Hello dear friends and family,
I hope your week is starting out well. Mine is going great! Today I went on a field trip with my kindergartners to the science museum. We had such a blast. I haven't been there in such a long time and the omni theatre is so neat! I like being with my kindergartners, because they help to remind me that it's ok to be a kid sometimes! He he!
Another beautiful day for a run! I definitely will not complain about this weather we've been having. I absolutely love it! Today I had to run 4 miles and for the most part it went ok. My legs did kind of feel heavy today and my hips were a little achey. It will be nice to have these next two days of rest. I have night class tomorrow and Thursday so I won't be laying around the house being a couch potato! Bummers! I actually have two presentations to give on Thursday. Wish me luck! Please keep on checking the blog because I will continue to post insightful information about CJD. I hope you all are finding this blog to be very informative. Please don't stop spreading the news!!!
Much love,
Lacy
Ps. I want to say a big thank you to Lisa Spencer. The note that you sent me was so very uplifting and thoughtful. I even hung it up on my fridge to help motivate me. It just amazes me beyond belief that there is someone special all the way in Texas that is supporting me. Thank you! I'm so sorry about your mother. I know the hole in your heart will never go away, but know that her love for you goes far beyond the grave. I can feel my dad near at such unexpected times, but it always seems to be when I need the comfort of his love the most. We will never be abandoned from their love for us. Remember that! Thank you for your generous donation and your determination to spread the word. You are a blessing!
Tuesday, July 8, 2008
Monday, July 7, 2008
Alzheimers and CJD
Alzheimer's and CJD
Michael Greger
16 June 1996
If indeed a form of Bovine Spongiform Encephalopathy (BSE) exists in the United States, one might expect to see a rise in the number of cases of Creutzfeldt-Jakob disease (CJD). CJD, however, is not a reportable illness in this country (Holman, 1995). Because the Centers for Disease Control (CDC) does not actively monitor the disease (Altman, 1996d) a rise similar to the one in Britain could be missed (Altman, 1996d).
Already, a number of U. S. CJD clusters have been found. In the largest known U. S. outbreak of sporadic cases to date(Flannery, 1996) a five-fold expected rate was found to be associated with cheese consumption in Pennsylvania's Lehigh Valley (Little, 1993) A striking increase in CJD was also reported in Florida (Berger, 1994) and there is an anecdotal report of an cluster in Oregon (Boule, 1996). An analysis of death certificates in a number of states, though, showed an overall stable and typical CJD incidence rate from 1979 to 1993 (World, 1996). To track the disease, the CDC has just initiated a four-state study of death certificates (Altman, 1996a), but since it is considered well known that death-certificate diagnoses are not always accurate (Davanpour, 1993) the survey may not provide an accurate assessment.
The true prevalence of prion diseases in this or any other country remains a mystery (Harrison, 1991). Compounding the uncertainty, autopsies are rarely performed on atypical dementias (Harrison, 1991), because medical professionals fear infection (Altman, 1996a). The officially reported rate in this country is less than 1 case in a million people per year (World, 1996). An informal survey of neuropathologists, however, registered a theoretical range of 2-12% of all dementias as actually CJD (Harrison, 1991). And hundreds of thousands of Americans suffer from severe dementias every year (Brayne, 1994; United, 1995). Two other studies average about a 3% CJD rate among dementia patients (Mahendra, 1987; Wade, 1987). A preliminary 1989 University of Pennsylvania study showed that 5% of patients diagnosed with dementia were actually dying from Creutzfeldt-Jakob disease (Boller, 1989). It would seem CJD is seriously underdiagnosed at present (Harrison, 1991).
The most common misdiagnosis of CJD is Alzheimer's disease (Harrison, 1991). CJD was even described by our government's top CJD researcher (Wlazelek, 1990a) as "Alzheimer's in fast forward (Wlazelek, 1990b)." The symptoms and pathology of both diseases overlap (Brown, 1989). There can be spongy changes in Alzheimer's, for example, and senile plaques in CJD (Brown, 1989). The causes may overlap as well; epidemiological evidence suggests that people eating meat more than four times a week for a prolonged period have a three times higher chance of suffering a dementia than long-time vegetarians (Giem, 1993), although this result may be confounded by vascular factors (Van Duijn, 1996).
