human prion diseases in the united states人类朊病毒疾病在美国.pdfVIP

Human Prion Diseases in the United States 1 1 1 1 2 Robert C. Holman *, Ermias D. Belay , Krista Y. Christensen , Ryan A. Maddox , Arialdi M. Minino , 1 1 1 2 1 Arianne M. Folkema , Dana L. Haberling , Teresa A. Hammett , Kenneth D. Kochanek , James J. Sejvar , Lawrence B. Schonberger1 1 Division of Viral and Rickettsial Diseases, National Center for Zoonotic, Vector-borne and Enteric Diseases, Centers for Disease Control and Prevention (CDC), U.S. Department of Health and Human Services (USDHHS), Atlanta, Georgia, United States of America, 2 Division of Vital Statistics, National Center for Health Statistics, Centers for Disease Control and Prevention (CDC), U.S. Department of Health and Human Services (USDHHS), Hyattsville, Maryland, United States of America Abstract Background: Prion diseases are a family of rare, progressive, neurodegenerative disorders that affect humans and animals. The most common form of human prion disease, Creutzfeldt-Jakob disease (CJD), occurs worldwide. Variant CJD (vCJD), a recently emerged human prion disease, is a zoonotic foodborne disorder that occurs almost exclusively in countries with outbreaks of bovine spongiform encephalopathy. This study describes the occurrence and epidemiology of CJD and vCJD in the United States. Methodology/Principal Findings: Analysis of CJD and vCJD deaths using death certificates of US residents for 1979–2006, and those identified through other surveillance mechanisms during 1996–2008. Since CJD is invariably fatal and illness duration is usually less than one year, the CJD