
Contents
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Introduction Introduction
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Sporadic Creutzfeldt-Jakob Disease Sporadic Creutzfeldt-Jakob Disease
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Clinical-pathological features Clinical-pathological features
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Incidence and basic demographics Incidence and basic demographics
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Risk factors Risk factors
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Variably Protease Sensitive Prionopathy Variably Protease Sensitive Prionopathy
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Variant Creutzfeldt-Jakob Disease Variant Creutzfeldt-Jakob Disease
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Clinical-pathological features Clinical-pathological features
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Incidence worldwide Incidence worldwide
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Risk factors Risk factors
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Prevalence Prevalence
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Person-to-person spread Person-to-person spread
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Kuru Kuru
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Iatrogenic Creutzfeldt-Jakob Disease Iatrogenic Creutzfeldt-Jakob Disease
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Incidence and sources of infection Incidence and sources of infection
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Human pituitary derived hormones Human pituitary derived hormones
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Cadaveric dura mater grafts Cadaveric dura mater grafts
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Other iatrogenic risk factors Other iatrogenic risk factors
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Genetic Prion Disease Genetic Prion Disease
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Incidence and geographic distribution Incidence and geographic distribution
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Clinical-pathological features Clinical-pathological features
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Risk factors Risk factors
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Animal Prion Diseases Animal Prion Diseases
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Scrapie Scrapie
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Bovine Spongiform Encephalopathy Bovine Spongiform Encephalopathy
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Chronic wasting disease Chronic wasting disease
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Conclusion Conclusion
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References References
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35 The neuroepidemiology of human prion disease
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Published:October 2020
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Abstract
Human prion diseases comprise a number of rare and fatal neurodegenerative conditions that result from the accumulation in the central nervous system of an abnormal form of a naturally occurring protein, called the prion protein. The diseases occur in genetic, sporadic, and acquired forms: genetic disease is associated with mutations in the prion protein gene (PRNP); sporadic disease is thought to result from a spontaneous protein misfolding event; acquired disease results from transmission of infection from an animal or another human. The potential transmissibility of the prion in any of these forms, either in disease states or during the incubation period, has implications for public health. Here we focus on Creutzfeldt-Jakob Disease (CJD), including variant Creutzfeldt-Jakob Disease (vCJD), although we will also discuss other forms of human prion disease.
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