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[一例伴有许多路易小体的多系统萎缩尸检病例——纹状体黑质变性、橄榄脑桥小脑萎缩及脊髓自主神经核受累]

[An autopsy case of multiple system atrophy with many Lewy bodies--striatonigral degeneration, olivo-ponto-cerebellar atrophy and autonomic nerve nucleus involvement in the spinal cord].

作者信息

Morioka E, Kuroda S, Kuyama K, Otsuki S, Hosokawa K, Namba R

出版信息

No To Shinkei. 1987 Apr;39(4):361-6.

PMID:3036191
Abstract

A 72 years old man developed slowness of the motion and orthostatic hypotension at the age of 69. Neurological examination showed slight finger tremor, rigidity of extremities, bradykinesia, and marked orthostatic hypotension. The illness progressed steadily and the patient died of pneumonia. At autopsy brain weighed 1220 g. Grossly the putamen was bilaterally shrunken, the color of the substantia nigra and locus ceruleus became pale. Base of the pons and the cerebellum were atrophic. Microscopically the most remarkable change was seen in the striato-nigral system. In the putamen, there were severe loss of small neurons and intense gliosis and brownish pigments were observed in the neuropil and within some of the astrocytes. There found neuronal loss and gliosis in the substantia nigra. A few Lewy bodies were seen in the substantia nigra. In the cerebellum there were slight loss of Purkinje cell and many torpedos were seen. There were demyelination and fibrirally gliosis in the cerebellar white matter except the hilus of dentate nucleus. The transverse pontocerebellar fibers were degenerated and fibrirally gliosis was seen there. The inferior olivary nuclei showed neuronal loss and astrocytosis. But the degeneration of the olivo-ponto-cerebellar system in this case was not so severe as the typical case of OPCA. In the spinal cord there was depletion of nerve cells in the intermediolateral nuclei and Onufrowitz nuclei. Slight neuronal loss and many spheroids were observed in the anterior horns and there was demyelination in the corticospinal tracts.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

一名72岁男性在69岁时出现运动迟缓及体位性低血压。神经系统检查发现有轻微手指震颤、肢体强直、运动徐缓以及明显的体位性低血压。病情稳步进展,患者死于肺炎。尸检时脑重1220克。大体上,双侧壳核萎缩,黑质和蓝斑颜色变浅。脑桥底部和小脑萎缩。显微镜下,纹状体 - 黑质系统出现最显著的变化。在壳核,小神经元严重缺失,胶质细胞增生明显,在神经纤维网和一些星形胶质细胞内可见褐色色素。黑质存在神经元丢失和胶质细胞增生。在黑质可见少量路易小体。小脑有轻度浦肯野细胞丢失,可见许多鱼雷样结构。除齿状核 hilus 外,小脑白质有脱髓鞘和纤维性胶质细胞增生。脑桥横纤维变性,可见纤维性胶质细胞增生。下橄榄核有神经元丢失和星形细胞增多。但该病例中橄榄 - 脑桥 - 小脑系统的变性程度不如典型的橄榄脑桥小脑萎缩(OPCA)病例严重。脊髓中间外侧核和Onufrowitz核神经细胞减少。前角有轻度神经元丢失和许多小球体,皮质脊髓束有脱髓鞘改变。(摘要截短于250字)

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