摘要
目的 为探讨Lewy小体病的病理特点 ,报道 1例 80岁 ,女性 ,以进行性痴呆、肌肉强直为特征 ,病理学上确认为Lewy小体病的国人尸体解剖病例。方法 临床诊断为老年性痴呆的尸检脑组织标本 ,应用HE染色、神经系统特殊染色、免疫组织化学染色 (ABC法 )和电子显微镜对脑的病理形态学、Lewy小体的分布和α 共核蛋白异常积聚进行了研究。结果 脑病理变化特征 :①中脑黑质、桥脑蓝斑和延髓迷走神经背核大量神经细胞脱落 ,星形胶质细胞增生 ,残存的神经细胞胞浆内有典型的脑干型Lewy小体 ;②大脑皮质 ,尤其在扣带回、岛叶、海马、海马旁回、内嗅叶皮质和杏仁核等处有大量皮质型Lewy小体存在 ;③海马CA2 / 3、海马旁回、内嗅叶皮质出现局限性空泡样变性和大量Lewy突起苔丝变性 ;④所有Lewy小体对α 共核蛋白呈强阳性表达 ;⑤无阿尔茨海默病样老年斑 ,神经原纤维缠结等病理变化。结论 Lewy小体病是一组独立的中枢神经系统变性疾病 ,其病理特征与阿尔茨海默病的病理变化有明显差异。Lewy小体和Lewy突起苔丝变性对泛素化蛋白呈阳性表达。α 共核蛋白可能是构成Lewy小体的主要成分。本例属单纯型弥漫性Lewy小体病。
Objective To investigate the pathological features of Lewy body disease. Methods An autopsy case of an 80 year old Chinese woman suffering from Lewy body disease was studied The patient had progressive dementia and myotonia for three years and was suspected with Alzheimer dementia clinically Brain tissue was analyzed neuropathologically by using HE staining and specific staining for nervous system, immunohistochemical staining for ubiquitin and by using α synuclein and electronic microscopy. Results Neuropathological examination revealed the most characteristic features as follows: (1) The number of melanin pigmented neurons were markedly decreased in the substantia nigra and locus ceruleus, and some typical brain stem type Lewy bodies in the residual neurons with moderate gliosis were found (2) There appeared numerous cortical type Lewy bodies in deep layers of the cerebral cortex particularly in the cingulate gyrus, hippocampus, parahippocampal gyrus, transentorhinal contex, amygdala and insula (3) There were selective spongiform changes and many Lewy related neurites in positive ubiquitin immunohistochemical staining on the CA2/3 sectors, parahippocampal gyrus and transentorhinal cortex (4) A strong positive α synuclein immunoreactivity was found in all Lewy bodies and Lewy related neurites (5) Alzheimer disease related changes such as senile plaques and neurofibrillary tangles were scarcely found only in hippocampus and parahippocampal gyrus. Conclusion Lewy body disease is a new group of degenerative disease of the central nervous system α synuclein may play an important role in the degeneration of neurons and their neurites may be a major component of Lewy bodies in Lewy body disease It is considered that the present case should be a pure type of diffuse Lewy body disease
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2001年第4期214-217,共4页
Chinese Journal of Neurology
基金
国家教育部留学回国人员科研启动基金资助项目( 2 0 0 0 -367号 )
