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 wreak havoc on 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:
Oct. 23, 2012
- Sporadically. Most people with classic CJD develop the disease for no apparent reason. CJD that occurs without explanation is termed spontaneous CJD or sporadic CJD and 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 bovine spongiform encephalopathy (BSE), the medical term for mad cow disease.
- Creutzfeldt-Jakob disease fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm. Accessed July 2, 2012.
- vCJD (variant Creutzfeldt-Jakob disease). Centers for Disease Control and Prevention. http://www.cdc.gov/ncidod/dvrd/vcjd/factsheet_nvcjd.htm. Accessed July 2, 2012.
- Imran M, et al. An overview of human prion diseases. Virology Journal. 2011;8:559.
- Zerr I, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain. 2009;132:2659.
- Rosenbloom MH, et al. The evaluation of rapidly progressive dementia. Neurologist. 2011;17:67.
- Greenberg DA, et al. Clinical Neurology. 7th ed. New York, N.Y.: The McGraw-Hill Companies; 2009. http://www.accessmedicine.com/content.aspx?aID=5143601. Accessed July 2, 2012.
- McPhee SJ, et al. Current Medical Diagnosis & Treatment 2012. 51st ed. New York, N.Y.: The McGraw-Hill Companies; 2012. http://www.accessmedicine.com/content.aspx?aID=17051. Accessed July 2, 2012.
- WHO infection control guidelines for transmissible spongiform encephalopathies. World Health Organization. http://www.who.int/csr/resources/publications/bse/WHO_CDS_CSR_APH_2000_3/en/. Accessed July 3, 2012.
- Vaccines and variant CJD (vCDJ) questions and answers. U.S. Food and Drug Administration. http://www.fda.gov/Drugs/ResourcesForYou/Consumers/QuestionsAnswers/ucm173909.htm?utm_campaign=Google2&utm_source=fdaSearch&utm_medium=website&utm_term=Insulin,%20vCJD&utm_content=10. Accessed July 3, 2012.
- Questions and answers on importing beef or pork insulin for personal use. U.S. Food and Drug Administration. www.fda.gov/BiologicsBloodVaccines/Vaccines/QuestionsaboutVaccines/ucm143522.htm - 12k - 2009-04-29. Accessed July 3, 2012.
- Current thinking on measures that could be implemented to minimize human exposure to materials that could potentially contain the bovine spongiform encephalopathy agent. http://www.fsis.usda.gov/oa/topics/bse_thinking.htm. Accessed July 3, 2012.