Genetic and Infectious Prion Diseases

Prusiner, Stanley B.

doi:10.1001/archneur.1993.00540110011002

Cited by 165 publications

(112 citation statements)

References 262 publications

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“…It was originally thought that the infectious agent was a slow virus or virus-like particle. However, increasing amounts of evidence suggest that unique agents, termed PrPs, are central in the etiology of these diseases (5). The prion is theorized to contain only protein, has no DNA or RNA and replicates by converting the structure of the normal cellular PrP into an abnormal one by a mechanism not yet known (5).…”

Section: What Is Cjd?mentioning

confidence: 99%

“…However, increasing amounts of evidence suggest that unique agents, termed PrPs, are central in the etiology of these diseases (5). The prion is theorized to contain only protein, has no DNA or RNA and replicates by converting the structure of the normal cellular PrP into an abnormal one by a mechanism not yet known (5). The prion diseases are characterized by microscopic intracellular vacuoles (spongiform degeneration), severe astrocytic gliosis and the deposition of prion amyloid plaques in the grey matter of the brain (5).…”

Section: What Is Cjd?mentioning

confidence: 99%

“…The prion is theorized to contain only protein, has no DNA or RNA and replicates by converting the structure of the normal cellular PrP into an abnormal one by a mechanism not yet known (5). The prion diseases are characterized by microscopic intracellular vacuoles (spongiform degeneration), severe astrocytic gliosis and the deposition of prion amyloid plaques in the grey matter of the brain (5). In animals, these diseases include scrapie in sheep and goats, bovine spongiform encephalopathy in cattle, chronic wasting disease in deer and elk, and transmissible mink encephalopathy.…”

Section: What Is Cjd?mentioning

confidence: 99%

“…In animals, these diseases include scrapie in sheep and goats, bovine spongiform encephalopathy in cattle, chronic wasting disease in deer and elk, and transmissible mink encephalopathy. In humans, four diseases were originally identified as TSEs: CJD, Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia and kuru (1,5,6). Two new forms of human TSEs have been recently identified: variant CJD (vCJD) and sporadic fatal insomnia (6,7).…”

Section: What Is Cjd?mentioning

confidence: 99%

“…Two new forms of human TSEs have been recently identified: variant CJD (vCJD) and sporadic fatal insomnia (6,7). All forms of TSEs are experimentally transmissible, some across species barriers (5,6,8).…”

Section: What Is Cjd?mentioning

confidence: 99%

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Creutzfeldt‐Jakob Disease and Infection Control

Johnston

Conly

2001

Canadian Journal of Infectious Diseases and Medical Microbiolog

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Over the past year, several situations have occurred in Canada in which patients who had recently undergone a surgical procedure were subsequently diagnosed with confirmed or suspected Creutzfeldt-Jakob disease (CJD). This raised concerns over contamination of surgical instruments: which instruments might have been contaminated from direct exposure to tissues; can instruments become cross-contaminated by exposure to other contaminated instruments; what assessment is necessary to determine cross-contamination; and what should be done with instruments that have been contaminated. Additionally, should there be a patient traceback in the face of potential but unproven exposure? Unfortunately, there are no easy answers to most of the above questions. Australia, the United Kingdom and the World Health Organization have developed guidelines for the infection control management of patients with CJD, as well as instruments and devices that come into contact with them and their tissues (1-3). Health Canada's draft CJD infection control guidelines, withdrawn from the Health Canada Web site until safety concerns regarding sodium hydroxide can be addressed, closely mirrored recommendations made in those documents. The Centers for Disease Control and Prevention guidelines for CJD are under revision. However, a recent American publication made recommendations on what procedures should be used for reprocessing items that have been in contact with the prion protein (PrP) (4). These recommendations differ substantially from the draft Canadian guidelines. This article reviews current knowledge about CJD, and highlights some of the infection control concerns and controversies.

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Section: What Is Cjd?mentioning

confidence: 99%

Section: What Is Cjd?mentioning

confidence: 99%

Section: What Is Cjd?mentioning

confidence: 99%

Section: What Is Cjd?mentioning

confidence: 99%

Section: What Is Cjd?mentioning

confidence: 99%

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Creutzfeldt‐Jakob Disease and Infection Control

Johnston

Conly

2001

Canadian Journal of Infectious Diseases and Medical Microbiolog

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An Introduction to the Molecular Genetics of Neurological Disease

Rosenberg¹

1993

Arch Neurol

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Risk of Transmission of Bovine Spongiform Encephalopathy to Humans in the United States<SUBTITLE>Report of the Council on Scientific Affairs</SUBTITLE>

Tan¹

1999

JAMA

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Current risk of transmission of BSE in the United States is minimal because (1) BSE has not been shown to exist in this country; (2) adequate regulations exist to prevent entry of foreign sources of BSE into the United States; (3) adequate regulations exist to prevent undetected cases of BSE from uncontrolled amplification within the US cattle population; and (4) adequate preventive guidelines exist to prevent high-risk bovine materials from contaminating products intended for human consumption.

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Genetic and Infectious Prion Diseases

Cited by 165 publications

References 262 publications

Creutzfeldt‐Jakob Disease and Infection Control

Creutzfeldt‐Jakob Disease and Infection Control

An Introduction to the Molecular Genetics of Neurological Disease

Risk of Transmission of Bovine Spongiform Encephalopathy to Humans in the United States<SUBTITLE>Report of the Council on Scientific Affairs</SUBTITLE>

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