Questions and Answers: Creutzfeldt-Jakob Disease Infection-Control Practices

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Questions and Answers: Creutzfeldt-Jakob Disease Infection-Control Practices
On this page:

• What is Creutzfeldt-Jakob disease?
• How is CJD diagnosed?
• Have there been any reports of iatrogenic transmission of CJD?
• How should surgical instruments used on suspected or confirmed CJD patients be reprocessed?
• What are the chemical and autoclave sterilization methods outlined in Annex III of the WHO infection control guidelines for transmissible spongiform encephalopathies?
• How should instruments used in patients with no clear diagnosis at the time of a neurosurgical procedure be reprocessed?
• How should heat-sensitive instruments or materials that come in contact with suspected or confirmed CJD patients be decontaminated?
• What kinds of precautions should be taken while embalming the bodies of patients with suspected or confirmed CJD?
• Additional recommendations for CJD infection control practices not addressed in this Q&A

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Prion Diseases

About Prion Diseases Cattle at a trough, and a buck deer in the wild. (Courtesy Ermias Belay) Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. The causative agents of TSEs are believed to be prions. The term "prions" refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins called prion proteins that are found most abundantly in the brain. The functions of these normal prion proteins are still not completely understood. The abnormal folding of the prion proteins leads to brain damage and the characteristic signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal. A List of Prion Diseases Listed below are the prion diseases identified to date. Click the linked diseases to go to their respective topic sites. CDC does not currently offer information here on every prion disease listed. Human Prion Diseases Creutzfeldt-Jakob Disease (CJD) Variant Creutzfeldt-Jakob Disease (vCJD) Gerstmann-Straussler-Scheinker Syndrome Fatal Familial Insomnia Kuru Animal Prion Diseases Bovine Spongiform Encephalopathy (BSE) Chronic Wasting Disease (CWD) Scrapie Transmissible mink encephalopathy Feline spongiform encephalopathy Ungulate spongiform encephalopathy Links to Organizations Outside CDC National Prion Disease Pathology Surveillance Center (From the Division of Neuropathology, Case Western Reserve University. National CJD surveillance system established in collaboration with CDC.)

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About BSE

Beef cattle grazing. (Image courtesy USDA)

BSE (bovine spongiform encephalopathy) is a progressive neurological disorder of cattle that results from infection by an unusual transmissible agent called a prion. The nature of the transmissible agent is not well understood. Currently, the most accepted theory is that the agent is a modified form of a normal protein known as prion protein. For reasons that are not yet understood, the normal prion protein changes into a pathogenic (harmful) form that then damages the central nervous system of cattle.
Research indicates that the first probable infections of BSE in cows occurred during the 1970's with two cases of BSE being identified in 1986. BSE possibly originated as a result of feeding cattle meat-and-bone meal that contained BSE-infected products from a spontaneously occurring case of BSE or scrapie-infected sheep products. Scrapie is a prion disease of sheep. There is strong evidence and general agreement that the outbreak was then amplified and spread throughout the United Kingdom cattle industry by feeding rendered, prion-infected, bovine meat-and-bone meal to young calves.
The BSE epizootic in the United Kingdom peaked in January 1993 at almost 1,000 new cases per week. Over the next 17 years, the annual numbers of BSE cases has dropped sharply; 14,562 cases in 1995, 1,443 in 2000, 225 in 2005 and 11 cases in 2010. Cumulatively, through the end of 2010, more than 184,500 cases of BSE had been confirmed in the United Kingdom alone in more than 35,000 herds.
There exists strong epidemiologic and laboratory evidence for a causal association between a new human prion disease called variant Creutzfeldt-Jakob disease (vCJD) that was first reported from the United Kingdom in 1996 and the BSE outbreak in cattle. The interval between the most likely period for the initial extended exposure of the population to potentially BSE-contaminated food (1984-1986) and the onset of initial variant CJD cases (1994-1996) is consistent with known incubation periods for the human forms of prion disease.

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Articles

Human Prion Diseases in the United States PDF - 363KB
(2010) PLoS ONE 5(1):e8521