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目的 探讨散发性多系统萎缩可能的病理机制。方法 对 1 2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 1 2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 2 5nm的嗜银性颗粒纤维丝是胶质细胞包涵体的主要成分 ,并且这些物质被抗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 -共核蛋白免疫反应性。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是?
Objective To explore the possible pathological mechanism of sporadic multiple system atrophy. Methods We performed hematoxylin and eosin (HE), Luxol Kenloblue - Tar purple, Holzer, s glial fibrillary staining and modified Gallyas Braak’s (GB) staining in 12 autopsy cases with multiple system atrophy and 4 normal controls. Silver staining, anti - α - synuclein and anti - ubiquitin antibody immunohistochemical staining. Immunoreactivity and ultrastructural features of glial inclusion bodies were studied. In case 1, podocytes with abundant glial inclusion bodies were examined by conventional electron microscopy, Gallyas Braak’s (GB) silver staining and immunoelectron microscopy. Results There were glioma inclusion bodies in 12 patients with multiple system atrophy. All ubiquitinated glial inclusions were immunoreactive with α - synuclein. However, the density of α-synuclein-positive gliocyte inclusions varies from patient to patient, and the density of glial inclusion bodies is not associated with the patient’s age, gender and multiple system atrophy subtype. The ultrastructural features show that the anilophilic granular fiber filaments having a diameter of about 25 nm are the major components of glial inclusion bodies and that these substances are selectively labeled by anti-α-synuclein antibodies. Glial cell inclusion bodies and α-synuclein immunoreactivity were not found in normal brain tissue. Conclusion Glial cell inclusion bodies are pathological changes that are characteristic of patients with sporadic multiple system atrophy. The deposition of α-synuclein in glial inclusion bodies may occur early in multiple system atrophy. Gliocyte inclusion body α-synuclein selective positive labeled abnormal microtubules may be?