Understanding and Preventing Prion Diseases

Posted on: 11 November 2016

Prion diseases occur due to abnormally folded proteins, which cause neurological damage. Although these neurological conditions are rare, there are some ways you can minimize your personal risk or the risk of passing the condition to your children.

Rare but Fatal Diseases

Fortunately, prion disease are rare, but this rarity also makes them harder to diagnose since many clinicians may not see a case. All prion diseases are inevitably fatal due to their destruction of the brain and other parts of the nervous system. Depending on the specific disease, the condition may progress over the course of weeks or two years. A specific concern for medical personnel is the possibility of cross-contamination of prion diseases from one person to another when the underlying diagnosis is unknown. The likely threat is surgical instruments that may have been used during surgery on the brain or spinal cord in an affected person. Unlike bacteria or viruses, prions are not destroyed with conventional sterilization techniques.

Genetic Elements

Some prion diseases mostly occur when the prion mutation is passed down from a parent. Since genetic testing is more widely available, it may be worth considering the test whether you want to know about your genetic profile for prion diseases and other conditions or plan to start a family. Since the emergence of prion diseases often occurs around middle age, those affected may have already started families before symptoms appear. For those with the genetic mutation, the odds of passing down the gene to any single child are 50%. Although there are genetic components to some prion diseases, the mutation may occur sporadically without any family history of the condition.

Food Contamination

One form of prion disease, variant Creutzfeldt-Jakob disease (vCJD) is closely related to bovine spongiform encephalopathy (BSE) or "mad-cow disease." vCJD can be contracted by a person's eating affected beef products. Once consumed, the prion can lie dormant for many years before symptoms appear. Fortunately, some countries meticulously screen cows for the disease to try to prevent nervous tissue from coming into contact with meat.

In rare cases where vCJD has occurred in the United States, the person has usually traveled to another country without any evidence of exposure occurring within the US. If you eat beef, you may want to avoid the protein altogether if you are visiting a region that engages in different manufacturing practices of beef. Additionally, some people and cultures consume the brain or other nervous-system tissues of cows for food. Since the prion associated with BSE is found within the nervous system, it is best to avoid eating these parts of the cow.

Prion diseases are a rare and deadly neurological conditions, making their identification and prevention important. Awareness by medical personnel and the public can reduce the instance of disease.