Variant Creutzfeldt-Jakob disease (vCJD) is a type of prion disease. Bovine spongiform encephalopathy is a prion disease that affects cows; there is evidence that this illness can be transmitted to humans, producing vCJD. This illness is often called “mad cow disease.”
Prion diseases are a unique form of infectious diseases. The disease is not produced by a bacterial or viral infection; instead, the illness is related to progressive accumulation of prions (infectious protein particles). The central nervous system is progressively damaged as these prions accumulate.
Exposure to prion containing tissue is the primary risk factor. Other risk factors include:
Five to ten percent of all cases of the nonvariant form of Creutzfeldt-Jakob are inherited.
The average age of people who get this disease is 29 years old. Rare cases have been reported in children.
Once you are exposed, it can take up to 20 years until symptoms develop. When symptoms develop they usually follow these three phases:
Psychiatric symptoms predominate:
Neurologic symptoms predominate:
The average length of time from first symptoms to death is 13 months (with a range of 6-39 months).
The clinical history and physical exam are the primary diagnostic tools. If your physician suspects vCJD, additional tests may be needed, such as:
In many cases, final diagnosis requires autopsy and pathological studies.
Currently, there is no cure for vCJD. Treatment is primarily supportive, maximizing function and minimizing discomfort.
About 200 worldwide cases of vCJD have occurred to date, most of which were associated with beef consumption in the United Kingdom. There is a great deal of controversy regarding safety of US beef. Two cases of bovine spongiform encephalopathy have been detected in the US. But, no cases of vCJD have been attributed to eating US beef.
To minimize risk, it is generally recommended that you avoid beef products, particularly processed meat (eg, sausage and hotdogs), or beef items containing brain, spinal cord, or bone marrow.
RESOURCES:
Creutzfeldt-Jakob Disease Foundation
http://www.cjdfoundation.org/
The National Creutzfeldt-Jakob Disease Surveillance Unit
http://www.cjd.ed.ac.uk/index.htm
World Health Organization
http://www.who.int/
CANADIAN RESOURCES:
Health Canada
http://www.hc-sc.gc.ca/
Public Health Agency of Canada
http://www.phac-aspc.gc.ca/index-eng.php/
References:
Creutzfeldt-Jakob spongiform encephalopathy. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated December 30, 2009. Accessed February 1, 2009.
Roos, RP. Prion diseases. In: Gilman S, ed. MedLink Neurology. San Diego, CA: MedLink Corporation. MedLink website. Available at: http://www.medlink.com . Accessed February 1, 2009.
Churg-Strauss syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed . Updated December 12, 2007. Accessed February 23, 2008.
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Moo-ve over, beef burgers: EN finds many alternatives. Environmental Nutrition . 2004 Aug;27:5.
NINDS Creutzfeldt-Jakob disease information page. National Institute of Neurological Disorders and Strokes website. Available at: http://www.ninds.nih.gov/disorders/cjd/cjd_pr.htm .
Prusiner SB. Detecting mad cow disease. Scientific American . 2004;291:60-67.
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Smith-Bathgate B. Creutzfeldt-Jakob disease: diagnosis and nursing care issues. Nursing Times . 2005;101:52.
Variant Creutzfeldt-Jakob disease. World Health Organization website. Available at: http://www.who.int/mediacentre/factsheets/fs180/en/ . Updated November 2002. Accessed March 17, 2009.
Zeidler M, Sellar R, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet . 2000;355:1412-1419.
Last reviewed January 2009 by J. Thomas Megerian, MD, PhD, FAAP
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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