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学科主题神经病学
Huntington舞蹈病大脑免疫病理改变及其和痴呆精神异常的关系
其他题名Pathological and immunopathological changes in Chorea Huntington and their relationship to dementia and psychiatric symptoms
袁云; 陈清棠; 高唯一; 张巍; 戴三冬; 高素荣
关键词杭廷顿 遍在蛋白质 痴呆
刊名中华神经科杂志
2001
DOI10.3760/j.issn:1006-7876.2001.01.005
34期:1页:12-15
收录类别中国科技核心期刊 ; CSCD
文章类型Journal Article
摘要目的 研究Huntington舞蹈病的组织病理改变特点,观察泛素阳性营养不良性神经突起和神经细胞核内包涵体在大脑不同部位的分布规律,探讨痴呆和精神异常的病理基础。方法 先症者为一49岁的男性病人,表现为进行性痴呆、舞蹈和精神异常,于病后17年死亡。家族中连续5代人中15例出现类似临床表现。先症者死亡后进行头部解剖和组织学检查,对大脑皮层和基底节不同区域进行tau蛋白和泛素免疫组织化学染色,分析不同区域的病理改变规律。结果 脑病理改变特点为新纹状体显著萎缩,神经细胞脱失伴胶质细胞增生。泛素阳性的营养不良性神经突起和神经细胞核内包涵体主要出现在大脑皮层的Ⅲ~Ⅴ层,神经细胞核内泛素阳性包涵体在大脑额叶前区皮层达22%,依次为顶叶7%、枕叶3%、颞叶1%和扣带回1%。相应细胞核出现变性改变,包涵体不含有tau蛋白。泛素阳性营养不良性神经突起在大脑额叶前区皮层最多,每低倍视野达5个以上,其次为顶叶、颞叶和扣带回的皮层,低倍视野在1~5个之间。海马和纹状体仅偶见营养不良性神经突起,没有神经细胞核内包涵体。结论 大脑皮层出现泛素阳性神经细胞核内包涵体和营养不良神经突起在大脑不同区域的分布存在很大的差异,由于额极也存在明显的病理改变,所以此病的智能减退属于额叶皮层-皮层下性痴呆。额叶、颞叶和扣带回皮层出现神经细胞核内包涵体或营养不良性神经突起可能是精神异常的病理学基础。 Objective To examine the distribution of ubiquitin positivestructures in brain of Chorea Huntington and pathological basis of cognitive and psychiatric symptoms in a large Northern-Chinese kindred.Methods A proband patient experienced a chronic onset of chorea with dementia at the age of 30 years. A few years later the patient developed psychiatric symptoms. He died at the age of 47 years. In his family there were 15 members from 5 generations who showed gradual dementia,progressive motor disability and psychiatric disturbance. Postmortem histological examination and immunohistochemical staining with antibodies against ubiquitin and Tau were performed in proband case. The distribution of ubiquitin positive dystrophic neurites and neuronal intranuclear inclusions were investigated. Results Morphologically,it was characterized by marked atrophy of bilateral striatum with loss of neurons and mild proliferation of astrocytes. Immunohistochemical study confirmed frequent appearance of ubiquitin positive neuronal intranuclear inclusions and dystrophic neurites in layer Ⅲ-V of cerebral cortex. The neuronal intranuclear ubiquitin positive inclusions usually associated with nuclear degeneration were more frequently found in frontal (22%) and parietal cortex (7%),less frequently in occipital (3%),temporal (1%) and limbic system (1%),but not found in hypocampus and striatum. Ubiquitin positive neurites distributed frequently in frontal cortex (over 5 per low field),less frequently in temporal and parietal cortex as well as limbic system (1-5 per low field),but occasionally in hypocampus and striatum. Conclusions Our study confirmed that the frequency of ubiquitin positive neuronal intranuclear inclusions and dystrophic neurites were varied markedly in different area of cerebral cortex. Owing to the marked neuropathological changes in frontal cortex,the cognitive symptoms should be considered as a frontal -subcortical dementia. The ubiquitin positive dystrophic neurites and neuronal intranuclear inclusions in frontal and temporal cortex as well as in limbic system might be a pathological basis in the development of psychiatric symptoms.
语种中文
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文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/41435
Collection北京大学第一临床医学院_神经内科
作者单位100034 北京大学第一医院神经内科
Recommended Citation
GB/T 7714
袁云,陈清棠,高唯一,等. Huntington舞蹈病大脑免疫病理改变及其和痴呆精神异常的关系[J]. 中华神经科杂志,2001,34(1):12-15.
APA 袁云,陈清棠,高唯一,张巍,戴三冬,&高素荣.(2001).Huntington舞蹈病大脑免疫病理改变及其和痴呆精神异常的关系.中华神经科杂志,34(1),12-15.
MLA 袁云,et al."Huntington舞蹈病大脑免疫病理改变及其和痴呆精神异常的关系".中华神经科杂志 34.1(2001):12-15.
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