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克雅氏病的一种非典型表现:类似中风,来自爱尔兰三级医疗中心的经验

An Atypical Presentation of Creutzfeldt-Jakob Disease as a Stroke Mimic: Experience From an Irish Tertiary Center.

作者信息

Al Taie Hassan, Vasandani Nikhil, Nasehi Armon, O'Malley Tom

机构信息

Internal Medicine, Mayo University Hospital, Castlebar, IRL.

Surgery, Royal College of Surgeons in Ireland, Dublin, IRL.

出版信息

Cureus. 2023 Aug 7;15(8):e43066. doi: 10.7759/cureus.43066. eCollection 2023 Aug.

DOI:10.7759/cureus.43066
PMID:37680437
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10481629/
Abstract

Sporadic Creutzfeldt-Jakob Disease (sCJD) is a rare neurodegenerative prion disease that presents with symptoms of rapid neuropsychiatric decline including dementia, behavioural abnormalities, and loss of higher cortical function. Patients commonly present with rapidly progressive neuromotor symptoms such as ataxia and myoclonus. Very few cases of CJD have been reported in which the patient initially presents with stroke symptoms such as hemiparesis as their primary presenting symptom. We present a case of a 56-year-old male who initially presented to the stroke unit with waxing and waning left-sided weakness and a non-corresponding ipsilateral left-sided acute parietal infarct on diffusion-weighted MRI. Over four weeks, his condition progressively worsened with declining cognitive function, motor dysfunction, sphincter dysfunction, and eventual death.

摘要

散发性克雅氏病(sCJD)是一种罕见的神经退行性朊病毒疾病,其症状表现为神经精神功能迅速衰退,包括痴呆、行为异常以及高级皮层功能丧失。患者通常会出现快速进展的神经运动症状,如共济失调和肌阵挛。仅有极少数克雅氏病病例报告显示,患者最初以偏瘫等中风症状作为主要表现症状。我们报告一例56岁男性患者,其最初因左侧肢体无力症状反复出现而入住卒中单元,弥散加权磁共振成像(MRI)显示左侧顶叶急性梗死灶,但梗死灶与左侧肢体无力症状不相符。四周内,其病情逐渐恶化,出现认知功能下降、运动功能障碍、括约肌功能障碍,最终死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/889d/10481629/e38fc8108d37/cureus-0015-00000043066-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/889d/10481629/e38fc8108d37/cureus-0015-00000043066-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/889d/10481629/e38fc8108d37/cureus-0015-00000043066-i01.jpg

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本文引用的文献

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The first reported case of Creutzfeldt-Jakob disease from Nepal.尼泊尔首例克雅氏病报告病例。
Clin Case Rep. 2019 Dec 12;8(1):198-202. doi: 10.1002/ccr3.2609. eCollection 2020 Jan.
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RT-QuIC: a new test for sporadic CJD.实时震颤诱导转化(RT-QuIC):一种用于散发性克雅氏病的新检测方法。
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