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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Perirhinal accumulation of neuronal alpha‐synuclein in a multiple system atrophy patient with dementia
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Perirhinal accumulation of neuronal alpha‐synuclein in a multiple system atrophy patient with dementia

机译：多系统萎缩患者痴呆症中神经元α-突触核蛋白的终极积累

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摘要

We report the case of a 79‐year‐old Japanese woman who developed cerebellar ataxia followed by rigidity, dysautonomia and cognitive disorders, and was thus clinically diagnosed as having possible MSA with dementia. Neuropathological findings demonstrated not only olivopontocerebellar and striatonigral degeneration with frequent glial cytoplasmic inclusions (GCIs), but also degenerative changes in the parahippocampal region, accentuated in the anterior portion of perirhinal cortex, where neuronal cytoplasmic inclusions (NCIs) and NFTs were numerous while GCIs were limited. NCIs were frequent in the deep layer, whereas NFTs were more frequent in superficial cortical layers. Other hippocampal subregions including subiculum, dentate fascia and cornu ammonis were minimally involved. NCIs in the perirhinal cortex showed intense argyrophilia with the Campbell‐Switzer silver impregnation method, but not argyrophilic with the Gallyas method. Most neuronal alpha‐synuclein aggregates in dendrosomatic fraction formed globular/tadpole‐like, and ultrastructurally comprised granular‐coated fine fibrils 12–24?nm in diameter. To the best of our knowledge, alpha‐synuclein‐related neuronal pathology localized in the perirhinal region without hippocampal involvement has not been previously reported in MSA, and may provide clues to elucidate how neuronal pathology evolves in the hippocampal/parahippocampal regions in MSA, particularly in cases with dementia.
机译：我们举报了一个79岁的日本女性，开发了小脑共济失调，其次是刚性，消化力学和认知障碍，因此临床诊断为具有痴呆症的MSA。神经病理学发现不仅证明了奥肝功能均表现和常见的胶质细胞质夹杂物（GCIS），而且在Perirhinal皮质前部的过程中突出的过程中的退行性变化，其中神经元细胞质夹杂物（NCIS）和NFT在GCIS的同时众多有限的。 NCIS在深层中经常出现，而NFT在浅表皮质层中更频繁。包括亚细胞，牙齿诱惑筋膜和玉米氨基的其他海马次区域最小化。菌根皮质中的NCIS显示出激烈的艾滋病与坎贝尔 - 瑞士银浸渍方法，但不是叙利的方法。大多数神经元α-突触核蛋白聚集体中形成球状/蝌蚪状，并且超微结构地包含直径的颗粒状涂覆的细原纤维12-24·nm。据我们所知，在没有海马受累的情况下局部化的α-突触核蛋白相关的神经元病理学未在MSA中报告，并且可以提供线索，以阐明神经元病理在MSA中的海马/总体疫苗区的发展方式，特别是在患有痴呆症的情况下。

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