Human prion disease and relative risk associated with chronic wasting disease.
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.
Mawhinney
Samantha
S
University of Colorado at Denver, Health Sciences Center, Denver, Colorado 80262, USA. Sam.MaWhinney@UCHSC.edu
Pape
W John
WJ
Forster
Jeri E
JE
Anderson
C Alan
CA
Bosque
Patrick
P
Miller
Michael W
MW
D000328:Adult; D000368:Aged; D000818:Animals; D003120:Colorado / Q000453:epidemiology; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology* / Q000401:mortality / Q000635:transmission; D003670:Deer; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D017096:Prion Diseases / Q000453:epidemiology* / Q000401:mortality / Q000635:transmission; D012189:Retrospective Studies; D034081:Wasting Disease, Chronic / Q000453:epidemiology* / Q000401:mortality / Q000635:transmission