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[一例大脑皮质有大量路易小体的特发性帕金森病尸检病例——弥漫性路易体病]

[An autopsy case of idiopathic parkinsonism with numerous Lewy bodies in the cerebral cortex--diffuse Lewy body disease].

作者信息

Akashi T, Maruyama N, Sawayama H

机构信息

National Center Hospital for Mental, Nervous, and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan.

出版信息

No To Shinkei. 1991 Feb;43(2):175-81.

PMID:1651748
Abstract

We report an autopsy case of a 73 year-old female with idiopathic parkinsonism, characterized pathologically by the wide spread appearance of Lewy bodies (LBs) not only in the pigmented neurons in the midbrain and brainstem but also in the cerebral cortex. Initial symptoms at the age of 62 were finger tremor and gait disturbance, which were followed mainly by mental deterioration, such as regression, dependency, auditory hallucination, depression, emotional incontinence, and a personality change. In the terminal stage, nuchal stiffness in extension, one of the hallmarks of progressive supranuclear palsy, and slow and generalized tremor in all 4 extremities were noted. She died of aspiration pneumonia. The brain was somewhat small and weighed 1100 g after the fixation by formalin. Macroscopical findings included mild cerebral atrophy with mild pial thickening both in the frontal and temporal lobes and slight expansion of the ventricular system. Histopathologically, severe loss of neuronal cells in both the pallidum and Luy's body and moderate loss of large cells in the putamen were noted in addition to the typical findings of Parkinson's disease in the substantia nigra and locus caeruleus including neuronal cell loss, depigmentation, and gliosis. These findings in the basal ganglia were more conspicuous than the two controls of classical Parkinson's disease. The distribution, stainability in the routine methods of staining, and shape of Lewy bodies in the cerebral cortex conformed to those of previous reports. The similar case reports in the literatures do not seem to have paid much attention to the findings of the basal ganglia observed in our case.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

我们报告一例73岁患特发性帕金森病的女性尸检病例,其病理特征为路易小体(LB)广泛分布,不仅见于中脑和脑干的色素神经元,还见于大脑皮质。62岁时的初始症状为手指震颤和步态障碍,随后主要出现精神衰退,如退行性变、依赖、幻听、抑郁、情感失禁和人格改变。在终末期,出现了进行性核上性麻痹的标志性症状之一——颈部伸展僵硬,以及四肢缓慢且全身性的震颤。她死于吸入性肺炎。大脑略小,经福尔马林固定后重1100克。宏观检查结果包括轻度脑萎缩,额叶和颞叶软脑膜轻度增厚,脑室系统轻度扩张。组织病理学检查发现,除黑质和蓝斑出现帕金森病的典型表现,包括神经元细胞丢失、色素脱失和胶质增生外,苍白球和路易体均有严重的神经元细胞丢失,壳核有中度大细胞丢失。基底神经节的这些表现比两例经典帕金森病对照更为明显。大脑皮质中路易小体的分布、常规染色方法中的可染性及形态与既往报道相符。文献中类似的病例报告似乎未对我们病例中观察到的基底神经节表现给予太多关注。(摘要截稿于250字)

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