Sporadic creutzfeldt-jakob disease: neuropsychiatric symptoms followed by a progressive congnitive decline: a case report
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Abstract
Creutzfeldt-Jakob disease (CJD) is the most common human prion disease and is unique among all known human disorders in being comprised of familial, sporadic, and transmitted forms. The prevailing hypothesis, based largely on studies in transgenic animals, suggests that CJD and other prion diseases are initiated and propagated by conversion of normal host-encoded prion protein (PrP) into a conformationally abnormal isoform (PrPreS). The latter has been shown to accumulate in the brain and is the biochemical hallmark of CJD. We report the case of a 37-year-old woman who died in third level hospital of sporadic CJD proven by autopsy. This case illustrates the great importance of a post mortem exam in such context. In light of this clinical observation, we discuss this rare diagnosis which should be considered in patients when confronted with a rapidly progressive dementia.
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