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Definition
By Mayo Clinic staffCreutzfeldt-Jakob (KROITS-felt YAH-kobe) disease is a degenerative brain disorder that leads to dementia and, ultimately, death. Symptoms of Creutzfeldt-Jakob disease (CJD) sometimes resemble those of other dementia-like brain disorders, such as Alzheimer's, but Creutzfeldt-Jakob disease usually progresses much more rapidly.
Creutzfeldt-Jakob disease captured public attention in the 1990s when some people in the United Kingdom developed a form of the disease — variant CJD (vCJD) — after eating meat from diseased cattle. However, "classic" Creutzfeldt-Jakob disease has not been linked to contaminated beef.
Although serious, CJD is rare, and vCJD is the least common form. Worldwide, there is an estimated one case of Creutzfeldt-Jakob disease diagnosed per million people each year, most commonly in older adults.
- 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.
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