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Rare Brain Illness Kills San Rafael Woman; 2nd Marin Case Diagnosed

SAN RAFAEL (KCBS) – A Marin County resident has died from – and another infected with – a brain disease related to what is sometimes incorrectly referred to as ‘mad cow disease.’

The woman who died had contracted Creutzfeldt-Jakob disease and was identified by the Marin Independent Journal as 59-year-old Aline Shaw of San Rafael.

Creutzfeldt-Jakob disease (CJD) causes a dementia that progresses more rapidly than others such as Alzheimer’s disease or vascular dementia. Patients eventually go into a coma and die.

The variant form of CJD is the human version of bovine spongiform encephalophathy (BSE), commonly known as ‘mad cow disease.’  BSE is a progressive neurological disorder of cattle that become infected with an abnormal protein in the meat and bone meal they are fed, according to the Centers For Disease Control and Prevention. Humans get the variant version of CJD when eating the meat of animals infected with BSE.

Shaw passed away suddenly on Jan. 27 from non-variant – or classic – form of Creutzfeldt-Jakob disease, according to the Marin County Health Dept. The non-variant form has only two known causes: genetic mutation and contaminants introduced during a medical procedure, according to Dr. Craig Lindquist, Marin County’s interim public health officer.

Lindquist said state officials notified him of the two cases on Friday. Lindquist could not identify the victims, but confirmed to the newspaper that one had died.

BSE spread among cattle in Great Britain and peaked with almost 1,000 cases a week in 1993, according to the CDC. Through the end of 2010, more than 145,500 cases were confirmed among more than 35,000 herds.

Lindquist said there is no link between the two Creutzfeldt-Jakob cases in Marin County, and it is not clear which form of the disease is afflicting the living victim.

“There is no threat or danger. Beef is very safe to eat,” Lindquist said.

Experts at the Centers For Disease Control and Prevention are aware of only 3 previous U.S. cases of variant Creutzfeldt-Jakob disease. CDC officials said those earlier victims were all exposed to BSE while abroad.

“We know that there have been no mad cow diseases originating in the United States,” said Dr. James Cullor, director of the UC Davis Veterinary Medical Teaching Center.

“The processing system, the surveillance system from the farm all the way through, is vigilant,” he said, making a local outbreak of mad cow very unlikely.

Marin County officials have yet to determine the source that infected the two Bay Area victims.

(Copyright 2012 by CBS San Francisco. All Rights Reserved. This material may not be published, broadcast, rewritten, or redistributed.)

More from Holly Quan
  • tr

    My dad just died of this disease, he is from the Bay Area and he has not been over seas. Please look into this, very concerning!

    • eaamon

      tr, this is concerning. my question is where did he eat?
      restaurant or fast foods? or what store did he buy beef at?
      several years ago there was a case of about 15 people who ate at the same golf clubhouse. steaks were the big thing on their menu. they all died from it. but when one relative started checking into it found the connection. how ever the club shut down several years before her research…..owners and restaurant manager all died. she could never find out where they bought their beef. a dead end…….

      • Barbara

        Eaamon, please tell me in which county these 15 people lived. I’m concerned always about food safety, especially after losing my dad to CJD. There was a group of people who died from CJD in Butte County the same year he died.

      • eaamon

        hi, I am not sure what county it happened in it was back in the 1990s I think. it was near new york new jersey area. I think the gal researched when her dad died of it. traced it to several families who also had members who died of it but never made the connection. she found out they all were golfers,same club, except one. i believe was a sheriff who ate at the club. she was from new york city and the club was where her dad lived in new jersey. I hope I am recalling it as it was some time ago…..and so many articles since.
        some times when I read articles like that I print but then save to PDF for future reference and save a copy of it on external source.

    • ja

      I’m so sorry for you! My father has this now, was diagnosed a couple months ago.
      He was a big traveller, so who knows where he got it from.
      It is weird that all this is in the Bay Area….makes me want to move.

      • Barbara

        If it is of any comfort, two weeks before my dad died, one of the physicians at CPMC in SF asked him if he had any pain. He was almost unable to speak but the few words he could say were that he didn’t have any pain. For us that was a relief after watching his suffering for 3 months. The doctors told us that the brain wastes away and probably there are no sensations of pain near the end of iife. I pray a cure is found so that no one has to suffer from this disease.

  • D

    geez, a loser in every blog.

  • eaamon

    about 20 years ago a Gilroy man died of this. but they say it is not the same since he only ate deer he shot in Colorado or Wyoming. but where he hunts the deer and cattle eat side by side. the feds gave it another name…to hide it.
    beef safe!?!?
    so what did the government do? well they had all ground beef mixed with thousands of them do dilute the risk of getting the Prions that cause this disease.
    this way everybody is exposed to it in small amounts. it will take years before it gets you. this way they can say it is just dementia setting in or alzheimers you are getting. will you believe it? the feds want you to……..

