What is CJD?

The acronym CJD stands for Creutzfeldt-Jakob Disease. CJD is a very rare brain disease which is rapidly degenerative and fatal in most cases. The disease affects around one person in every million per year worldwide. The disease ordinarily appears in later life, but progresses rapidly – about 90% of patients die within a year of diagnosis.

CJD is a disease which is categorised within a family of diseases called TSEs, or Transmissible Spongiform Encephalopathies. TSEs can be found in humans and animals, and CJD is the most common form of this type of disease. The word ‘spongiform’ refers to the physical appearance of infected tissue, which adopts a microscopic sponge-like appearance. Bovine Spongiform Encephalopathy or BSE is a form of TSE which specifically affects cows, and is often referred to as Mad Cow Disease.

There are three categories of CJD

  • Sporadic CJD This refers to the fact that the patient has no known risk factors associated with the appearance of CJD. This is in fact the most common category of CJD.
  • Hereditary CJD There may be a family history of CJD, or test positive for the genetic mutation which is associated with the disease.
  • Acquired CJD In this instance the disease appears after the patient has been exposed to nervous system or brain tissue which is infected (ordinarily via certain medical procedures). This is an extremely rare form of the disease.

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