First hundred cases of variant Creutzfeldt-Jakob disease: retrospective case note review of early psychiatric and neurological features
BMJ 2002; 324 doi: https://doi.org/10.1136/bmj.324.7352.1479 (Published 22 June 2002) Cite this as: BMJ 2002;324:1479
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Sir,
Has John Fryer [1] not even heard of Mark Purdey and his interesting
ideas about the origin of CJD?
In case he hasn't, then he should look at the new web page from the
Taunton farmer behind that news item:
http://www.purdeyenvironment.com/
It is a hypothesis remarkably close to the comments John Fryer makes.
[1] BMJ letter, vCJD - some thoughts, John Fryer (26 June 2002)
Competing interests: No competing interests
The work of Professor Will and his team is world famous and unique.
I would ask whether there is any chance of a chemical cause of vCJD.
The most likely candidate being OP toxic nerve agents.
The increase in their use could explain the rise of not just vCJD,
which is a very specific illness, but also many other more common
illnesses.
Chemicals have a wide action and the combination of such a high
exposure for many years could easily explain the condition if we assume
past cases have escaped notice.
The predictable outcome of such a hypothesis would be that control
and epidemics are manageable - so it is important to be sure there is no
pesticide (from animal flea products in shop situations or direct use) or
medical connection (head lice control).
The dramatic but temporary nature of phenothiazine on the
presentation of neurological symptoms is a case in point as it acts on the
same biochemical sites as OP type substances so could be predicted to
have some effect. If a true infection then why have an effect or just a
temporary effect. The role of phenothiazine seems reminiscent of the
initial and spectular success of L-Dopa as portrayed in the film -
Awakenings.
In respect of protein folding - are the researchers aware of the role
of antibiotics and chemical mimics in preventing fromation of mRNA and the
subsequent protein synthesis - puromycin, cyclohexamide et al. This in
conjunction with associated ion change - eg raised sodium ions leads to
deformation of protein structures. Work of generations ago and analogous
to work of Prusiner and his team on the prion protein.
John Fryer Chemist
Competing interests: No competing interests
In iatrogenic CJD direct infection of the brain by an infected needle
or dural graft had a much shorter incubation period than distant
intramuscular injection of cadaver growth hormone*.
Thus the first cases of variant CJD should be those caused by direct
infection - if this has occurred at all.
Mental disturbance was usually the first sign of iatrogenic CJD by
direct infection, and cerebellar unsteadiness of distant infection.
The finding that psychiatric signs appeared before physical in the
variant CJD so far seen suggests that there may indeed have been direct
infection of the brain by BSE. This may account for their small number and
perhaps for the age of the patients.
It may presage a clinically different and wider epidemic when a
second incubation period is over.
*Brown P, Preece M, Will R. 'Friendly fire' in medicine. Hormones,
homografts and Creutzfeldt Jakob disease. Lancet 1992;340:24-7.
Competing interests: No competing interests
First hundred cases of variant Creutzfeldt-Jakob disease
Editor
"Dreading Richard Smith's editorial stiletto,1 I hesitate to cavil at
the BMJ's processes guiding selection of what it publishes. But I
wonder why this paper was. It is a descriptive study of a series of cases
of a disease which is not new, by inference seeks to prop
up a flawed hypothesis and states the blindingly obvious."
Creutzfeldt’s eponymous case2 fits readily into the set of clinical
features characteristic of "new variant" Creutzfeldt Jakob
disease. So do most cases of sporadic CJD – especially the younger ones3
and many cases of kuru.
What the paper shows is that patients with psychiatric symptoms get
referred to psychiatrists and if they develop neurological
features are referred to neurologists – hardly surprising.
Slightly more surprising is, yet again, the assertion that there is
"compelling evidence of the link", (implied as causal), between the
BSE agent and variant CJD. This evidence amounts to the frequent
reiterations of a plausible explanation, assiduous selection and
pursuit of studies which might support it and the ignoring or dismissal of
contrary evidence which continues to accumulate.
The number and range of electronic responses (as yet unpublished) to
my paper gives an indication of the fragility of the current
orthodoxy and some legitimate alternative explanations of the BSE epidemic
and detection of cases of variant CJD. Some are
equally if not more plausible than the prion recycling hypothesis and
merit detailed research.
Spongiform encephalopathy is a pathological end state accompanied by
a spectrum of clinical features and likely to result from a
range of causes. For humans, eating bovine prions is not one of them.
There is no epidemic and little prospect of one5. Improved
ascertainment and categorical shifts are the likeliest explanations of the
rediscovery of this putatively new disease.
Locking on to a crumbling hypothesis and defending it as a faith does
science a disservice. Science is about opening doors not
closing them. It is time to move on, to encourage and support research
into other possible causes of spongiform encephalopathy on
equal terms with prion based ones.
1.Smith R. Shooting the Messenger, Editor’s Choice. BMJ 2002;324
(29th June)
2.Creutzfeldt HG Uber eine Eigenartige Erkrankung der
Zentralnervensystems. Zeitschrift f.d. gesamte Neurologie u Psychiatrie 1920;57:1-183
3.Belay E D, Gambetti P, Schonberger LB, Parchi P, Lyon D R et al.
Creutzfeldt-Jakob Disease in Unusually Young
Patients Who Consumed Venison.
Arch Neurol.2001;58:1673-78
4.Venters G. New variant Creutzfeldt-Jakob disease: the epidemic that
never was
BMJ 2001;323:858-61
5. Monthly Surveillance Figures for Creutzfeldt-Jakob Disease. SCIEH
Weekly Report ;36 No 2002/22:147 (Scottish Centre
for Infection and Environmental Health, Glasgow. Web
Address:http://www.show.scot.nhs.uk/scieh/)
Competing interests: No competing interests