nvCJD in Florida?
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nvCJD in Florida?
Hansen's report on Florida CJD case
New style CJD sufferers demand public probe
News from Steve Dealler's site
Experts to watch for signs of CJD epidemic
Grisly progress of the new CJD

nvCJD in Florida?

10 Sept 97  BBC
BBC and Channel Four have been carrying reports of a case of nvCJD (mad cow disease crossed over to people) in the United States. The case reportedly involves a 51-year old man in Florida with confirmed CJD (14-3-3 test) and symptoms including dysaesthesia, choreoform movement and coma. The family of the deceased declined to permit a tonsil or spleen biopsy, EEG, or autopsy.

A group of Florida neurologists has recently reported an excess of CJD cases in the Tampa area (after adjusting for an elderly population), 13 cases in 18 months. The victim was a patient of Michael Gold, MD in Gainesville, a former colleague of Joseph Berger, MD, now doing squirrel brain - CJD studies at the University of Kentucky. Both doctors have been associated with a patients' group in Florida called the CJD Foundation. Mayra Lichter of that group suggests that the connection looks good.

MAFF took the line that a case of nvCJD in the US proved that nvCJD could not be from BSE because this strain has not been acknowledged to even occur in the US. This was evidently a gambit to offset the upcoming Moira Bruce report confirming the BSE-nvCJD by classical strain-typing as well as a rumored report on infectivity of milk. The nvCJD specialists in England have been scrambling like everyone else to find out more details of the Florida case.

Michael Hansen, the CJD expert at the Consumer's Union has been following this case closely and concluded that the evidence is insufficient at this time to reach any definitive conclusion as to what type of CJD is involved here.

New style CJD sufferers demand public probe

September 10, 1997 PA News Paul Peachey
In a letter to the New Statesman magazine, 16 of the 21 families who had relatives who contracted new-variant CJD today called for an independent public inquiry into the BSE saga. They were joined by Professor Hugh Pennington who led the E. coli outbreak inquiry in Scotland. In the letter they warned that a large proportion of Britons may have been exposed to the risk of infection by eating beef.
The letter said: "No one can venture an accurate estimate of the number of cases of this grim disease which can be anticipated in the next five, 10 or 15 years."
It also said that two successive governments had not instituted any inquiry into how BSE arose in cattle or how new-variant CJD has got into the food chain. The families said a decision was expected by Agriculture Minister Dr Jack Cunningham soon on a public inquiry. "The minister, and those scientists and clinicians advising him, are bound by government protocol to remain silent on this issue. We are not.
"In the interest of those who feed Britain, those who are fed and most of all the victims of this terrible crisis, we demand a public judicial inquiry."

News from Steve Dealler's site

1. 'Biggest public failure since the Suez crisis'
This was a quote of Eric Millstone, of Susssex University's science policy research unit at the major meeting organised by the Transport and General Union in the UK about how the Government in the UK should procede. The main things that seemed to come out from the meeting was that the loss of public confidence in the Government's position on food had to be restored.

The Consumers Association representative showed how the population in the country no longer believed what the MAFF said about the safety of food and hence a new body had to be set up with powers to ensure the safey of food. Professor James, who had produced a document for a Food Standards Agency that would carry out this was applauded. MAFF response was that they all did not understand the real problems at the time.

The main damning data that appeared in this was Millstone's description that members of the Advisory Committee on Pesticides and the Veterinary Products Committee would arrive at MAFF to discuss and produce advice simply to find the minutes of the meeting already made by MAFF who had decided what the committee should advise in advance after discussion with the Minister. (Same story from other sources - webmaster).

2. Certain change in the wake of rendering aid phase-out.
The loss of much more aid to the rendering industry seems to be simply transferring the costs onto the farmer or the abattoir. They in turn seem to be simply putting up the price of their product.

3. Reports on chicken TSE from MAFF being argued.
Apparently Harash Narang's chicken with spongiform change and amyloid plaques had its brain removed in official circumstances and sent to MAFF. When it got there the neuropathologist 'claimed that it was not from a chicken', 'suggested that the photographs of the brain under the microscope were not from the sample sent', and that the sample did not have any spongiform change etc. Then MAFF has published an article in the Veterinary Record saying that they have no evidence that chickens have any TSE. This sounds to Narang as if someone has fiddled the samples but what is certain is that the original slides that Narang took were from the chicken and that the photos were valid. More argument is on the way.

Hansen's Report on Florida CJD case

Monday 15 Sept 1997 posting
This is a report on the supposed nvCJD case in Southern Florida that the BBC reported on this past week. The reporters clearly got the story wrong. I talked on Thursday with Dr. Michael Gold of the University of South Florida. The suspect nvCJD patient is one of his patients as he is studying a cluster of CJD cases in Southern Florida. The cluster consists of 13 cases--11 of which have been confired at autopsy, with 2 still alive--in Southern Florida over a 20 month period. The CJD rate for the whole state is 15 cases a year. Even if you control for the age of the population [many elderly people live in south Florida], there still is a higher incidence that the 1 in a million prediction.

The suspect nvCJD patient is a 51 year old male who showed some unusual symptomology--a lot of chorioform movement and dysesthetic pain--that has also been seen in nvCJD cases. When asked if the patient had the characteristic EEG pattern seen in nvCJD cases (where the abnormal triphasic EEG is seen only in the end stages not throughout the course of the disease as in sporadic CJD), Gold responded that he couldn't tell as the patient came to him in a coma and was in the final stages of disease. After the patient died, the family refused an autopsy so there was NO confirmation that this was a case of nvCJD. As far as Dr. Gold is concerned the case is closed. All we know is that this is a case of CJD with some unusual symptomology.

