Creutzfeldt-Jakob disease


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Prevention

By Mayo Clinic staff

There is no known way to prevent sporadic CJD. If you have a family history of neurological disease, you may benefit from talking with a genetics counselor, who can help you sort through the risks associated with your particular situation.

Preventing iatrogenic CJD
Hospitals and other medical institutions follow explicit policies to prevent iatrogenic CJD. These measures have included:

  • Exclusive use of synthetic human growth hormone, rather than the kind derived from human pituitary glands
  • Destruction of surgical instruments used on the brain or nervous tissue of someone with known or suspected CJD
  • Single-use kits for spinal taps (lumbar punctures)

To help ensure the safety of the blood supply, people with a risk of exposure to CJD or vCJD aren't eligible to donate blood. This includes people who:

  • Have a biological relative who has been diagnosed with CJD
  • Have received a dura mater brain graft
  • Have received human growth hormone
  • Spent a total of at least three months in the United Kingdom from 1980 to 1996
  • Spent five years or more in France from 1980 to the present
  • Received a blood transfusion in the U.K. between 1980 and the present
  • Have injected bovine insulin at any time since 1980

Preventing vCJD
The risk of contracting vCJD in the United States remains extremely low. So far, only three cases have been reported in the U.S. According to the Centers for Disease Control and Prevention, strong evidence suggests that these cases were acquired abroad — two in the United Kingdom and one in Saudi Arabia.

In the United Kingdom, where the majority of vCJD cases have occurred, fewer than 200 cases have been reported. After its first appearance in 1995, CJD incidence peaked between 1999 and 2000, and has been declining since.

Regulating potential sources of vCJD
Most countries have taken steps to prevent BSE-infected tissue from entering the food supply, including tight restrictions on importation of cattle from countries where BSE is common; restrictions on animal feed; strict procedures for dealing with sick animals; surveillance and testing methods for tracking cattle health; and restrictions on which parts of cattle can be processed for food.

The risk of vCJD from the following sources is estimated to be extremely low:

  • Vaccines. Some parts of cows, including blood, enzymes and amino acids, are used to grow the bacteria and viruses needed to make certain vaccines. Not all vaccines are grown in cattle parts, however, and the Food and Drug Administration (FDA) recommends that companies producing such vaccines use cattle parts only from low-risk countries. These recommendations apply to cosmetics as well. The FDA keeps a listing on its website of companies that use cattle from countries that aren't classified as low-risk.
  • Insulin. Insulin sold in the United States isn't derived from cattle, but you're allowed to import beef insulin from other countries if you follow specific guidelines. Because there's no way to guarantee the safety of imported insulin, talk to your doctor about the best way to obtain insulin from sources outside the United States.
References
  1. 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.
  2. 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.
  3. Imran M, et al. An overview of human prion diseases. Virology Journal. 2011;8:559.
  4. Zerr I, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain. 2009;132:2659.
  5. Rosenbloom MH, et al. The evaluation of rapidly progressive dementia. Neurologist. 2011;17:67.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.
  11. 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.
DS00531 Oct. 23, 2012

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