A new human genotype prone to variant Creutzfeldt-Jakob diseaseBMJ 2006; 332 doi: https://doi.org/10.1136/bmj.332.7551.1164 (Published 18 May 2006) Cite this as: BMJ 2006;332:1164
All rapid responses
I was surprised by the total absence of posted responses to either
this editorial or the associated original research paper (1) when I first
looked and still there are none.
If it really is an important topic, are readers not interested, apathetic
or perhaps beyond commenting as the juggernaut seems to roll on under its
The financial and social burden consequent upon Bovine Spongiform
Encephalopathy (BSE) and New variant Creutzfeldt-Jakob disease (vCJD) ,
already huge, will continue to increase significantly if, as a society, we
persist in basing our overall management on what may be a false premise
and incur both direct and, perhaps more importantly, indirect extra costs.
The false premise in question is that the condition vCJD is simply caused
by a transmissable infectious agent to support which there was (2) and, I
would contend, still remains, a very poor standard of evidence on the
basis of which the precautionary actions being taken are perhaps, rather,
excessive and out of proportion (3).
Dire predictions for the future were, and continue to be, based upon
this supposed risk to humans. A language of fear is used, all of it
predicated upon there being a transmissable infectious agent; makes you
worry just to read…..tramsmit, asymptomatic carriers, epidemic, iatrogenic
spread, …new evidence ‘may rekindle fears of a larger epidemic....an
ongoing threat. Designed to create a climate of acceptance, much like the
‘war on terror’.
We should, in fact be very cautious in interpreting the results of
the study which simply demonstrated 3 out of 12674 samples as containing
prion protein; full stop! No need to suggest that the true prevalence
should be higher or overinterpret the data or put the frighteners on with
‘secondary spread’ from surgery or blood transfusion (1) or that single
cases should have major implications for future estimates of vCJD in the
161 cases overall is not a major problem; why are the findings of the
new study so worrisome? With so few cases, perhaps part of the problem,
shocking as it may be, is that (bad for the industry) there in fact is no
future for the vCJD ‘epidemic’ (5) and other possible causes are not
considered. We do need to continue surveillance, investigate and diagnose
both clinical and pre-clinical forms of the disease (4) but not while
Prion proteins exist but we still need to find out why, exactly and
quite specifically, in the 1980s BSE hit the UK. Something happened then
which probably isn’t applicable now. Perhaps it’s simply down to a
susceptibility or priming based upon genetic (and other) factors and
animals (and humans) become AFFECTED rather than INFECTED, a certain
‘load’ of prion protein affecting susceptible patients at risk.
Carcinogens are not (with a few exceptions) infectious agents but produce
the disease in susceptible individuals after very variable rates or
degrees of exposure.
Society is becoming more risk averse, risk elimination rather than
proper management the goal, and encouraged to be so by issues such as this
and the health and safety industry in general. We are being made fearful
of each other and living.
The need remains to challenge the current orthodoxy and overcome the
vested interest and status quo surrounding vCJD. Risk aversion is the
illness and hyper-caution the epidemic (6).
1. Ironside JW, Bishop MT, Connolly K, Hegazy D, Lowrie S, Le Grice M
et al. Variant Creutzfeldt-Jakob disease: prion protein genotype analysis
of positive appendix tissue samples from a retrospective prevalence study.
BMJ 2006; 332: 1186-8.
2. Venters GA. New variant Creutzfeldt-Jakob disease: the epidemic
was. BMJ 2001; 323: 858-861.
3. Wilson K, Ricketts MN. Transfusion transmission of vCJD: a crisis
avoided? Lancet 2004; 364: 477-9.
4. Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical
vCJD after blood transfusion in a PRNP codon 129 heterozygous patient.
Lancet 2004; 364: 527-9.
5. Aguzzi A, Glatzel M. vCJD tissue distribution and transmission by
tranfusion – a worst-case scenario come true? Lancet 2004; 363: 411-2.
6. Jenkins S. Don’t panic – or our culture of caution will be the
death of us. The Sunday Times June 11, 2006; 1:18.
Competing interests: No competing interests