Paul Brown, medical director for the U.S. Public Health Service (Gruzen, 1996), said that the brains of the young people who died from the new CJD variant in Britain even look like Alzheimer's brains (Hager, 1996). Stanley Prusinger, the scientist who coined the term prion, speculates Alzheimer's may in fact turn out to be a prion disease (Prusiner, 1984). In younger victims the disease could look like multiple sclerosis or a severe viral infection, according to Alzheimer's expert Gareth Roberts (Brain, 1996).
An estimated two to three million Americans are afflicted by Alzheimer's (Scully, 1993); it is the fourth leading cause of death among the elderly in the U.S (Perry, 1995). Twenty percent or more of people clinically diagnosed with Alzheimer's disease are found at autopsy to not have had Alzheimer's at all (McKhann, 1984). At Yale, out of 46 patients clinically diagnosed with Alzheimer's, 6 were proven to be CJD at autopsy (Manuelidis, 1989). In another post-mortem study 3 out of 12 "Alzheimer" patients actually died from a spongiform encephalopathy (Teixeira, 1995).
Carleton Gajdusek, who was awarded a Nobel Prize for his work with prion diseases (Manuelidis, 1985), estimates that 1% of people showing up in Alzheimer clinics actually have CJD (Folstein, 1983). That means that hundreds of people (Hoyert, 1996; United, 1995) may already be dying from mad cow disease each year in the United States.
Source: http://www.mad-cow.org/Alzheimer_cjd.html
Michael Greger
16 June 1996
If indeed a form of Bovine Spongiform Encephalopathy (BSE) exists in the United States, one might expect to see a rise in the number of cases of Creutzfeldt-Jakob disease (CJD). CJD, however, is not a reportable illness in this country (Holman, 1995). Because the Centers for Disease Control (CDC) does not actively monitor the disease (Altman, 1996d) a rise similar to the one in Britain could be missed (Altman, 1996d).
Already, a number of U. S. CJD clusters have been found. In the largest known U. S. outbreak of sporadic cases to date(Flannery, 1996) a five-fold expected rate was found to be associated with cheese consumption in Pennsylvania's Lehigh Valley (Little, 1993) A striking increase in CJD was also reported in Florida (Berger, 1994) and there is an anecdotal report of an cluster in Oregon (Boule, 1996). An analysis of death certificates in a number of states, though, showed an overall stable and typical CJD incidence rate from 1979 to 1993 (World, 1996). To track the disease, the CDC has just initiated a four-state study of death certificates (Altman, 1996a), but since it is considered well known that death-certificate diagnoses are not always accurate (Davanpour, 1993) the survey may not provide an accurate assessment.
The true prevalence of prion diseases in this or any other country remains a mystery (Harrison, 1991). Compounding the uncertainty, autopsies are rarely performed on atypical dementias (Harrison, 1991), because medical professionals fear infection (Altman, 1996a). The officially reported rate in this country is less than 1 case in a million people per year (World, 1996). An informal survey of neuropathologists, however, registered a theoretical range of 2-12% of all dementias as actually CJD (Harrison, 1991). And hundreds of thousands of Americans suffer from severe dementias every year (Brayne, 1994; United, 1995). Two other studies average about a 3% CJD rate among dementia patients (Mahendra, 1987; Wade, 1987). A preliminary 1989 University of Pennsylvania study showed that 5% of patients diagnosed with dementia were actually dying from Creutzfeldt-Jakob disease (Boller, 1989). It would seem CJD is seriously underdiagnosed at present (Harrison, 1991).
The most common misdiagnosis of CJD is Alzheimer's disease (Harrison, 1991). CJD was even described by our government's top CJD researcher (Wlazelek, 1990a) as "Alzheimer's in fast forward (Wlazelek, 1990b)." The symptoms and pathology of both diseases overlap (Brown, 1989). There can be spongy changes in Alzheimer's, for example, and senile plaques in CJD (Brown, 1989). The causes may overlap as well; epidemiological evidence suggests that people eating meat more than four times a week for a prolonged period have a three times higher chance of suffering a dementia than long-time vegetarians (Giem, 1993), although this result may be confounded by vascular factors (Van Duijn, 1996).
