区：
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--
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1989年，我们首次描述了多系统萎缩（MSA）中的胶质细胞质和包涵体（GCI）(1)。在此之前，与其他神经退行性疾病不同，MSA缺乏任何病理病变，神经病理学诊断基于非特异性的，通常难以捉摸的神经元丢失，髓磷脂苍白和受影响区域的星形细胞增多。在ori，报告和随后的两篇论文中，我们全面描述了gci（2,3）。这些包裹体是亲银性的：Gallyas(4)首先在银浸染中观察到它们，以证明阿尔茨海默氏型神经原纤维的改变，尽管它们的结构有时表面上类似于神经原纤维缠结，但它们的细胞定位、尺寸、超微结构、免疫细胞化学谱和区域分布都不同。我们最初的观察是基于对11例MSA的全面神经病理学检查，已经被其他研究者证实，这些发现最近被回顾（5,6）。gci的发现引起了人们对神经胶质病理学，特别是神经胶质包涵体的极大兴趣。严格意义上的神经胶质细胞、星形胶质细胞和少突胶质细胞在神经退行性疾病中的研究相对较少，其中的研究并非不合理地集中在神经元病理学上。星形胶质细胞的变化，包括肥大和增生，长期以来被认为是星形胶质细胞对神经元损伤和丧失的一种典型反应。然而，对少突胶质细胞的研究更少。目前对se58af、英国神经胶质病理学的强烈兴趣导致了各种神经退行性疾病（包括阿尔茨海默病、皮克病、MSA、皮质基底变性（CBD）和进行性核上性麻痹（PSP））中少突胶质细胞和星形胶质细胞的广泛细胞改变的描述（回顾见6和7）。然而，这些最近的观察提出了一个基本问题。哪些不同的胶质包涵体对其发生的特定疾病具有实践重要性和理论意义？实际的考虑显然是诊断和重要的执业神经病理学家。这些胶质包涵体是特定疾病的病理特征还是没有任何诊断价值的非特异性？从理论的角度来看，这些细胞内病变在疾病发病机制中的作用必须进行批判性评估。它们是导致神经变性的细胞和分子机制的组成部分吗？本综述的目的是通过定义其诊断和生物学意义，并将其与最近观察到的其他胶质细胞变化进行比较，来澄清与MSA中gci相关的这些问题。
In 1989 we were the fust to describe glial cytoplast& inclusions (GCI) in multiple system atrophy (MSA) (1). Until then MSA, unlike other neurodegenerative disorders, lacked any pathognomonic lesion, and the neuropathological diagnosis was based on non-specific, and often elusive changes of neuronal loss, myelin pallor and astrocytosis of the affected areas. In the ori, report and two subsequent papers we have comprehensively characterized GCIs (2,3). These inclusions are argyrophilic: they were first observed in silver impregnation developed by Gallyas (4) to demonstrate Alzheimer-type neurofibrillary changes, and although their configuration sometimes superficially resembled neurofibrillary tangles, their cellular localization, dimensions, ultrastructure, immunocytochemical profile and regional distribution were all different. Our original observations, based on a comprehensive neuropathological examination of 11 cases of MSA, have been conf m e d by other investigators and these findings have been recently reviewed (5,6). The discovery of GCIs has initiated considerable interest in glial pathology, particularly in glial inclusions. Neuroglial cells, astrocytes and oligodendrocytes in strict sense of definition, have been relatively little investigated in neurodegenerative diseases in which research, not unreasonably, has focused on neuronal pathology. Astrocytic changes, including hypertrophy and hyperplasia, have, for long, been recognised as a stereotypic response of astrocytes to neuronal damage and loss. However, oligodendrocytes have remained even less investigated. The current intense interest in SE5 8AF, UK glial pathology hiis resulted in the description of a wide range of cellular alterations both in oligodendrocytes and astrocytes in various neurodegenerative diseases, including Alzheimer's disease, Pick's disease, MSA, corticobasal deigeneration (CBD) and progressive supranuclear palsy (PSP) (for review see 6 and 7). These recent observations, however, raise a fundamental question. Which of the different glial inclusions have both practical importance and theoretical significance for the particular disorder in which they occur? The practical consideration is clearly diagnostic and important for the practising neuropathologist. Are any of these glial inclusions pathognomonic of a particular disease or are they non-specitic without any diagnostic value? From a theoretical point, the role of these intracellular lesions in the pathogenesis of the disease has to be critically evaluated. Are they an integral part of the cellular and molecular mechaniscis which result in neurodegeneration? The aim of this review is to clarify these issues in relation to GCIs in MSA, by defining their diagnostic and biological significance and by comparing them with other, more recently observed glial changes.