泛素阳性包涵体在运动神经元病的表达

Appearance of ubiquitin positive inclusions in central nervous system of motor neuron disease

目的 探讨泛素阳性包涵体在运动神经元病 (MND)中枢神经系统的分布、成分及与神经细胞变性的关系。方法 对 5例MND和 9例非神经系统变性病对照患者脑和脊髓进行苏木素 伊红、快蓝和Bodian常规染色 ,抗 泛素、抗 tau蛋白、抗 胶质纤维酸性蛋白和抗 神经丝蛋白抗体免疫组化染色。结果 5例MND抗泛素免疫组化染色在其中 4例前角细胞胞质内发现单线 线团样包涵体、透明包涵体、路易体样包涵体和不规则包涵体 ,相应前角细胞存在轻中度变性。 3例海马颗粒细胞内出现泛素阳性包涵体。 1例患者脊髓前角细胞内少数泛素阳性包涵体抗神经丝蛋白染色也阳性 ,各种泛素阳性包涵体抗tau蛋白及胶质纤维酸性蛋白染色均为阴性。结论 脊髓前角细胞胞质内的泛素阳性包涵体是MND较为特异的免疫病理改变 ,不同类型包涵体之间可能存在一定的演变规律 ,与相应神经细胞的变性相关。少数泛素阳性包涵体含有神经丝蛋白成分。海马颗粒细胞内出现泛素阳性包涵体提示此病同时累及非运动系统 。

Objective To study the distribution,morphological characteristics and composition of ubiquitin positive inclusions in motor neuron disease (MND) To determine the relationship between neuronal degeneration and presence of the inclusions. Methods 5 MND patients and 9 controls were studied Immunohistochemical staining was carried out on sections of brains and spinal cords from the two groups Antibodies against ubiquitin, tau, neurofilament and glial fibrillary acidic protein (GFAP) were used as primary antibodies. Results Various forms of intracytoplasmic ubiquitin positive inclusions presented in anterior horn cells in 4/5 patients, in which Bunina bodies, linear bodies, skein like inclusions, hyaline inclusions, lewy body like or irregular inclusions were found The related neurons appeared in various degenerations from mild lesions to the formation of ghost cells The inclusions also presented in hippocampal granular cells in 3/5 MND patients In one MND patient, neurofilament appeared in a few ubiquitin positive inclusions in spinal anterior horn cells. Conclusions Appearance of intracytoplasmic ubiquitin positive inclusions in anterior horn cells showed an immunopathological feature in MND, which might be related to neuronal degeneration There might exist a developmental process from linear bodies to irregular inclusions at the end stage Neurofilament appeared only in a few ubiquitin positive inclusions Appearance of ubiquitin positive inclusions in hippocampal granular cells may imply that the non motor areas maybe also involved in MND, leading to mental abnormalities