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首页> 外文期刊>Virology Journal >A probable role of copper in the comorbidity in Wilson’s and Creutzfeldt-Jakob’s Diseases: a case report
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A probable role of copper in the comorbidity in Wilson’s and Creutzfeldt-Jakob’s Diseases: a case report

机译：铜在威尔逊和Creutzfeldt-Jakob疾病中的合并症中的可能作用：案例报告

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To the best of our knowledgedd, there is currently no case in the literature reporting the comorbidity of Wilson’s and Creutzfeldt-Jakob disease (CJD), linked through copper. A 44-year-old male with a history of inherited Wilson’s disease (hepatolenticular degeneration), which manifested as mild liver injury and psychiatric symptoms, was admitted to our department due to speech and cognitive disturbances. Upon his admission, he had motor aphasia as well as psychomotor retardation with an otherwise normal neurological examination. Laboratory tests, including liver enzymes, copper and serum ammonia were all within normal range. The brain MRI showed increased T2 signal in the caudate nuclei, attributed to copper deposition in the context of Wilson’s disease. In the electroencephalogram, periodic sharp discharges were eminent, initially unilateral and then generalized. The positive 14–3-3 protein in the cerebrospinal fluid (CSF) and the new brain MRI, that demonstrated elevated DWI signal not only in the basal ganglia but also in parts of the cerebral cortex (cortical ribbon sign), all supportive of a possible CJD diagnosis. The detection of PrPSc in the patient’s CSF, using the RT-QuIC method, which has a 99.4–100% specificity for CJD, made the diagnosis of CJD highly probable. This is the first report of Wilson’s and Creutzfeldt-Jakob diseases co-morbidity in the literature, which could evoke a possible role of copper in the pathogenesis of CJD.
机译：据我们所知，目前在文献中没有任何案例报告威尔逊和Creutzfeldt-Jakob疾病（CJD）的合并，通过铜连接。一个44岁的男性，历史遗传威尔逊疾病（肝细胞变性），这表现为轻度肝损伤和精神症状，由于言语和认知的紊乱，我们的部门被录取为我们的部门。在入场时，他患有摩托性厌氧菌以及精神脉冲抑制，另一种正常的神经检查。实验室测试，包括肝酶，铜和血清氨在正常范围内。脑MRI在硫核状核中显示出增加的T2信号，归因于威尔逊疾病的背景下的铜沉积。在脑电图中，定期尖锐的放电始终是单侧的，然后是普遍的。脑脊液（CSF）和新的脑MRI中的阳性14-3-3蛋白，不仅在基底神经节中展示了升高的DWI信号，而且在脑皮层（皮质丝带标志）的部分中，所有支持者可能的CJD诊断。使用RT-Quic方法检测患者的CSF中的PRPSC，该方法对CJD具有99.4-100％的特异性，使CJD高度可能的诊断。这是威尔逊和克雷斯菲尔特 - 雅各布疾病在文献中的第一个报告，可以在文献中巩固铜的可能作用。

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