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Rapidly progressing dementia as a manifestation of the Creutzfeldt-Jakob disease: an analysis of two cases

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Seweryn Bartosz S., Natalia Leksa N., Dominika Uberman-Kluz D., Artur Szymczak A., Marek Biesiadecki M., Sabina Galiniak S.
European Journal of Clinical and Experimental Medicine
|
2019
|
issue 1
89-93
EN
Introduction. Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease of the central nervous system which is caused by an infectious protein called prion. Multiple forms of CJD have been classified including sporadic (more than 90% cases), familial, iatrogenic and variant type of disease. CJD, especially in its early stages, is a highly challenging illness to diagnose. Aim. Article aims to present cases of Creutzfeldt-Jackob disease with early symptoms of rapidly progressing dementia at the initial stage of CJD. Description of the cases. This paper describes two cases of patients with suspected CJD with a history of rapidly progressive dementia admitted to the Department of Neurology, MSWiA Hospital in Rzeszów. Conclusion. Despite the fact that CJD is an incurable illness and there is no cure guaranteeing recovery, it is important to make the right diagnosis. Assay of 14-3-3 protein in cerebrospinal fluid is a sensitive and specific marker which is helpful in the diagnosis of CJD. The only relevant method of correctly confirming a diagnosis of this disease is by performing a brain biopsy.
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Molekularny Dr Jekyll i Mr Hyde

84%
Lisiecki K.
Edukacja Biologiczna i Środowiskowa
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2013
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issue 2
23-32
EN
Prion proteins are as yet very poorly known family of proteins. However, their unique characteristics make them, since their discovery (which was rewarded with the Nobel Prize) intensively investigated object. In addition, a surprising nature of prion proteins – proteins with two faces – makes them an extremely interesting biological curiosity.
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