Chapter 32PRIONS: CREUTZFELDT–JAKOB DISEASE (CJD) and RELATED TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES (TSEs)

Dennis J. Darcey

Common names for disease: Sporadic Creutzfeldt–Jakob disease, new variant Creutzfeldt–Jakob disease, fatal familial insomnia, kuru, Gerstmann–Sträussler–Scheinker disease

Classification: Prion diseases

OCCUPATIONAL SETTING

There are theoretical but unproven risks to healthcare workers including physicians, surgeons, pathologists, nurses, dentists, laboratory technicians, veterinarians, veterinary technicians, agriculture workers, farmers, meat processors, butchers, abattoir workers, and cooks exposed to blood and uncooked animal products.

EXPOSURE (ROUTE)

Transmission of Creutzfeldt–Jakob disease (CJD) from human to human has been reported for patients receiving corneal transplants, dural grafts, and human growth hormone and gonadotropins derived from pooled human cadaver pituitary glands. Transmission has also been linked to inadequately sterilized instruments and stereotactic electrodes. In addition, transmission of kuru by ritual cannibalism has been documented. Although there have been case reports of healthcare personnel, veterinarians, and farmers developing CJD, there is no firm epidemiologic evidence to date to suggest that work in these occupations increases the risk for developing CJD.

A new variant Creutzfeldt–Jakob disease (nvCJD) has been associated with consumption of brain and spinal cord in sausages, hamburger, and other processed ...

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