Human Prion Disease: Creutzfeldt-Jakob Disease
Journal Title: Journal of Neurological Sciences-Turkish - Year 2007, Vol 24, Issue 2
Abstract
Prion diseases such as Creutzfeldt-Jakob disease (CJD) in humans, scrapie in sheep, and BSE in cattle are transmissible and fatal neurodegenerative diseases. The infectious agent of these diseases has been designated as “prion”. CJD, which is usually sporadic without a known infectious source, but in a small percentage of people is inherited as an autosomal dominant trait or accidentally or iatrogenically transmitted. The appearance of a variant of CJD in humans, which is most likely caused by the consumption of BSE-infected food in the UK, is cause for major concern particularly since there is no known effective treatment of prion diseases. Making a clinical diagnosis of sporadic CJD relies on the evaluation of rapidly progressive dementia, ataxia, myoclonus, changes on the electroencephalogram, and other neurological signs. A definite diagnosis, however, is confined to cases that have been evaluated neuropathologically or by equivalent diagnostic techniques.
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Ozlem OZEN
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