New Idaho CJD Cluster -
Looking In The
Wrong Places?
By Colm A Kelleher, PhD
With the death of the fifth person from CJD, and the investigation of a sixth since January 2005 in a small area in Idaho, health authorities are scrambling to figure out why. CJD, the 100% fatal brain wasting disease, is supposed to be vanishingly rare. Scientists from the Center for Disease Control (CDC) in Atlanta are puzzled. According to Reuters (ref 1): "Is what is happening in Idaho an anomaly, a statistical fluke? That is possible," said Ermias Belay, a top CJD expert with the CDC who is helping advise officials in Idaho. "But once it exceeds 1.5 or 2 per million, you start asking questions."
Authorities are conducting autopsies to determine if any of the Idaho victims had variant CJD, the type of CJD definitively linked with eating mad cows from which over 160 people have died in Britain and in Europe. If the Idaho autopsy results come back indicating sporadic, not variant CJD, there will be a collective sigh of relief. After all, so the conventional wisdom goes, people cannot get sporadic CJD from eating mad cow infected meat.
But increasingly and distressingly, the science suggests otherwise. As the months tick by, the science is increasingly highlighting the possibility that some cases of sporadic CJD might be linked to mad cow disease. Consider the following facts:
(1) In 2002 a bombshell paper from Professor John Collinge's research group provoked a stir in academia. The paper showed that genetically engineered mice, when injected with mad cow disease protein, unexpectedly produced a molecular profile that closely resembled sporadic CJD, as well as the expected variant CJD profile (ref 2). Collinge's group is a global leader in mad cow disease research. They followed up with confirmatory results in 2004 (ref 3) essentially saying the same thing. The consequence for humans was that mad cow disease might manifest as BOTH variant and sporadic CJD. Collinge's research suggests that maybe we should consider the possibility that some cases of sporadic CJD may arise from eating mad cow meat. This has implications for the Idaho CJD cluster.
(2) In Europe, new and bizarre forms of mad cow disease are being discovered as research accelerates. In Italy a new strain of mad cow disease was announced that bore striking molecular resemblances to sporadic CJD in humans (ref 4). This new strain was called Bovine Amyloidotic Spongiform Encephalopathy or BASE. Again the implication from the Italian research was a link between strains of mad cow disease and sporadic CJD. Could there be strains of mad cow disease present in the United States, that give rise to sporadic CJD, for example in the cluster of cases in Idaho? Nobody knows, because compared with Europe or Japan, only a tiny percentage of United States cattle are being tested for mad cow disease.
(3) Another angle being explored in Idaho is the possibility that some of the CJD victims might have eaten venison from chronic wasting disease (CWD) infected deer. CWD, the brain wasting disease in deer and elk, is caused by the same deadly prion that causes mad cow disease. CWD is currently spreading like wildfire through the United States. Can these deadly prions jump the species barrier to infect cattle or humans? Research from the United States Agriculture labs in Ames Iowa shows CWD can be transmitted from deer to cattle (ref 5). A few isolated and worrying cases of young hunters dying from sporadic CJD have been documented (ref 6), but the CDC says this was a statistical fluke.
What are the implications of this new scientific research for the 2005 CJD cluster in Idaho, for the 2004 CJD cluster in Ulster County, New York and for the New Jersey CJD cluster reported in 2003? The implications are that we should begin to look beyond variant CJD. We should begin to face the disturbing possibility that at least some cases of sporadic CJD in the United States might be caused by consumption of prion contaminated beef or venison. We should also begin to look for "atypical molecular CJD patterns, ie new molecular patterns that do not conform to EITHER sporadic or variant CJD.
Colm Kelleher is the author of Brain Trust: the Hidden Connection Between Mad Cow and
Misdiagnosed Alzheimer's Disease. http://www.colmkelleher.com. He is a senior research scientist in the biotechnology sector.
(1) Tanner, Adam (2005). Idaho probes sudden deaths from rare brain disease. Reuters. August 15, 2005.
(2) Asante, EA et al (2002). BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO Journal, 2002 Dec 2;21(23):6358-66.
(3) Wadsworth JD et al (2004). Human prion protein with valine 129 prevents expression of variant CJD phenotype. Science. 2004 Dec 3;306(5702):1793-6. Epub 2004 Nov 11.
(4) Casalone C et al (2004). Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A. 2004 Mar 2;101(9):3065-70. Epub 2004 Feb 17.
(5) Hamir AN et al (2005). Experimental transmission of chronic wasting disease agent from mule deer to cattle by the intracerebral route. J Vet Diagn Invest. 2005 May;17(3):276-81.
(6) Belay ED (2002). Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol. 2001 Oct;58(10):1673-8.



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