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A probable role of copper in the comorbidity in Wilson’s and Creutzfeldt-Jakob’s Diseases: a case report

Authors :
Effrosyni Koutsouraki
Dimitrios Michmizos
Olga Patsi
John Tzartos
Martha Spilioti
Marianthi Arnaoutoglou
Magda Tsolaki
Source :
Virology Journal, Vol 17, Iss 1, Pp 1-5 (2020)
Publication Year :
2020
Publisher :
BMC, 2020.

Abstract

Abstract Background 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. Case presentation 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. Conclusion 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.

Details

Language :
English
ISSN :
1743422X
Volume :
17
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Virology Journal
Publication Type :
Academic Journal
Accession number :
edsdoj.6cc47d37064d455b95538e334f8919ef
Document Type :
article
Full Text :
https://doi.org/10.1186/s12985-020-01309-x