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Prion Diseases Issue Toolkit

Background | Resources | Articles



 

Background

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 agent of TSEs is believed to be a prion. A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristics signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

(Source: CDC)

Resources


Prion Diseases General information about prion diseases from the WHO.
Prion Diseases General information about prion diseases from the CDC.
MMWR: Guidelines for Infection Control in Dental Health-Care Settings — 2003 Contains a section on prion diseases.
NINDS Creutzfeldt-Jakob Disease Information Page Information from the National Institute of Neurological Disorders and Stroke


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Articles

Could Alzheimer's Be Infectious, Like Mad Cow, CJD? The brain damage seen in some cases of Alzheimer's disease could have its roots in an infectious prion-like disease, such as that seen in bovine spongiform encephalopathy (mad cow) and its human form Creutzfeldt-Jakob disease (CJD), according to an international study published this week in the journal Molecular Psychiatry that was led by the University of Texas Medical School at Houston in the US.
Prion disease Human prion diseases are classified into 3 categories according to etiologies: idiopathic of unknown cause, acquired of infectious origin, and genetic by PRNP mutation.
Researchers’ Discovery May Revolutionize Treatment of ALS A team of researchers from the University of British Columbia and the Vancouver Coastal Health Research Institute have found a key link between prions and the neurodegenerative disease ALS (Amyotrophic Lateral Sclerosis), also known as Lou Gehrig’s disease. The discovery is significant as it opens the door to novel approaches to the treatment of ALS.
NIH study describes fast, sensitive blood test for human prion disease Scientists from the National Institute of Allergy and Infectious Diseases (NIAID), part of the National Institutes of Health (NIH), report that they have developed a method—10,000 times more sensitive than other methods—to detect variant Creutzfeldt-Jacob disease (vCJD) in blood plasma. vCJD is a type of prion disease in humans that leads to brain damage and death. The NIAID researchers also used the test to rapidly detect scrapie, a prion disease of sheep, in infected hamsters, some pre-symptomatic.
New Research Focuses on Prion Diseases Prion diseases, which include Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy ("mad cow" disease) in cattle, are caused by prions — unconventional pathogens composed of infectious protein particles and resistant to conventional sterilization procedures. Presently there is no known agent or procedure that can halt or reverse damage caused by prion disease.
Adsorption of prion and tissue proteins to surgical stainless steel surfaces and the efficacy Iatrogenic transmission of the infectious prion protein (PrPSc) is a potential threat due to its resistance to many chemical and enzymatic decontamination protocols and its strong adhesive properties to stainless steel. The conditions in which surgical instruments are handled during and after surgery may affect the level of tissue protein, prion attachment and the efficacy of subsequent decontamination regimes.
Blood Test Could Quickly Detect Prion Diseases Scientists at NIAID are progressing toward a faster, more practical way to screen people and animals for prion diseases, which have baffled researchers for decades.
Airborne Transmission of Prions Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, and other infectious diseases caused by prions have long been thought to spread almost exclusively by ingestion and direct inoculation. That assumption has now been challenged by results of a study by Haybaeck and colleagues, who conducted a series of experiments demonstrating airborne transmission of the prion disease, scrapie, to mice.
Prion disease: the implications for dentistry The aim of this article was to provide the dental community with a brief overview of the characteristics, risk of transmission, and the infection-control implications of prions in dentistry.
Implications for Creutzfeldt-Jakob disease (CJD) in dentistry: a review of current knowledge This review explores our current understanding of the risks of (variant) Creutzfeldt-Jakob disease transmission via dental practice, and whether they merit the rigorous enforcement of improved standards of instrument cleaning and decontamination.
Fears of new kinds of CJD

The warning was issued after the discovery that the infectious proteins that causes the disease come in much greater variety than previously thought.

Implications of prion diseases for dentistry: an update Although no contagion has been reported in the dental setting to date, prions resist the usual dental sterilization systems and transmission of this type of disease remains a potential risk. It is therefore important for dentists to be aware of these diseases, to identify high-risk patients by obtaining an adequate clinical history, and to know the appropriate procedures to be followed.
Risk assessment of transmission of sporadic Creutzfeldt-Jakob disease in endodontic practice in absence of adequate prion inactivation The risk of sCJD transmission through endodontic procedure compares with other health care risks of current concern such as death after liver biopsy or during general anaesthesia. These results show that single instrument use or adequate prion-decontamination procedures like those recently implemented in dental practice must be rigorously enforced.
Cleanability of dental instruments--implications of residual protein and risks from Creutzfeldt-Jakob disease Cleaning of dental instruments is the first line of control in reducing the adherent bioburden. The threat of vCJD and the difficulty in removing prion protein has provided a new challenge for cleaning surgical and dental instruments.


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