Human prion disease and relative risk associated with chronic wasting disease

• Published in: Emerging infectious diseases Vol. 12; no. 10; pp. 1527 - 1535
• Main Authors: Mawhinney, Samantha, Pape, W John, Forster, Jeri E, Anderson, C Alan, Bosque, Patrick, Miller, Michael W
• Format: Journal Article
• Language: English
• Published: United States U.S. National Center for Infectious Diseases 01-10-2006; Centers for Disease Control and Prevention
• Subjects: Adult; Aged; Animals; chronic wasting disease; Colorado - epidemiology; Creutzfeldt-Jakob syndrome; Creutzfeldt-Jakob Syndrome - epidemiology; Creutzfeldt-Jakob Syndrome - mortality; Creutzfeldt-Jakob Syndrome - transmission; Deer; Female; Humans; Male; Middle Aged; Prion diseases; Prion Diseases - epidemiology; Prion Diseases - mortality; Prion Diseases - transmission; Retrospective Studies; Risk factors; transmissible spongiform encephalopathies; United States; Wasting Disease, Chronic - epidemiology; Wasting Disease, Chronic - mortality; Wasting Disease, Chronic - transmission; Wasting syndrome; Colorado; United States; USA, Colorado
• ISSN: 1080-6040; 1080-6059
• DOI: 10.3201/eid1210.060019

The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40-1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73-1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.