CJD is a rare and fatal degenerative brain disease of humans. CJD is one of a group of diseases known as transmissible spongiform encephalopathies (TSEs) or prion disease that affects humans and animals. In animals, the best known TSE is bovine spongiform encephalopathy (BSE) commonly referred to by the media as ‘Mad Cow’ disease.

Creutzfeldt-Jakob disease (CJD)

For simplicity, CJD is the term used in the Australian National CJD Infection Control Guidelines to describe all forms of Transmissible Spongiform Encephalopathies (TSE) or prion diseases except variant CJD (vCJD) which is an acquired form of prion disease that is quite different to CJD. Variant CJD, also commonly known as ‘Mad Cow Disease’ is related to the consumption of BSE contaminated beef, and to date, we have had no reported cases of vCJD in Australia.

CJD occurs at about the same frequency in all countries; 1 to 2 cases per million individuals per year. In Australia, we average about 35 cases of CJD a year.

CJD and other prion diseases includes:

  • Sporadic CJD (sCJD)
  • Genetic or inherited forms of CJD and other prion diseases
    1. Familial CJD (fCJD)
    2. Gerstmann-Sträussler Scheinker Syndrome (GSS)
    3. Fatal Familial Insomnia (FFI)
  • Acquired forms of CJD and other prion diseases
    1. Health Care associated or medically acquired (iatrogenic) CJD (iCJD)
    2. Kuru
    3. Variant CJD (vCJD)

There is currently no effective treatment or cure for CJD but research continues on many drugs. CJD is still only confirmed by autopsy although a test performed on cerebrospinal fluid to detect a protein marker can help diagnose CJD in people who already show clinical symptoms of the disease.