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Creutzfeldt-Jakob disease

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Definition:

Creutzfeldt-Jakob disease (CJD) is a form of brain damage that causes a rapid decrease of mental function and movement. It is believed to result from a protein called a prion. A prion folds abnormally. This seems to encourage other proteins to have bad shapes, which affects their ability to function.

Alternative Names:

Transmissible spongiform encephalopathy; vCJD; CJD

Causes, incidence, and risk factors:

There are several types of Creutzfeldt-Jakob disease (CJD). The disorder itself is rare, occurring in about 1 out of 1 million people. It usually first appears in midlife, beginning between ages 20 and 70, with average age at onset of symptoms in the late 50s.

The "classic" types of CJD are:

• Sporadic CJD makes up the most cases. It often occurs for no known reasons.
• Familial CJD results when a person inherited the abnormal protein (prion). Inherited CJD is rare.

NOTE: Classic CJD is not related to "mad cow" disease.

New variant Creutzfeldt-Jakob disease (vCJD) is an infectious form of CJD that is related to "mad cow disease" (bovine spongiform encephalitis ). The agent responsible for mad cow disease is believed to be the same agent responsible for vCJD in humans. This type of CJD was first described in 1996 in the United Kingdom.

New variant Creutzfeldt-Jacob disease accounts for less than 1% of cases, and tends to affect younger people. New variant CJD can result when someone is exposed to contaminated products.

Some cases of nvCJD have occurred in adolescents who have received growth hormone made from the pituitary glands of cadavers (dead bodies). Prions cannot be destroyed by ordinary disinfection techniques used to prevent transmission of viruses and bacteria. As a result, the hormone remains contaminated. Cadaver-derived growth hormone has been replaced by synthetically manufactured growth hormone, so this source of contagion is no longer a problem.

Other vCJD cases have occurred when people were given corneal transplants from infected donors, and from contaminated electrodes that were used in brain surgery (before it was known how to properly disinfect instruments).

There have not been any cases of nvCJD reported in the U.S.

Once symptoms appear, the disorder progresses rapidly and may be confused with other types of dementia -- like Alzheimer's disease. Both forms of CJD, however, are distinguished by extremely rapid progression from onset of symptoms to disability and death. Early symptoms include personality changes and difficulty with coordination.

Creutzfeldt-Jakob disease may be related to several other diseases also thought to be caused by prions, including kuru (seen in New Guinea women who ate the brains of deceased relatives as part of a funerary ritual), scrapie (found in sheep), and other rare human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia.

References:

Llewelyn CA, Hewitt PE, et al. Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 2004;363:417-421.

Peden AH, Head MW, et al. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient.  Lancet 2004;264:527-529.

Brown P, Will RG, Bradley R, Asher DM, Detwiler L. Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease: Background, Evolution, and Current Concerns. EID . January-February 2001; 7(1):6-16.

Noble J. Textbook of Primary Care Medicine . 3rd ed. St. Louis, Mo: Mosby; 2001:1551-1569.

• Review Date: 10/17/2005
• Reviewed By: Kevin Sheth, M.D., Department of Neurology, Brigham and Women's Hospital and Massachusetts General Hospital, Harvard Medical School, Boston, MA. Review provided by VeriMed Healthcare Network.

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