Paul Brown, medical director for the U.S. Public Health Service (Gruzen, 1996), said that the brains of the young people who died from the new CJD variant in Britain even look like Alzheimer's brains (Hager, 1996). Stanley Prusinger, the scientist who coined the term prion, speculates Alzheimer's may in fact turn out to be a prion disease (Prusiner, 1984). In younger victims the disease could look like multiple sclerosis or a severe viral infection, according to Alzheimer's expert Gareth Roberts (Brain, 1996).
An estimated two to three million Americans are afflicted by Alzheimer's (Scully, 1993); it is the fourth leading cause of death among the elderly in the U.S (Perry, 1995). Twenty percent or more of people clinically diagnosed with Alzheimer's disease are found at autopsy to not have had Alzheimer's at all (McKhann, 1984). At Yale, out of 46 patients clinically diagnosed with Alzheimer's, 6 were proven to be CJD at autopsy (Manuelidis, 1989). In another post-mortem study 3 out of 12 "Alzheimer" patients actually died from a spongiform encephalopathy (Teixeira, 1995).
Carleton Gajdusek, who was awarded a Nobel Prize for his work with prion diseases (Manuelidis, 1985), estimates that 1% of people showing up in Alzheimer clinics actually have CJD (Folstein, 1983). That means that hundreds of people (Hoyert, 1996; United, 1995) may already be dying from mad cow disease each year in the United States.
Source: http://www.mad-cow.org/Alzheimer_cjd.html
Sunday, July 6, 2008
Third week of training...DONE!! Lots more to go!
I had a very lazy Sunday today. I hope all of you did as well. It's going to be hard to go back to work this week. Anyway, it was so hot and humid today that I decided to run later in the evening. I never would of made it if I ran in the dead heat of the afternoon. It was like a sauna outside. When I got home from a great weekend with my family, I unpacked my stuff and headed out for a run. It was still quite steamy at 7pm, but there was a tiny breeze that kept me going. I had a lot on my mind during my run, so it actually went by pretty fast. I usually don't stop for water, but I had to this time because of the heat. I was parched! The best part of my run was at the end when I decided to take a dip in Lake Harriet. I took off my shoes and socks, but went in with all of my running gear. I got some strange looks, especially when I walked home all soaking wet but I didn't care because that felt darn good! I wasn't really feeling like running today and I was even thinking about going tomorrow instead, but when I do something for my dad I get a whole new motivation. I don't want to let him down. So I'm happy to say now that I did it and I definitely could feel him at my side pushing me along.
I want to say thank you to John Wilson who left a comment in my last post. Your message was so very sweet. It warms my heart that somebody I've never met is moved by my story and wants to donate to the cause. Your support is so deeply appreciated. Thanks again to both you and your daughter and I'm so sorry for your loss. Your wife is definitely shining through your hearts.
Much love,
Lacy
I want to say thank you to John Wilson who left a comment in my last post. Your message was so very sweet. It warms my heart that somebody I've never met is moved by my story and wants to donate to the cause. Your support is so deeply appreciated. Thanks again to both you and your daughter and I'm so sorry for your loss. Your wife is definitely shining through your hearts.
Much love,
Lacy
Saturday, July 5, 2008
This is a long reading, but please check it out!!
Hey all-
I'm still resting and enjoying some time home with my family. I hope you all are enjoying your 4th of July weekend! Tomorrow will be my long run of 8 miles so I will do some yoga today to get my muscles flexible and ready to go. Wish me luck!
I've been doing more research on CJD and I would like you to read this article about the link between CJD & BSE (Mad Cow's Disease)and also check out the links below to see what the government is doing to monitor the issue. I was shocked to read the findings. I don't want all of you to think that I'm totally believing that my dad's CJD diagnosis is a direct result of eating contaminated meat. I know that the autopsy results say his form was "sporadic", but that really doesn't help at all. I know that there isn't always an explanation for the turns that life takes, but there is information out there that may help me find out more. Before I was just in a daze that my dad truly passed away and now I'm taking the initiative to understand what is being found or said about CJD. This is such a scary disease and I don't want anybody to have to go through what my dad endured. If we are all at risk and there are measures we can take lower that, then we need to know! Please read the information below.
