Prion diseases are a group of rare, invariably fatal brain disorders which occur both in humans and certain animals.
In humans the best known of the prion diseases is Creutzfeldt-Jakob Disease (CJD), which reportedly affects around one to two persons per million population each year. In the United States approximately one in every 6,239 deaths are due to CJD every year, which equates to approximately 400-500 new cases per year. It is well known that CJD is very difficult to diagnose leading to speculation that the one case per million report may be incorrect. Most of the cases are "sporadic" CJD (sCJD), occurring for no known reason. The sporadic form accounts for approximately 85% of the cases, and the familial form approximately 10% to 15%. There have also been a few cases that have occurred from contamination via medical procedures; this type is known as iatrogenic or Acquired CJD. In the 1980s and 1990s, another type of Acquired CJD called variant (vCJD) was identified in young people. CJD has been linked to ingestion of beef tainted with BSE (bovine spongiform encephalopathy), most cases have occurred in, or been tied to food from, the United Kingdom.
In animals, prion diseases first came to public attention in the mid 1980s in the form of the BSE epidemic in the United Kingdom. BSE (bovine spongiform encephalopathy) is a prion disease in cattle. Tissue from infected animals may have contaminated cattle feed, leading to the silent spread of the BSE epidemic. There is also a theory that BSE came from feed contaminated with scrapie, the long established sheep prion disease. Inevitably, concern over whether BSE could pass to humans mounted.
For information on the forms of CJD, click the links below:
For an overview on CJD, please visit:
"Progress in Detecting Prions and Diagnosing Prion Diseases," Byron Caughey, Ph.D.
Click here to watch Dr. Caughey's presentation at the 2015 CJD Foundation Family Conference in Washington, D.C.
"Prion Disease Overview," Brian Appleby, M.D., CJD Foundation Medical Director
"The Centers for Disease Control and Prevention Report: A CDC CJD Q&A," Ryan A. Maddox, Ph.D.
For more information on how to care for a patient with CJD, click here.