Editorial : Tragedy of variant Creutzfeldt-Jakob disease

[Guest guest]

Dear All,

I've not been able to read all the mail that came to CJD Voice and CJD

Blood in

my recent absence, but am passing on the following editorial from The Lancet,

March 20, 1999, on the off-chance that you may have missed receiving it.

Best wishes etc., Lynette.

http://www.thelancet.com/newlancet/reg/issues/vol353no9157/body.editorial9

39.html>http://www.thelancet.com/newlancet/reg/issues/vol353no9157/body.edi

torial939.html

THE LANCET, Volume 353, Number 9157 20 March 1999

 

Tragedy of variant Creutzfeldt-Jakob disease

The unfinished tragedy of variant Creutzfeldt-Jakob disease (CJD) in the UK

begins with a distasteful practice; progresses through protracted scientific

investigation, hampered by political farce; and culminates in continued

uncertainty--is there to be the massive epidemic of variant CJD that was

predicted in 1996, or is the scare a product of an imaginative diagnostic

definition and doom-mongering statistical prognostication?

For physicians unconcerned by these questions, because the prospect of variant

CJD affecting themselves or their patients appears remote, or because the

number of deaths to date (39) is trivial compared with the toll of more common

diseases or of natural disasters, it is worth briefly reviewing the story of

variant CJD so far: there are lessons to be learned by physicians and

politicians worldwide.

A spongiform encephalopathy affecting cattle (BSE) in the UK was identified in

1986. To the end of January, 1999, 173 718 cases had been recorded in the UK.

BSE, which is pathologically similar to a spongiform encephalopathy of sheep

(scrapie), was attributed to the feeding of sheep offal to cattle. This

distasteful practice was banned in 1988. Notifications of BSE decreased,

but in

January this year there were still 297 reported new cases. After considerable

public disquiet, a BSE inquiry was convened in March, 1998, and was due to

report in June, 1999. The inquiry will , predictably, not report on time.

Meanwhile, in March, 1996, a unit set up to monitor spongiform

encephalopathies

in human beings (the CJD Surveillance Unit, Edinburgh, UK) reported ten cases

of CJD (variant CJD) which appeared to be clinically and pathologically

distinct from sporadic and other varieties of CJD (Lancet 1996; 347:

921-25). An

understandable suspicion that eating BSE-infected beef was to blame for

variant

CJD provoked mayhem: sale of UK beef was banned throughout the European Union;

millions of UK cattle were slaughtered; and the UK public were treated to the

unedifying spectacles of a senior politician affirming his belief in the

safety

of UK beef by feeding hamburgers to his children.

First signs of scientific proof that BSE may be responsible for variant CJD

appeared in 1997, with a report that infective prions associated with BSE and

with variant CJD appeared identical. Still, the vaunted epidemic in human

beings failed to materialise. Notifications to the CJD Surveillance Unit

varied

(insignificantly) from zero to four per quarter from the first quarter of 1995

to the third quarter of 1998.

Then there was a disquieting change in notification rate,

reported in a fast-tracked

Research letter in this week's issue (p 979) . In the last quarter of 1998,

there were nine deaths from variant CJD notified to the Surveillance Unit.

This

increase is statistically significant, but it is by no means conclusive

evidence that people who were infected by eating beef before sheep offal was

banned from the foodchain are now reaching the end of their variant-CJD

incubation periods and that worse is to come. Curiously, this information on

notifications of variant CJD has been in the public domain for over 2 months

without arousing comment. A glance at the graph might lead even the most

statistically-naive observer to wonder if something unusual is happening.

Perhaps scientists, doctors, and journalists are tired of a topic that since

the furore of 1996 seems to have degenerated into political pointscoring.

They should not tire of the topic. Whether there is a drastic epidemic of

variant CJD in the offing, or whether there is not, does not alter the

take-home warning messages of the tragedy. The first is that to feed

cattle--ruminants equipped efficiently to digest only grass and other

fodder--neural tissue from a species known to be prone to an endemic brain

disease is a dangerous folly. The second, that when a threat is posed to the

public health, precipitant reactions by politicians without any proper attempt

either to inform themselves or the public is counterproductive.

The outlook, from many aspects, is grim. In the UK, the BSE inquiry will

almost

certainly publish an anodyne report replete with hand-wringing but conclude

that no one is to blame. Worldwide, animal-feeding practices will continue to

be driven by the prospect of a quick profit and not by considerations of sound

animal husbandry. Clinicians, though, now have at least one good reason to

maintain a high index of suspicion concerning unusual presentations of

diseases, and to support strict surveillance of these when they occur.

The Lancet