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 2 2 % ,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 Objective To study the histopathological changes of Huntington chorea and to observe the distribution of inclusion bodies of ubiquitin-positive dystrophic neurons and neurons in different parts of the brain and to explore the pathological basis of dementia and mental disorders. Method The first symptom was a 49-year-old male patient presenting with progressive dementia, dancing and mental disorders, who died 17 years after illness. Fifteen of the 5 consecutive generations of the family developed similar clinical manifestations. The first symptom of death after head anatomy and histological examination of the different regions of the cerebral cortex and basal ganglia tau protein and ubiquitin immunohistochemical staining, analysis of different regions of the pathological changes. Results The pathological changes of brain were characterized by significant atrophy of new striatum, loss of nerve cells and glial cell proliferation. Ubiquitin-positive dystrophic neurites and neuronal nuclear inclusions mainly in the cortex of the Ⅲ ~ Ⅴ layer, neurons in the umbilical nucleus within the prefrontal cortex in up to 22%, followed by the parietal lobe 7%, occipital 3%, temporal lobe 1% and cingulate gyral 1%. Corresponding denaturation of the nucleus changes, inclusions do not contain tau protein. Ubiquitin-positive dystrophic neurons in the prefrontal cortex of the brain most, each low power field of 5 or more, followed by the parietal lobe, temporal lobe and cingulate cortex, low power field in between 1 to 5 . The hippocampus and striatum only occasionally malnutrition neurites, there is no neuronal nuclear inclusions. Conclusions The distribution of ubiquitin-positive neuron nuclear inclusions and dystrophic neurites in different regions of the brain is quite different in the cerebral cortex. Because the frontal pole also has obvious pathological changes, the wisdom of the disease