Volume 10, Number 6—June 2004
Perspective
Chronic Wasting Disease and Potential Transmission to Humans
Table 2
Creutzfeldt-Jakob disease patients investigated for a possible causal link of their illness with chronic wasting disease of deer and elk, United Statesa
Case no. | Age at death (y) | Year of death | Codon 129 | Western blot | Final diagnosis | Eating of venison from CWD-endemic area |
---|---|---|---|---|---|---|
1 |
25 |
2001 |
M/V |
Type 1 |
GSS 102 |
Yes |
2 |
26 |
2001 |
M/M |
Type 2 |
CJD |
No |
3b |
28 |
2002 |
nd |
nd |
GSS 102 |
No |
4 |
28 |
1997 |
M/M |
nd |
CJD |
No |
5 |
28 |
2000 |
M/V |
Type 1 |
CJD |
No |
6 |
30 |
1999 |
V/V |
Type 1 |
CJD |
No |
7 |
54 |
2002 |
V/V |
Type 2 |
CJD |
No |
8c |
55 |
1999 |
M/M |
Type 1 |
CJD |
No |
9d |
61 |
2000 |
M/M |
Type 1 |
CJD |
Yes |
10 |
63 |
2002 |
V/V |
Type 1 |
CJD |
No |
11e |
64 |
2002 |
M/M |
Type 1 |
CJD |
Yes |
12 | 66 | 2001 | M/M | Type 1 | CJD | No |
aCWD, chronic wasting disease; GSS, Gerstmann-Sträussler-Scheinker syndrome; CJD, Creutzfeldt-Jakob disease; nd, not done.
bImmunohistochemical analysis of postmortem brain tissue was consistent with GSS, and a GSS 102 mutation was confirmed in the family.
cInvestigated as part of a cluster of three case-patients who participated in “wild game feasts” in a cabin owned by one of the decedents.
dPatient grew up in an area known to be endemic for CWD and ate venison harvested locally; however, the CJD phenotype fits the most common form of sporadic CJD.
ePatient may have been successful in harvesting two deer since 1996 from a CWD-endemic area, but both deer tested negative for CWD.