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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >An autopsy case of neuronal intermediate filament inclusion disease with regard to immunophenotypic and topographical analysis of the neuronal inclusions
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An autopsy case of neuronal intermediate filament inclusion disease with regard to immunophenotypic and topographical analysis of the neuronal inclusions

机译：关于神经元夹杂物的免疫表型和地形学分析的神经元中间丝状夹杂物疾病的尸检病例

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We report an autopsy case of neuronal intermediate filament inclusion disease (NIFID), in which pyramidal motor dysfunction preceded cognitive disturbance for 3 years from the onset. A 41-year-old Japanese man presented progressive spastic tetraparesis followed by cognitive impairment. His neurological symptoms gradually deteriorated and he died of pneumonia 16 years from the onset. His brain showed severe generalized atrophy with enlargement of ventricles. The microscopic examination revealed severe neuronal loss with gliosis and sponginess predominantly in the fronto-temporal cortices, caudate and putamen. Many hyaline conglomerate inclusions (HC) without immunoreactivity for fused in sarcoma' protein (FUS) and some granular and small round FUS-immunoreactive (FUS-ir) neuronal cytoplasmic inclusions (NCI) were observed in the remaining neurons. Some neurons with HC had small basophilic inclusions which showed positive FUS-ir, attached to HC in the cytoplasm. Otherwise, FUS-ir large compact inclusions (so-called Pick-like) were also observed but were scarce. In the cerebral cortex and the neostriatum, frequency of the inclusions was well correlated with neuronal loss. In the brainstem, neuronal loss was mild and FUS-ir inclusions dominated. In the subthalamic nucleus and red nucleus, there was no HC but there were many FUS-ir inclusions without neuronal loss. The above findings suggest that cytoplasmic mislocalization and aggregation of FUS appear at the early stage of the disease, and the FUS aggregate process may not be a direct precedent structure of HC.
机译：我们报告了神经元中间细丝包涵体疾病（NIFID）的尸检案例，其中锥体神经功能障碍在发病前3年出现认知障碍。一名41岁的日本男子出现进行性痉挛性四肢轻瘫，随后出现认知障碍。他的神经系统症状逐渐恶化，自发病16年后死于肺炎。他的大脑显示严重的全身萎缩，脑室扩大。显微镜检查发现严重的神经元丧失，神经胶质增生和海绵状，主要发生在额颞皮层，尾状和壳状核。在剩余的神经元中观察到许多对肉瘤蛋白（FUS）融合无免疫反应性的透明卵石包涵体（HC），以及一些颗粒状和小的圆形FUS免疫反应性（FUS-ir）神经元胞浆内含物（NCI）。一些带有HC的神经元具有小的嗜碱性包涵体，表现出FUS-ir阳性，并附着于细胞质中的HC。否则，还观察到FUS-ir大的紧凑夹杂物（所谓的Pick-like），但很少。在大脑皮层和新纹状体中，内含物的频率与神经元丢失密切相关。在脑干中，神经元丢失较轻，FUS-ir夹杂物占主导。在丘脑下核和红核中，没有HC，但有许多FUS-ir夹杂物，而没有神经元丢失。以上发现表明，FUS的胞质错位和聚集出现在疾病的早期，FUS聚集过程可能不是HC的直接先例结构。

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