- Hello Jeff - This is very important information. It
means that the cause of SOME cases of 'traditional' or 'normal' CJD may
be a direct result of eating BSE-infected meat. Until now, medical science
has insisted that 'traditional' CJD has been around for a number of years
and was NOT caused by Mad Cow/BSE meat...which is the KNOWN cause of what
is called vCJD (variant) in humans.
- If we take this one LOGICAL step further, we might be
inclined to theorize that some cases of CJD and sporadic CJD might have
been the direct result of eating Chronic Wasting Disease infected deer,
elk and moose.
- The cases of CJD and sCJD resulting from CWD would be
cases in the US. The CDC and other authoritarian organizations which claim
the US does not have any BSE "mad cow" disease in its beef or
dairy industry are NOT correct if one considers the above hypothesis as
it pertains to Chronic Wasting Disease and sCJD and CJD resulting from
eating the CWD-infected meat.
- Patricia Doyle
- BSE May Have Caused Some Cases Of CJD As Well
- By James Meikle
The Guardian - UK
- Measures to protect the public from bovine spongiform
encephalopathy (BSE)-like diseases were called into serious question last
night as researchers suggested that the BSE epidemic in cattle might have
caused 2 separate fatal brain conditions, not one as thought. Their stunning
conclusion, that eating cheap cow meat might be responsible for some cases
of sporadic Creutzfeldt-Jakob disease (CJD), as well as variant CJD [abbreviated
as vCJD or CJD (new var.) in ProMED-mail] or human BSE, will cause further
reassessment of risks still posed by food and by infection through cross-contamination
of surgical instruments and blood.
- Experiments with mice by John Collinge and colleagues
at University College London appear to strengthen the possibility that
more animals, and by extension humans, can act as infective carriers of
the killer diseases well before full-blown symptoms occur. They cast another
shadow over the safety of sheep, reinforcing concerns that they were infected
with BSE as well as scrapie, a disease not known to be harmful to humans,
and that one disease had "masked" the other.
- The Department of Health will have to reconsider the
operation and size of its compensation scheme for families that have a
human BSE sufferer. The money is not available for sporadic CJD victims
or relatives, and there is no way yet that scientists can distinguish between
cases that arose from spontaneous changing in the form of the prion protein
linked to both diseases, and those that might be diet-related. The research,
outlined in the journal of the European Molecular Biology Organisation
(EMBO) [see comment below], could mean huge changes in the counselling
of sporadic CJD patients and their families. Professor Collinge said last
night: "When you counsel those who have the classical sporadic disease,
you tell them that it arises spontaneously out of the blue. I guess we
can no longer say that."
- The results come from a continuing long term study of
laboratory mice [engineered to express] the human prion protein, then injected
in the brain with BSE-infected material. Some showed a molecular signature
indistinguishable from human BSE; others a signature the same as that left
by one of 3 forms of sporadic CJD.
- Deaths from sporadic CJD reported in Britain in the 1990s
peaked at around 60 a year between 1997 and 1999, far more than the 28
variant CJD cases in 2000, the worst year so far for variant CJD. Urgent
reviews will be made as to whether some sporadic cases might be BSE-related,
although hard evidence would be extremely difficult to find. The pattern
of infectivity through the body is markedly different between sporadic
CJD and variant CJD, meaning surveillance might now become more complicated.
- There are no blood tests yet available. Hemophiliac patients
who were treated with blood clotting factors by the Scottish blood transfusion
service between 1987 and 1989 are being informed that one donor of blood
for the concentrates had later contracted variant CJD. All hemophiliacs
in Scotland are now treated with genetically engineered substitutes, but
these are not universally available in England, where warnings of possible
contamination have been issued several times. The Haemophilia Society last
night said it would increase pressure on the Department of Health to ensure
that alternatives were available to patients in England.
- Gillian Turner, of the CJD Support Network, said: "This
research will be welcomed by many families who have been affected by sporadic
CJD. They have been concerned for a long time that it was diet-related."
Lester Firkins, of the Human BSE Foundation, said: "This could throw
all the work that people have been doing on modelling [the possible spread
of diseases] up in the air again. You might see clustering of cases if
you have more numbers."
