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新型冠状病毒肺炎后克雅氏病:感染诱导的朊病毒蛋白错误折叠?一例报告。

Creutzfeldt-Jakob disease after COVID-19: infection-induced prion protein misfolding? A case report.

机构信息

Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, Udine, Italy.

Department of Medicine, University of Udine, Udine, Italy.

出版信息

Prion. 2022 Dec;16(1):78-83. doi: 10.1080/19336896.2022.2095185.

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare, fatal disease presenting with rapidly progressive neurological deficits caused by the accumulation of a misfolded form (PrPSc) of prion protein (PrPc). Coronavirus disease 2019 (COVID-19) is a primarily respiratory syndrome caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2); many diverse neurological complications have been observed after COVID-19. We describe a young patient developing CJD two months after mild COVID-19. Presenting symptoms were visuospatial deficits and ataxia, evolving into a bedridden state with preserved consciousness and diffuse myoclonus. Diagnostic work-up was suggestive of CJD. The early age of onset and the short interval between respiratory and neurological symptoms might suggest a causal relationship: a COVID-19-related neuroinflammatory state may have induced the misfolding and subsequent aggregation of PrPSc. The present case emphasizes the link between neuroinflammation and protein misfolding. Further studies are needed to establish the role of SARS-CoV-2 as an initiator of neurodegeneration.

摘要

克雅氏病(CJD)是一种罕见的致命疾病,其特征是由于朊病毒蛋白(PrPc)的错误折叠形式(PrPSc)的积累而导致的迅速进行性神经功能缺损。2019 年冠状病毒病(COVID-19)是一种主要的呼吸道综合征,由严重急性呼吸系统综合征冠状病毒 2(SARS-CoV-2)引起;在 COVID-19 之后,已经观察到许多不同的神经并发症。我们描述了一名年轻患者在轻度 COVID-19 后两个月发生 CJD。首发症状为视空间障碍和共济失调,进展为卧床状态,意识保持清醒,出现弥漫性肌阵挛。诊断检查提示 CJD。发病年龄较早,呼吸道和神经症状之间的间隔较短,可能提示存在因果关系:COVID-19 相关的神经炎症状态可能导致 PrPSc 的错误折叠和随后的聚集。本病例强调了神经炎症与蛋白质错误折叠之间的联系。需要进一步的研究来确定 SARS-CoV-2 作为神经退行性变的启动子的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/de38/9255144/da261cfbe558/KPRN_A_2095185_F0001_OC.jpg

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