Causes of CJD

What Causes Creutzfeldt-Jakob Disease?

The latest thinking is that CJD is caused by a prion, which is a protein.^[3] In CJD, this prion is formed or folded incorrectly and forms clumps that disrupt brain function.

Sporadic Creutzfeldt-Jakob Disease Causes

While an improperly folded prion is involved in sporadic CJD, it is not known why or how people get the disease.^[5]

Familial or Genetic Creutzfeldt-Jakob Disease Causes

Familial or genetic CJD is inherited and involves a mutation in the gene responsible for making the prion. The mutation causes the prion to fold improperly.^[1]

Acquired or Iatrogeneic Creutzfeldt-Jakob Disease Causes

In acquired CJD, disease occurs by receiving CJD-contaminated tissues during medical procedures. Disease may develop after receiving a cornea (white part of the eye), skin or the covering of the brain from someone with CJD. Before 1985, it was possible to develop CJD from growth hormone taken from someone with CJD. Growth hormone from humans is no longer used in medical procedures. Recently, there has been a focus on the possibility that blood transfusions from persons with CJD may cause disease.

Variant Creutzfeldt-Jakob Disease Causes

In vCJD, disease occurs from ingesting the brain tissue of cows with bovine spongiform encephalopathy (BSE) known as mad cow disease. Researchers believe that it takes from 10 to over 20 years for disease to develop after eating contaminated beef.^[6]

Prions cause several diseases known as transmissible spongiform encephalopathies. All of these diseases result in declining brain function and lengthy periods of apparent health before symptoms appear. Prion disease affects both humans and animals^[7] and includes CJD and vCJD in humans. Among animals, bovine spongiform encephalopathy occurs in cows, chronic wasting disease in hoofed animals such as elk, moose, and deer, and scrapie8 in sheep and goats.