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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Extensive distribution of glial cytoplasmic inclusions in an autopsied case of multiple system atrophy with a prolonged 18-year clinical course.
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Extensive distribution of glial cytoplasmic inclusions in an autopsied case of multiple system atrophy with a prolonged 18-year clinical course.

机译：在多系统萎缩的尸检病例中，神经胶质细胞质内含物的分布广泛，临床病程延长了18年。

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摘要

We describe herein an autopsied case of multiple system atrophy (MSA) with prolonged clinical course of 18 years, and evaluate the extent of neurodegeneration and glial cytoplasmic inclusions (GCIs) in the entire brain of this rare case. A 64-year-old woman presented with typical neurological symptoms and imaging features of MSA. Thereafter, she became bedridden, and breathing was assisted through a tracheostomy for 12 years. She died at the age of 82 after 18 years from the initial symptom. Post mortem examination revealed severe neurodegeneration in the inferior olive, pontine nuclei, substantia nigra, locus ceruleus, putamen and cerebellum. Notably, phosphorylated alpha-synuclein (p-alpha-syn)-positive GCIs were found in these areas, but their number was very low. In contrast, the density of GCIs was much higher in such regions as the tectum/tegmentum of the brainstem, pyramidal tracts, neocortices and limbic system, which usually contain a small number of GCIs. Another constituent of GCIs, ubiquitin (Ub) and Ub-associated autophagy substrate p62, were also positive in some GCIs, and distribution of Ub/p62 immunoreactivity was proportionate to that of p-alpha-syn+ GCIs despite the very long duration of the disease. Furthermore, this case had complicated hypoxic encephalopathy, but p-alpha-syn+ GCIs were also found in the damaged white matter, indicating the contribution of alpha-syncleinopathy as well as hypoxic effect to the secondary myelin and axonal loss in the white matter. Together, this rare case suggests the contribution of the disease duration to the prevalence of GCIs, and the possible involvement of the limbic system in extensive-stage disease.
机译：我们在本文中描述了多系统萎缩（MSA）的尸体解剖病例，临床病程延长了18年，并评估了这种罕见病例的整个大脑中神经变性和神经胶质细胞质内含物（GCI）的程度。一名64岁的女性表现出典型的神经系统症状和MSA的影像学特征。此后，她卧床不起，并通过气管切开术辅助呼吸了12年。最初症状出现18年后，她去世，享年82岁。验尸后发现下橄榄，桥脑核，黑质，黑斑病，壳核和小脑严重神经变性。值得注意的是，在这些区域发现了磷酸化的α-突触核蛋白（p-α-syn）阳性的GCI，但其数量非常少。相反，在通常包含少量GCI的区域，例如脑干的顶盖/盖骨，锥体束，新皮质和边缘系统中，GCI的密度要高得多。 GCI的另一种成分，遍在蛋白（Ub）和与Ub相关的自噬底物p62在某些GCI中也呈阳性，尽管病程很长，但Ub / p62免疫反应性的分布与p-alpha-syn + GCI的分布成比例。此外，该病例患有复杂的缺氧性脑病，但在受损的白质中也发现了p-alpha-syn + GCI，这说明了α-晕厥病以及缺氧对白质继发性髓鞘和轴突丧失的作用。总之，这种罕见的病例提示疾病持续时间对GCI患病率的贡献，以及边缘系统可能参与了广泛期疾病。

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