Primary prevention

The following are useful for prevention of prion diseases.

  • Use of synthesized human GH or other pituitary-derived products in place of nonsynthetic pituitary-derived products would prevent iatrogenic transmission.

  • Maximizing screening of blood or blood products for transfusion to prevent iatrogenic transmission.

  • Minimizing consumption of beef from countries with endemic bovine spongiform encephalopathy is pertinent to prevent variant Creutzfeldt-Jakob disease (vCJD).

  • Minimizing consumption of deer, elk, and moose from countries with endemic chronic wasting disease (CWD) will reduce the theoretical risk of transmission to humans. (CWD has never caused a case of human prion disease to date, but has been successfully transmitted to squirrel monkeys.[58])

  • Genetic testing for families at risk for genetic CJD allows those afflicted to make informed reproductive choices related to the disease.

  • Destruction, or appropriate decontamination, of neurosurgical or invasive ophthalmologic equipment after use on patients with potential CJD is paramount in prevention. Country-specific protocols, where available, should be followed. UK Department of Health: minimise transmission risk of CJD and vCJD in healthcare settings Opens in new window[63]​​

  • Women who are at an increased risk of CJD are not eligible to donate breast milk.[64]

Secondary prevention

  • As transmission rates of all forms of CJD (except variant) are not known, patients and family members of those with prion disease should refrain from donating blood products. Additionally, they should notify healthcare professionals of their condition so that the necessary precautions are taken during medical procedures. For noninvasive medical procedures, universal precautions should suffice.

  • Due to the low incidence of prion disease, screening asymptomatic people is only recommended prior to blood donation and/or if there is a positive family history for prion disease. In the UK, asymptomatic people were found to have prions in their appendices at a rate of 1 in 2000, but subsequent studies of appendices collected before the BSE epidemic (1962-1979) and from people born after 1996 who had appendices removed between 2000 and 2014 have raised the possibility that these were false positives or staining artifacts, because similar rates were found in cohorts not exposed to BSE and patients with vCJD have prions in non-neuronal tissues (gut, liver, kidney, bone marrow).[26][145][146]​​​​​ To date there have been only five reported cases of variant CJD transmission and all were via blood transfusion, making screening important in the prevention of potential contamination to the blood supply.[10][29][30][31]​​​ Screening for all patients should include taking a thorough medical and family history. Patients should also be questioned about travel to bovine spongiform encephalopathy (BSE)-positive countries, including dates of travel and a record of beef and venison consumption.

  • If found to have probable CJD, patients can seek trial treatments in the earliest stages of the disease and help prevent iatrogenic transmission. They and their families can better plan for their future. ClinicalTrials.gov Opens in new window

  • Asymptomatic patients with a family history of prion disease should also undergo neurologic testing and brain MRI scans, and consider genetic testing, although the latter may be the only evidence of genetic prion disease. Care should be taken when interpreting prion protein gene (PRNP) mutations versus polymorphisms as the latter may not cause disease.[7]

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