Creutzfeldt–Jakob Disease - Epidemiology

Epidemiology

Although CJD is the most common human prion disease, it is still rare, occurring in about one out of every one million people every year. It usually affects people aged 45–75, most commonly appearing in people between the ages of 60–65. The exception to this is the more recently-recognised 'variant' CJD (vCJD), which occurs in younger people.

CDC monitors the occurrence of CJD in the United States through periodic reviews of national mortality data. According to the CDC:

• CJD occurs worldwide at a rate of about 1 case per million population per year.
• On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million persons in the United States.
• In the United States, CJD deaths among persons younger than 30 years of age are extremely rare (fewer than five deaths per billion per year).
• The disease is found most frequently in patients 55–65 years of age, but cases can occur in people older than 90 years and younger than 55 years of age.
• In more than 85% of cases, the duration of CJD is less than 1 year (median: four months) after onset of symptoms.