摘要目的探计散发性多系统萎缩可能的病理机制.方法对12例多系统萎缩的尸检病例和4例正常对照例的脑和脊髓进行苏木素-伊红、luxol坚劳蓝焦油紫、Holzer,s胶质纤维染色和改良型Gallyas-Braak's(GB)银浸润染色法,抗α-共核蛋白和抗泛素抗体免疫组织化学染色.对胶质细胞包涵体的免疫反应性和超微结构特征进行了研究.其中对病例1,含有丰富的胶质细胞包涵体的桥脑白质进行常规电镜,Gallyas-Braak's(GB)银浸润法电镜和免疫电镜检查.结果 12例多系统萎缩患者均发现有胶质细胞包涵体存在.所有泛素化的胶质细胞包涵体对α-共核蛋白的免疫反应呈现阳性.然而,α-共核蛋白阳性的胶质细胞包涵体的密度在患者之间有差异,并且胶质细胞包涵体的密度与患者的年龄、性别和多系统萎缩的亚型无关.超微结构特征显示具有直径约25nm的嗜银性颗粒纤维丝是胶质细胞涵体的主要成,并且这些特质被抗α-共核蛋白抗体选择性标记.在正常对照例的脑组织中没有发现胶质细胞包涵体和α-共核蛋白免疫应性.哌结论胶质细胞包涵体是散发性多系统萎缩患者特有的病理变化.在胶质细胞包涵体内的α-共核蛋白沉积可能发生在多系统萎缩的早期.胶质细胞包涵体内α-共核蛋白选择性标记阳性的异常微管可能多系统萎缩的重要病理机制.

abstractsObjective To understand the possible pathogenesis of sporadic multiple system atrophy (MSA).Methods The immunoreactivity and ultrastructural features of glial cytoplasmic inclusions (GCIs) in 12autopsy patients with MSA and 4 normal control groups were studied. All regional sections from eachsubject were evaluated with HE staining, KIuver-Barrera (KB), Holzer's, modified Gallyas-Braak's (GB)methods and immunohistochemical staining with α-synuclein and ubiquitin antibodies. Pontine white matterwith abundant GCls from case 1 was examined, using conventional electron microscopy, Gallyas-Braak'selectron microscopy and immunoelectron microscopy.Results The presence of GCls as constantly demonstrated in all MSA patients. Strong α-synucleinimmunoreactivity was observed in all of the ubiquitinated GCls. However, the density of α-synuclein positiveGCIs differed from case to case, and there was no relationship between the density of GCls and age, sex,or MSA subtype. Ultrastructural features indicated that argyrophilic granule-associated filaments of about 25nm in diameter were the predominant constituents of GCls, and the anti α-synuclein antibody selectivelylabeled in these filaments. No GCls and α-synuclein immunoreaction were found in control brain tissues.Conclusions GCI was a pathognomonic change in sporadic MSA patients. Accumulation of α-synuclein inGCIs may occur during the early stags of MSA. Seletcive α-synuclein positive abnormal microtubules inGCls therefore play an important role in the pathogenesis of MSA.