Human Prion Diseases in the United States

摘要: Background Prion diseases are a family of rare, progressive, neurodegenerative disorders that affect humans and animals. The most common form human prion disease, Creutzfeldt-Jakob disease (CJD), occurs worldwide. Variant CJD (vCJD), recently emerged is zoonotic foodborne disorder almost exclusively in countries with outbreaks bovine spongiform encephalopathy. This study describes the occurrence epidemiology vCJD United States. Methodology/Principal Findings Analysis deaths using death certificates US residents for 1979–2006, those identified through other surveillance mechanisms during 1996–2008. Since invariably fatal illness duration usually less than one year, incidence estimated as rate. During 1979 2006, an 6,917 cause were reported States, annual average approximately 247 (range 172–304 deaths). age-adjusted was 0.97 per 1,000,000 persons. Most (61.8%) occurred among persons ≥65 years age 4.8 this population. whites (94.6%); 2.7 times higher blacks (1.04 0.40, respectively). Three patients who died since 2004 vCJD; epidemiologic evidence indicated their infection acquired outside Conclusion/Significance Surveillance continues to show rate about 1 case marked differences rates by race Ongoing remains important monitoring stability rates, detecting occurrences possibly novel