To think that garbled reporting on this case led the MAAF to announce that there has been a confirmed case of nvCJD in the US which proves that BSE can't be the cause of nvCJD is just mind boggling. Don't good reporters try to confirm their stories before reporting on them? As Dr. Gibbs would say, this looks like a case of "mad media."

Michael Hansen
Consumer Policy Institute/Consumers Union
101 Truman Ave.
Yonkers, NY  10703-1057
ph:  914-378-2452

Experts to watch for signs of CJD epidemic

PA News Mon, Sep 15, 1997 By John von Radowitz, Medical Correspondent, PA News
A special panel of experts has been set up to keep track of any emerging epidemic of CJD cases thought to have been caused by eating BSE-infected beef, the Government announced today. The move coincided with a rise in the numbers of "new variant" cases of Creutzfeldt-Jakob Disease, or nvCJD.

A total of 23 have come to light since the new strain became known in March last year, although the official figure stands at 21. All the current victims are believed to have been infected through eating meat from cattle suffering from mad cow disease before the Government's ban on high-risk beef offal came into force in 1989. But no one knows how many more people are likely to become ill with the disease in the future.

The worst-case scenario is that the current numbers suffering from nvCJD are just the beginning of a serious epidemic of a disease with a long incubation period. Some experts fear, if this is true, the final number of victims could run into hundreds of thousands.

Against this background, Chief Medical Officer Sir Kenneth Calman today announced the formation of a new sub-group of the Spongiform Encephalopathy Advisory Committee (SEAC) to look at the prevalence of nvCJD. Sir Kenneth said:

"There is still a lot we do not know about this disease, including important information on the incubation period, the route of infection, the level of exposure required to cause disease and the role of genetic susceptibility.

"With the agreement of its chairman Professor John Pattison, I have decided to set up a sub-group of SEAC to assess the information about the epidemiology of nvCJD and develop as far as possible advice on trends in the disease."

Asked if the move reflected growing concern over the pattern of nvCJD incidence, a Department of Health spokesman said:
"What it reflects is our and SEAC's belief that a dedicated group is best able to look at all of the new research coming forward, and to produce more informed reports on the likely shape of the epidemic."
Initially 10 cases of nvCJD were reported. The disease attacked people who were much younger than the normal victims of CJD and produced distinct symptoms and changes in the brain. The figure rose to 16 by the end of January this year and then remained stable, just one more case being added in April. Then in May two more cases came to light, followed by a further two over the next two months.

The latest official tally is 21 confirmed and "probable" - where the diagnosis has not been verified by a post mortem or biopsy - cases of nvCJD. But two more cases, not included in the official update, have recently attracted publicity. One was that of Clare Tomkins, 24, from Tonbridge, Kent, a vegetarian who had not eaten meat for 11 years. The other was 36-year-old fitness fanatic Chris Warne of Ripley, who was named as Britain's 23rd nvCJD victim.

Grisly progress of the new CJD

PA (PA News) Mon, Sep 15, 1997  By John von Radowitz, Medical Correspondent, PA News
A panel of experts is to track the new variant of CJD, the brain disease thought to be caused by eating BSE-infected beef. The move come amid signs that the number of cases of the new strain is increasing:

-- Creutzfeldt-Jakob Disease, like BSE, is a poorly understood condition that attacks the brain, leaving it full of holes like a bath sponge. Victims display symptoms of dementia, becoming unsteady, confused and losing their memory. Death normally occurs about six months after onset.

-- Last year 10 cases of a new strain of CJD were discovered and set alarm bells ringing. They had characteristics strikingly different to any known before -- victims were much younger than normal, had unusual symptoms of depression and anxiety, and had the disease for a year or more before dying. The disease also attacked their brains in an unusual way, producing tell-tale "plaques".

-- In March last year the Government said it had accepted scientific advice that the new strain was probably caused by eating infected beef. Since then experts have become more and more convinced that this is the case.

-- The number of official recorded cases of new variant CJD rose from an initial 10 to 14 in October last year, 16 by the end of January this year, 19 in May, and 21 in July. Two further cases have come to light but are not yet included in the official figures. -- BSE -- bovine spongiform encephalopathy -- was first diagnosed in November 1986. Since it emerged, scientists and consumers have been haunted by the thought that it could be transmitted to humans.

-- In 1986 certain cattle offals known to have the highest risk of BSE infection were banned from human consumption, even though at that time there was no firm evidence of a threat to humans. The ban included the brain, spinal cord, the spleen and lymph glands. -- In 1994 The Government's chief medical officer, Dr Kenneth Calman, attacked "irresponsible scare stories" when reports suggested that a 16-year-old girl might have caught CJD after eating a contaminated burger.

-- In November the following year, neuropathologist Sir Bernard Tomlinson announced that he was no longer eating products likely to contain beef offal, such as meat pies, pates and consomme, because of the "unjustifiable risk".

-- The fears were vindicated in March last year when the then-Health Secretary Stephen Dorrell made a statement to the House of Commons admitting for the first time that there was a probable link between BSE and new variant CJD. Previously he had said there was "no conceivable risk" in eating beef. Because of the length of the incubation period, people with the new strain are thought to have caught it before the 1989 offal ban.

-- No-one yet knows how big the new variant CJD epidemic will be. If the peak is occurring now, it is good news. But if the end of the incubation period for most victims has not yet been reached there could be a sudden flood of new cases. The worst case scenario is an epidemic as bad as Aids and HIV, claiming up to 500,000 victims early in the next century.

-- BSE is thought to have been triggered in cattle after they were fed remnants of sheep affected by a similar disease, scrapie. It is possible BSE then jumped the species barrier to produce new variant CJD in humans.

-- All these diseases are thought to be caused by a kind of protein called a prion which has been altered to make it dangerous. But the mode of transmission is still largely a mystery.

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