Creutzfeld-Jakob disease: recommendations for infection control

Creutzfeld-Jakob disease, an infectious, progressive, degenerative neurologic disorder, has a presumably long incubation period but a rapid, fatal course. Brain tissue at autopsy resembles that seen in spongioform encephalopathies of other species. Creutzfeld-Jakob disease is transmitted by a proteinaceous infectious agent, or "prion." Epidemiologic patterns remain uncertain; various studies have reported conflicting risk factors in different populations, and genetic susceptibility may be involved. Although natural transmission routes are still unclear, both iatrogenic and nosocomial transmissions have been identified. Transmission has occurred through contaminated electrodes, contaminated biologic products from cadaveric brains, and infected donor tissues, including dura mater and corneas. Because the prion is difficult to eradicate, stringent sterilization precautions must be taken with all surgical instruments. Some tissues and body fluids (e.g., brain, ocular, central nervous system) from the patient with Creutzfeld-Jakob disease are highly infectious and must be contained or incinerated. Some body fluids, however, are not considered infectious. Persons with known or suspected Creutzfeld-Jakob disease, or with exposure to potential sources of iatrogenic infection, should not be considered as donors for any tissues or biologic products. Occupational transmission to health care and pathology workers is also possible. Therefore, specific preventive measures are necessary. Many questions remain regarding transmission and risk factors for Creutzfeld-Jakob syndrome, and the precautions presented here must be considered only preliminary.