Creutzfeldt-Jakob disease and its variants belong to a broad group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs). The name derives from the spongy holes, visible under a microscope, that develop in affected brain tissue.

The cause of Creutzfeldt-Jakob disease and other TSEs appears to be abnormal versions of a kind of protein called a prion. Normally these proteins are harmless. But when they're misshapen, they become infectious and can harm normal biological processes.

How CJD is transmitted

The risk of CJD is low. The disease can't be transmitted through coughing or sneezing, touching or sexual contact. The three ways it develops are:

  • Sporadically. Most people with classic CJD develop the disease for no apparent reason. Termed spontaneous CJD or sporadic CJD, this type accounts for the majority of cases.
  • By inheritance. In the United States, about 5 to 10 percent of people with CJD have a family history of the disease or test positive for a genetic mutation associated with CJD. This type is referred to as familial CJD.
  • By contamination. A small number of people have developed CJD after being exposed to infected human tissue during a medical procedure, such as a cornea or skin transplant. Also, because standard sterilization methods do not destroy abnormal prions, a few people have developed CJD after undergoing brain surgery with contaminated instruments.

Cases of CJD related to medical procedures are referred to as iatrogenic CJD. Variant CJD is linked primarily to eating beef infected with mad cow disease (bovine spongiform encephalopathy or BSE).

Sept. 09, 2015