Human prion diseases in the Czech Republic
Authors:
Z. Rohan 1; R. Rusina 2,3; M. Marešová 4; R. Matěj 1,2
Authors place of work:
Národní referenční laboratoř pro lidská prionová onemocnění, Oddělení patologie a molekulární medicíny, Thomayerova nemocnice, Praha
1; Neurologická klinika a Centrum klinických neurověd, Univerzita Karlova v Praze, 1. lékařská fakulta a Všeobecná fakultní nemocnice v Praze
2; Oddělení neurologie, Thomayerova nemocnice, Praha
3; Protiepidemické oddělení HSHMP, pobočka Praha-jih
4
Published in the journal:
Epidemiol. Mikrobiol. Imunol. 64, 2015, č. 3, s. 115-120
Category:
Review Article
Summary
Human prion diseases are a group of very rare diseases with a unique pathogenesis and, due to an inauspicious prognosis and unavailability of therapy, with fatal consequences. The etiopathogenetic background is the presence of pathologically misfolded prion protein, highly resistant to denaturation, the aggregation and presence of which in the brain tissue causes irreversible neuronal damage. The most frequent prion disease in humans is Creutzfeldt-Jakob disease (CJD) which occurs in sporadic, hereditary/familial, or acquired/infectious/iatrogenic forms. A new form of CJD, variant CJD, is considered to be linked to dietary exposure to beef products from cattle infected with bovine spongiform encephalopathy (BSE) and to infection via blood transfusion. The clinical picture of these diseases is characterized by a rapidly progressing dementia, cerebellar and extrapyramidal symptoms, and rather specific MRI, EEG, and CSF findings. Clinically, the diagnosis is described as possible or probable prion disease and needs to be confirmed by neuropathological or immunological investigation of the brain tissue. Epidemiological data from the Czech Republic spanning the last decade are presented.
Key words:
neurodegenerative disease – transmissible spongiform encephalopathy – prion protein – Creutzfeldt-Jakob disease – Gerstmann-Sträussler-Scheinker
Zdroje
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Štítky
Hygiene and epidemiology Medical virology Clinical microbiologyČlánok vyšiel v časopise
Epidemiology, Microbiology, Immunology
2015 Číslo 3
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