wernickke - korsakoff综合征是散发性克雅氏病的一种罕见表型。

IF 1.9 3区 生物学 Q4 BIOCHEMISTRY & MOLECULAR BIOLOGY Prion Pub Date : 2018-03-04 Epub Date: 2018-02-09 DOI:10.1080/19336896.2018.1433988
Joanna Bielewicz, Anna Szczepańska-Szerej, Magdalena Ogórek, Piotr Dropko, Katarzyna Wojtal, Konrad Rejdak
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引用次数: 1

摘要

我们报告了一例以散发性克雅氏病(sCJD)为早期临床表现的wernickke - korsakoff综合征(WKs)患者。66岁女性主诉头晕和不平衡,主要发生在走路时。神经学检查显示了WKs的三联症状特征,如凝视性麻痹、四肢和躯干共济失调以及虚构的记忆障碍。这种紊乱在病程中增加,导致患者在症状出现4个月后死亡。患者最终被诊断为sCJD疾病。支持sCJD诊断最有用的辅助检查结果是脑弥散DWI MRI(弥散加权磁共振成像)和脑脊液14-3-3蛋白的存在。由于sCJD的表现非常独特,在做出最终诊断时应考虑其他原因。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease.

We reported the case of a patient with Wernicke-Korsakoff syndrome (WKs) as an early clinical manifestation of sporadic Creutzfeld-Jakob disease (sCJD). The 66-year-old female complained of dizziness and imbalance which mostly occurred while walking. A neurological examination revealed a triad of symptoms characteristic for WKs such as gaze paresis, ataxia of limbs and trunk as well as memory disturbances with confabulations. The disturbances increased during the course of the disease, which led to the death of the patient four months after the appearance of the signs. The patient was finally diagnosed with sCJD disease. The most useful ancillary examination results supporting sCJD diagnosis were brain diffusion DWI MRI (diffusion weighted magnetic resonance imaging) and the presence of 14-3-3 protein in CSF (cerebrospinal fluid). Since that manifestation of sCJD is very unique other causes should be taken into consideration while making a final diagnosis.

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来源期刊
Prion
Prion 生物-生化与分子生物学
CiteScore
5.20
自引率
4.30%
发文量
13
审稿时长
6-12 weeks
期刊介绍: Prion is the first international peer-reviewed open access journal to focus exclusively on protein folding and misfolding, protein assembly disorders, protein-based and structural inheritance. The goal is to foster communication and rapid exchange of information through timely publication of important results using traditional as well as electronic formats. The overriding criteria for publication in Prion are originality, scientific merit and general interest.
期刊最新文献
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