-Caveat Lector-

http://www.guardian.co.uk/bse/article/0,2763,849293,00.html

BSE may have caused second strain of CJD

James Meikle, health correspondent
Thursday November 28, 2002
The Guardian

Measures to protect the public from BSE-like diseases were called into serious 
question last
night as researchers suggested that the BSE epidemic in cattle might have caused two
separate fatal brain conditions, not one as thought.

Their stunning conclusion, that eating cheap cow meat might be responsible for some 
cases
of sporadic CJD, as well as variant CJD 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, 
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 this is not related to what you read in the newspapers, 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 fitted with 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 three strains 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.

Haemophiliac 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 haemophiliacs 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."

The diseases compared

Age when obvious symptoms occur after many years of incubation:

Sporadic
Identified in 1920s. Disease mainly of middle-aged and elderly. Incidence roughly one 
in a
million, between 29 and 63 a year since 1990. Found worldwide

vCJD
Identified in 1996. Average age late 20s. Found mainly in Britain: 129 cases with 117
deaths so far. Six in France, 1 each in Italy, Ireland, Canada, and United States

Cause:

Sporadic
Until now assumed a normal prion protein in the brain spontaneously changed into
abnormal dangerous form. Recently suggested some cases possibly caused by surgical
contamination during operations. BSE or similar diseases could now be factor

vCJD
Largely blamed on consumption of cheap cattle meat and offals during 1980s. Tough food
controls meant to have significantly reduced risk. Concern remains over whether sheep
might also have become infected with BSE and entered food chain

Duration of illness and symptoms:

Sporadic
Time between obvious symptoms and death typically a few months. Loss of balance, sense
of direction, and control over limbs and bodily functions. Fear, anxiety

vCJD
First symptoms often indicate psychological problems. Duration often well over a year.
Several physical symptoms similar to sporadic CJD

Guardian Unlimited © Guardian Newspapers Limited 2002

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