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[成人起病的痴呆伴大量类似进行性核上性麻痹的神经原纤维缠结]

[Adult-onset dementia with abundant neurofibrillary tangles resembling progressive supranuclear palsy].

作者信息

Hitoshi S, Mizutani T, Amano N, Bando M, Yamanouchi H

机构信息

Department of Neurology, Tokyo Metropolitan Geriatric Medical Center.

出版信息

Rinsho Shinkeigaku. 1994 Jun;34(6):557-62.

PMID:7955713
Abstract

We described an autopsy case of 79-year-old woman with clinically unclassified senile dementia. The patient developed forgetfulness at the age of 73, and later, persecution mania, apathy and episodic stupor, but no extrapyramidal symptoms. Neuropathological examination revealed severe neuronal loss and gliosis of substantia nigra, moderate neuronal loss and marked grumose degeneration of dentate nucleus, and mild astrocytosis of subthalamic nucleus. Abundant neurofibrillary tangles (NFT) were observed in subthalamic nucleus, globus pallidus, substantia nigra, locus ceruleus, tegmentum of brain stem, pontine nucleus, inferior olive, and dentate nucleus. Gallyas silver impregnation method showed a wide distribution of argyrophilic grains and threads in cerebrum, brain stem and cerebellum. Although absence of clinical and neuropathological hallmarks excluded the diagnosis of progressive supranuclear palsy (PSP), the distribution of NFT and argyrophilic grains in this patient resembled PSP.

摘要

我们描述了一例79岁临床未分类老年痴呆症女性的尸检病例。该患者73岁时出现健忘,随后出现迫害妄想、淡漠和发作性木僵,但无锥体外系症状。神经病理学检查显示黑质严重神经元丢失和胶质细胞增生,齿状核中度神经元丢失并伴有明显颗粒样变性,以及丘脑底核轻度星形细胞增多。在丘脑底核、苍白球、黑质、蓝斑、脑干被盖、脑桥核、下橄榄核和齿状核中观察到大量神经原纤维缠结(NFT)。加利亚斯银浸染法显示嗜银颗粒和细丝在大脑、脑干和小脑中广泛分布。尽管缺乏临床和神经病理学特征排除了进行性核上性麻痹(PSP)的诊断,但该患者NFT和嗜银颗粒的分布与PSP相似。

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