Prion Diseases

Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.

The causative agents of TSEs are believed to be prions. The term “prions” refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain. The functions of these normal prion proteins are still not completely understood. The abnormal folding of the prion proteins leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

Prion diseases are incredibly rare. Only about 300 cases are reported each year in the United States. 

Types of prion diseases include: 

  • Creutzfeldt-Jakob Disease (CJD). A person can inherit this condition, in which case it's called familial CJD. Sporadic CJD, on the other hand, develops suddenly without any known risk factors. Most cases of CJD are sporadic and tend to strike people around age 60. Acquired CJD is caused by exposure to infected tissue during a medical procedure, such as a cornea transplant. Symptoms of CJD (see below) quickly lead to severe disability and death. In most cases, death occurs within a year. 
  • Variant Creutzfeldt-Jakob Disease (vCJD). This is an infectious type of the disease that is related to “mad cow disease.” Eating diseased meat may cause the disease in humans. The meat may cause normal human prion protein to develop abnormally. This type of the disease usually affects younger people. 
  • Variably protease-sensitive prionopathy (VPSPr)
  • Gerstmann-Sträussler-Scheinker disease (GSS)
  • Kuru
  • Fatal insomnia (FI)

Animal Prion Diseases: 

  • Bovine spongiform encephalopathy (BSE), or Mad Cow Disease. Cattle can have a prion disease called Bovine spongiform encephalopathy (BSE) or Mad Cow Disease. For reasons that are not yet understood, the normal prion protein changes into a pathogenic (harmful) form that then damages the central nervous system of cattle. The disease was first diagnosed in the United Kingdom in 1986. Since then, the disease has occurred in many European countries as well as Japan, Canada and the United States. Most of the reported cases of BSE (95%) have occurred in the United Kingdom. Humans who eat BSE contaminated beef products can develop variant Creutzfeldt-Jakob disease (vCJD). Cases are incredibly rare.
  • Chronic Wasting Disease (CWD) Animals such as deer, elk and moose can have a prion disease called Chronic Wasting Disease. No cases have been found in people so far, but eating meat from infected animals is not recommended and may present some risk. CWD was first identified in the US in the 1960s in captive deer. As of mid-2023, CWD has been detected in deer, elk, or moose in over 30 U.S. states. CWD was first identified in the mid/northwest US, however it has recently been found in  states in the southeast. It is expected to continue expanding into other areas.  
  • Scrapie
  • Transmissible mink encephalopathy
  • Feline spongiform encephalopathy
  • Ungulate spongiform encephalopathy

General Resources: 

Georgia Specific Resources:

Georgia DNR

Contact DPH Epidemiology

For questions about disease surveillance contact public health.

Epidemiology Main Line:  (404) 657-2588

Fax:  (404) 657-2608

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Page last updated 1/23/2025