  • eaamon

    do not eat American beef until it is all tested.
    in Japan all their cattle is tested before it is sold to the public.
    we could do this in the US too!!!
    but big business says it will cut into their profits………..

  • ja

    I’m so sorry for you! My father has this now, was diagnosed a couple months ago.
    He was a big traveller, so who knows where he got it from.

    It is weird that all this is in the Bay Area….makes me want to move.

  • jojo

    The story says in one part quite clearly that the two women died from NON-VARIANT JCD and that “mad cow” is VARIANT JCD. Yet the headline says they died from mad cow and the rest of the story implies it. Please correct the headline!

  • Barbara

    There are four types of CJD:
    * Sporadic (or classical) CJD is the most common form, accounting for around 85 per cent of cases. The cause is unknown. It mainly affects the over 50s and has a sudden onset. The course of the disease is measured in months.

    * Genetic CJD (sometimes known as familial CJD) is an inherited form of the disease, with younger onset and a longer time course than sporadic CJD.

    * Iatrogenic CJD occurs through contamination with infected tissue via medical procedures, for example, the use of human growth hormone, blood transfusion or surgical instruments.

    * Variant CJD is caused by exposure to bovine spongiform encephalopathy, or BSE, and typically affects younger people. It has a relatively longer time course – an average of 14 months from onset of symptoms to death. There is a combination of psychiatric, neurological and physical symptoms.

    Please correct the information. Check the Center for Disease Control website; the CJD support group and search CJD for reports on the Nobel Prize winning Stanley Pruisner at UCSF.

    Tragic disease that is incurable and untreatable. The Department of Agriculture and the FDA should be doing more to monitor the cattle industry but the cattle industry is very STRONG. 200 cases per year of CJD in the US. NOT 3. CBS PLEASE CORRECT THIS ARTICLE.

    • eaamon

      I agree on the number of deaths. but if you recall aids, so many died from it but were listed as natural causes or some other illness.
      and I think your number is one that should be multiplied by ten to get the real number.
      it is one number the cattle industry wants to hide!

    • jmo

      i really like to figure out how they come up with 1 in every million people get cjd my mothers die;n of it and i would like to thank ucsf for being under standing,and honest,i tell you 1 thing theres no way 1in a million because in my moms case they would of never have claimed it cjd they just kept telling her your fine keep taken your b-12 shots even though she;s cry’n out that she;s getting worse and worse -it took me 6month before i new something aint right here and went to her town got a 3rd doctor that was honest and said scjd pos

  • JJseemour

    Does no one proofread these articles before publishing it?

    • eaamon

      the government had them change the story of how she died to make it sound like the food supply is safe…..

  • Ken Azebu

    And there was a recent fatality from CJD in San Jose, a woman who was a teacher at The Harker School.
    Here is an article by Gabe Kirchheimer who details a lot of information about the state of the cattle industry and our food chain:
    If you read the section about downer cows and then recall the PETA video from a few years ago you realize the horror should not have been about animal cruelty, but Mad Cow Disease.
    In addition there were two small studies (less than 50 bodies) where researchers exhumed people who died of Alzheimers and tested for CJD. Both studies found that over 10% of the Alzheimers deaths had CJD.
    My wife died of CJD in 2008. Contact the CJD Foundation for up to date information and statistics.

  • J.Q. Public

    The URL someone sent me and that I am currently using says “mad-cow-disease-kills-san-rafael-woman”. The article headline says “Rare Brain Illness Kills San Rafael Woman”. Was the headline changed? The article talks about mad cow disease a lot, but does eventually mention that mad cow disease is the variant form of CJD, and in a different paragrah that the woman died of non-variant CJD. In other words, NOT MAD COW DISEASE, and the writer knows it. The truth seems to be deliberately obscured, and most readers seem to be missing it.

    Terrible, terrible journalism. CBS should be ashamed.

  • Montana Murdoch

    Forgive my bluntness, but this story is shockingly mis-informed. Particularly shocking because one of the leading researchers of this disease–in the world–Nobel prize winner Dr. Stanley Prusiner–is right here in the Bay Area. Researching this disease couldn’t be easier.

    And I know, because my mother died of non-variant CJD, here in the Bay Area, more than a decade ago.

    Do your homework–if you’re going to bring some attention to this disease, do it with integrity. This story is an embarrassment.

    • eaamon

      thanks for your reply, the government wants people to believe the food supply is safe.

      I have been following CJD since the 1980s and so many stories are covered up.

  • Dr. Blodgett

    Montana, i wish it were only an embarrassment. It almost looks more like dis-information, meant to purposely mislead. I am personally aware of many more than the paltry 3 cases stated in this article. Unfortunately, both the NIH and the CDC have fallen into the habit of occasionally spreading this sort of ‘dis-information’ from time to time, particularly when market values vs. truth conflict.

    The most reliable source I’ve seen for statistics on this disease and it’s variants is:

    There are as many as 300 new cases diagnosed each year in the U.S. alone, and not just one type, but all types are diagnosed. I’ve been in the medical field for over 35 years, and it just about startled me speechless when I read this cbs article. This pile of drivel, whether purposely published as is or not, contradicts itself even by the simplest of logic.