- Sporadic CJD was identified in 1920s. The disease affects
mainly the middle-aged and elderly. The incidence is roughly one in a million,
between 29 and 63 a year since 1990. The disease is found worldwide. Time
between obvious symptoms and death is typically a few months. Until now
it has been assumed that a normal prion protein in the brain spontaneously
changed into abnormal dangerous form. BSE or similar diseases could now
- vCJD was identified in 1996, with the average age of
onset in the late 20s. Found mainly in Britain: 129 cases with 117 deaths
so far, plus 6 in France, 1 each in Italy, Ireland, Canada & the USA.
Largely blamed on consumption of cheap cattle meat and offal during 1980s.
Tough food controls were meant to have significantly reduced risk. Concern
remains over whether sheep might also have become infected with BSE and
entered the food chain. First symptoms often [imitate] psychological problems.
Duration is often well over a year. Several physical symptoms are similar
to sporadic CJD.
- ProMED-mail <email@example.com>
- The scientific paper upon which this report is based
is published in the current issue of the EMBO Joural, Vol. 21, No. 23,
- Title: BSE prions propagate as either variant CJD-like
or sporadic CJD-like prion strains in transgenic mice expressing human
- Authors: Emmanuel A. Asante, Jacqueline M. Linehan, Melanie
Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew
F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth and John Collinge. MRC
Prion Unit and Department of Neuro-degenerative Disease, Institute of Neurology,
University College, Queen Square, London WC1N 3BG, UK.
- Abstract: Variant Creutzfeldt-Jakob disease (vCJD) has
been recognized to date only in individuals homozygous for methionine at
PRNP codon 129. Here we show that transgenic mice expressing human PrP
methionine 129, inoculated with either bovine spongiform encephalopathy
(BSE) or variant CJD prions, may develop the neuropathological and molecular
phenotype of vCJD, consistent with these diseases being caused by the same
prion strain. Surprisingly, however, BSE transmission to these transgenic
mice, in addition to producing a vCJD-like phenotype, can also result in
a distinct molecular phenotype that is indistinguishable from that of sporadic
CJD with PrP\Sc type 2. These data suggest that more than one BSE-derived
prion strain might infect humans; it is therefore possible that some patients
with a phenotype consistent with sporadic CJD may have a disease arising
from BSE exposure.
- In an interview with the London Times Health Editor,
Professor Collinge stated that the number of cases of sporadic CJD cases
had been increasing at about the same rate as the vCJD figures, and that
the Swiss had reported recently a 2 to 3 fold increase in sporadic CJD.
He also expressed concern that the transgenic mouse experiments had revealed
instances of subclinical infection; although appearing normal in life,
these mice when examined post-mortem exhibited extensive brain lesions.
Subtle changes in mice are hard to detect, but changes might be more obvious
in humans in terms of psychiatric symptoms. A subclinical form of CJD in
humans would increase the hazard of transmission of disease via surgical
- Professor Collinge stated that it was now a matter of
urgency to conduct a large scale study of tonsil tissue in order to establish
how widespread CJD infection is. He said that he remained concerned about
the ultimate size of the CJD epidemic. The tonsil study that has been
carried out so far employed a relatively crude test. He stated that new
cases of kuru, the neurological disease caused by cannibalism some 50 years
ago are still coming to light. Consequently these are very early days for
a human prion epidemic, which could have a 30 year incubation period. -
- [see also: CJD, increased incidence - Switzerland 20020714.4756
CJD, long incubation period 20020612.4478 CJD, surgical instrument re-use
- UK 20021030.5671 CJD, suspected cluster - USA (Wisconsin) 20020721.4827
CJD (new var.) - UK: 10th Annual Report 20020711.4727 CJD (new var.) -
UK: update Sep 2002 20020908.5258 2001 ---- CJD, death rate increase -
Switzerland 20011225.3110 CJD, surgical transmission? - Canada (Ontario)
20010512.09 2000 ---- CJD, possible surgical transmission - USA (Louisiana)