TY - JOUR
T1 - Creutzfeldt-Jakob disease in unusually young patients who consumed venison
AU - Belay, Ermias D.
AU - Gambetti, Pierluigi
AU - Schonberger, Lawrence B.
AU - Parchi, Piero
AU - Lyon, Douglas R.
AU - Capellari, Sabina
AU - McQuiston, Jennifer H.
AU - Bradley, Kristy
AU - Dowdle, Gerrie
AU - Crutcher, J. Michael
AU - Nichols, Craig R.
PY - 2001
Y1 - 2001
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0034793570&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0034793570&partnerID=8YFLogxK
M3 - Article
C2 - 11594928
AN - SCOPUS:0034793570
VL - 58
SP - 1673
EP - 1678
JO - Archives of Neurology
JF - Archives of Neurology
SN - 0003-9942
IS - 10
ER -