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Ermias D. Belay, MD; Pierluigi Gambetti, MD; Lawrence B. Schonberger, MD; Piero Parchi, MD; Douglas R. Lyon, MD; Sabina Capellari, MD; Jennifer H. McQuiston, DVM; Kristy Bradley, DVM; Gerrie Dowdle, MSPH; J. Michael Crutcher, MD; Craig R. Nichols, MPA

Arch Neurol. 2001;58:1673-1678.

Background  Creutzfeldt-Jakob disease (CJD) in humans and chronic wasting disease (CWD) in deer and elk occur in the United States. Recent reports of 3 unusually young patients with CJD who regularly consumed deer or elk meat created concern about the possible zoonotic transmission of CWD.

Objective  To examine the possible transmission of CWD to humans.

Patients  Three unusually young patients (aged 28, 28, and 30 years) with CJD in the United States during 1997-2000.

Methods  We reviewed medical records and interviewed family members and state wildlife and agriculture officials. Brain tissue samples were tested using histopathologic, immunohistochemical, immunoblot, or prion protein gene analyses.

Main Outcome Measures  Presence or absence of established CJD risk factors, deer and elk hunting in CWD-endemic areas, and comparison of the evidence for the 3 patients with that of a zoonotic link between new variant CJD and bovine spongiform encephalopathy.

Results  None of the patients had established CJD risk factors or a history of travel to Europe. Two patients hunted game animals and 1 was a daughter of a hunter. Unlike patients with new variant CJD, the 3 patients did not have a unique neuropathologic manifestation, clinicopathologic homogeneity, uniformity in the codon 129 of the prion protein gene, or prion characteristics different from those of classic variants.

Conclusions  Although the occurrence of 3 unusually young patients with CJD who consumed venison suggested a possible relationship with CWD, our follow-up investigation found no strong evidence for a causal link. Ongoing CJD surveillance remains important for continuing to assess the risk, if any, of CWD transmission to humans.

From the Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Ga (Drs Belay, Schonberger, and McQuiston); the National Prion Disease Pathology Surveillance Center, Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio (Drs Gambetti, Parchi, and Capellari); the Utah Department of Health, Salt Lake City (Dr Lyon, Ms Dowdle, and Mr Nichols); and the Oklahoma State Department of Health, Oklahoma City (Drs Bradley and Crutcher).

Corresponding author and reprints: Ermias D. Belay, MD, Centers for Disease Control and Prevention, Mailstop A-39, 1600 Clifton Rd, Atlanta, GA 30333.

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