    They must think we’re all total idiots, expecting us to believe what’s been written in this article. The writer, and I use that term very loosely, should be ashamed of herself. Her credibility is now dead, and nothing she ever writes will be believable. The lack of research into the subject matter shows a remarkable level of laziness on her part . We should all be apalled at being treated like fools. And we should all be concerned about this disease.

  • eaamon

    quote:DrBlodgett,”we should all e concerned about this disease.”
    how true! there was a beef processing plant owner in Western Kansas about ten years ago. he wanted to start testing every animal that went through his processing plant. he felt he should do this because he wanted to protect his grandchildren from CJD. the FDA stepped in and denied him the process. they said it was not necessary or required.

    I really think it was the giant plant in Western Nebraska near Ogallala. i believe they processed tens of thousands there daily. since operating this plant now closed i saw workers there using a dozer to lift up 1 1/2 foot thick slabs of cow dung and loading it into dump trucks to remove it from the property.
    since then it has been transformed to a very small operation of about 100 cattle. this is after about a 7 year period.

    and yes!!! we should be appalled at the treatment we get from our company run government. food is tested in Japan. and it is hard to forget what happened in England where the whole beef supply was destroyed it was so rampant…..

    yet we still do not test!?!?
    back in the 1990s there were no variants until the Gilroy man died from it and the venison (deer meat) he was eating. that was the first time the word variant was used.
    I have been watching CJD and the stories since the 1980s…….
    it may not be the writers fault…editor maybe…..

  • Barbara

    When my dad died of CJD 11 years ago, I tried to find out as much as possible about Creutzfeldt-Jakob disease, BSE and other brain-wasting diseases. It was shocking and frightening to learn about the connection to “mad cow disease” in Europe and the lack of required testing here in the US.

    My work colleagues thought it was a big joke when I wouldn’t eat beef or beef products. Everyone thought I had become paranoid and wouldn’t listen to any of the facts about CJD or BSE. They happily munched their burgers and still today laugh about my paranoia.

    Stanley Pruisner’s group at UCSF were able to collect spinal fluid before my dad died. UCSF as well as Case Western received his brain and samples of other organs.

    For those who think this is a “silly” subject, they need to look into the facts about BSE in Europe and other places around the world where BSE and CJD have had deadly consequences. Australia and Japan BSE are very aware of the danger and test accordingly.

    SHAME SHAME SHAME on the US Government Dept of Agriculture and the lobbyists who represent the cattle and feed industry for not requiring testing of ALL cattle. Their lax standards still allow blood meal and blood products to be fed to “veal” calves. Poultry feed contains meat by-products. Some workers feeding dairy cattle either can’t read English labels or won’t know the difference between the feed for pigs, poultry and cattle. Feeding ground up animals resulted in wide spread BSE in Europe but the US government doesn’t care enough to make the changes desperately needed.

    • eaamon

      I did reply to your question above.

  • eaamon

    in the Colorado/Wyoming up to Montana area cattle and deer graze together.
    if they poop onto grass where one another eats, I believe it can be spread.

    just a note in the 1990s the Colorado university in Denver was collecting all the brains from local hunters who shot deer in the Northeastern part of the state for testing for mad cow…note I said deer.

    note we were using ground up animals here in the US for food too.
    I have no complaints from the turkey burgers I eat now.

    I just thought the big cover up was for big med to sell Alzheimer drugs to everyone. there is new ones coming out constantly.

    • eaamon

      is should be are, there are new ones coming out constantly.
      I think one was just released this past week….

  • jay

    this is to agree and can show proof of cover up cjd my mother was being tricked by her doctor and a specialist telling her she was fine just low b12 which all the time having here results of a mri saying cjd they never treat her for never even tell her of it they would of keep it quiet if i didnt take her to a 3rd doctor that said he confirmed cjd like her last mri

  • ja

    Just a repost to my original posting. I am now sitting in hospice with my father who is getting End of Life care and has CJD. Just last week, he was awake and he knew us. He couldn’t do much, but he was alert and able to walk around. Now he’s 100% immobile. he cannot blink his eyes or move his eyeballs. when the nurses change him, he groans, when he breathes, every exhale is a moan. He’s on morphine to ensure no pain is felt, he can no longer swallow so no food or drink. he’s literally wasting away before me. His CJD is sporadic, not genetic or from tainted food. WE NEED A CURE so NO one else in the world has to go through what I’m going through. He was almost normal at Christmas, laughing and joking around, holding my kids and playing. He sang christmas carols. It’s only March 15. Less than three months and the doctor gives him HOURS to live.
    Thank you to the wonderful employers who give us this time off, to spend with him in his last moments. PHarm companies need to stop making drugs that give you diarhea and suicidal thoughts to hide anxiety and CURE CJD!! Do something USEFUL.

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