Prion diseases are rare, fatal neurodegenerative disorders caused by abnormal, misfolded proteins called prions. These prions accumulate in the brain, leading to irreversible damage. They primarily affect humans, manifesting as Creutzfeldt-Jakob Disease (CJD), but also occur in animals.
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Prion diseases were first recognized through conditions like Scrapie in sheep and Kuru among the Fore people of Papua New Guinea. The unique transmissible nature of Kuru, linked to ritualistic cannibalism, was a pivotal discovery. In the 1980s, Stanley Prusiner proposed the revolutionary concept of 'prions' (proteinaceous infectious particles) as the causative agent, for which he received the Nobel Prize.
Prion diseases can occur spontaneously (sporadic forms), be inherited (familial forms), or be acquired through exposure to contaminated tissue. Acquired forms include iatrogenic transmission (e.g., contaminated surgical instruments, dura mater grafts) and dietary routes (e.g., consumption of BSE-infected beef products, or Kuru via ritualistic cannibalism). They are not spread through casual contact, coughing, or sneezing.
Contagious Period: Varies by disease
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Chronic wasting disease continues to spread through Louisiana's deer populations, with 55 confirmed cases now detected across four parishes since initial identification in 2022. The recent detection in Ouachita Parish prompted emergency expansion of control areas and implementation of feeding restrictions to limit environmental transmission of this invariably fatal prion disease. While current prevalence remains substantially lower than heavily affected states, the geographic expansion pattern and environmental persistence of prions indicate ongoing risk of establishment in Louisiana's cervid populations. Although there is no confirmed risk to humans, the disease's zoonotic potential and environmental persistence remain under investigation, prompting caution among hunters and ongoing research into transmission pathways.
Source: BEACON - View Full Report
Seeing what my mom went through, I do not want anyone else to have to experience that, nor their family members. While no one is certain, experts think that another always-fatal prion disease -- this one currently known to occur only in cervids such as deer, moose, and elk -- may behave the same wa... Source: CIDRAP - Read Full Article
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View WHO Resources →Disease information on Virus Watcher is reviewed by our Chief Epidemiologist, a former CDC lead analyst for FluSight forecasting. Outbreak data is aggregated from verified sources including BEACON, ProMED, WHO, CDC, and 50+ national health agencies. This information is for educational purposes and should not replace professional medical advice.
Last reviewed: 2026-06-03
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