Sincerely,
Lacy
MAD COW DISEASE Written by, Suzanne Sutton in 1997
Mad Cow Disease, known scientifically as bovine spongiform encephalopathy (BSE) in cattle, is an incurable spongy degenaration of the brain and central nervous system. A similar complex disease in humans, known as Creutzfeldt-Jakob Disease (CJD), acts like an accelerated form of Alzheimer's, and is characterized by an irreversible degeneration of brain tissue-holes formed in the brain, disabling and finally killing the victim. The fear of this unusually cruel and fatal neurological disease has sent shock waves throughout Europe and around the globe, creating one of the biggest consumer panics ever experienced in the industrialized world.
Q. How do cattle and humans contract this disease?
A. For decades British and North American farmers have been feeding their beef and dairy cattle, which are of course herbivores, cheap protein supplements made from things which include sheep brains, spinal cords, and other animal parts. Sheep, as any farmer will testify, have for centuries carried scrapie--a fatal, degenerative brain disease, which is remarkably similar to Mad Cow Disease and CJD. It is feared that this disease can be transmitted to humans who eat meat from infected cattle.
Since 1989 Britain has banned sheep offal (the ground remains of the dead animal) from cattle feed. Indeed, all mammal tissue has been banned from all agricultural feed in that country, and, furthermore, the World Health Organization is now endorsing a ban for all countries. However, in the United States this practice continues up to the present time as a routine process, designed to boost milk and meat production. Indeed, offal from sheep, cattle and other animals, as well as animal feces, is routinely fed to American food animals (cattle, pigs, poultry and fish) in the form of rendered pellets, powder or meal. In addition, massive quantities of blood meal, bone meal and other animal byproducts find their way into food animal's feed. It is grossly unnatural and dangerous to feed blood and other animal parts to cattle, which are natural vegetarians. Animal diseases may very well be passed on in the process.
Various diseases may also be transmitted to human beings who eat infected animals. Indeed, from feed, to cow, to the human brain, appears to be the progression of Mad Cow Disease, which has leaped across the species barrier to become a varian of CJD.
Cattle with the disease show symptoms of staggering, drooling, aggression, and confused behavior, appearing to have gone "mad." Afflicted humans show symptoms similar to Alzheimer's disease--dementia, confusion, convulsions, loss of speech, sight, and hearing, and ending with a coma and death. This disease, one of the most mysterious known to human beings, is always fatal and there is no treatment for it. The incubation period seems to be four to thrty years.
The causative agent appears to be a deformed molecule called a "prion," (pronounced PREE-on), a mysterious and abnormal infectious protein. This strange-acting, never-before-seen infectious agent, which is neither a bacteria nor a virus, is distinct from anything encountered before--an infectious agent that defies the accepted rules of nature. Smaller than the tiniest virus, they do not contain nucleic acid which makes up the RNA and DNA that carry the genetic codes of normal viruses, bacteria, plants, cows, humans and virtually all other living things. Yet they are able to replicate and spread, but do not activate an immune response. Unfortunately, they are highly resistant to heat, UV light, radiation and most common chemical disinfectants.
Q. Surely the proper authorities in this country are taking actions to prevent this disease from gaining a foothold here. Is not this the case?
A. Tragically, this is not so. The very practice that apparently caused and fostered Mad Cow Disease in England--feeding cattle processed remains of other animals--is commonplace in America. The image of contented cows grazing on sweet grass and hay should forever be dispelled. No "Green Acres" here! Ground up carcasses of sheep, cows and other animals, including their tonsils, intestines, spinal cords, brains, spleens, and so on, are a regular part of the daily bill of fare of food animals, which are mass produced by intensive, risky, pro-duction-driven, farming methods. The poor aimals are crowded and confined by the thousands on factory-style farms. These, and other horrors of modern animal food production, give rise to various chronic, insidious, and complex groups of diseases.
Recently, the United States Department of Agriculture (USDA) considered a ban on feeding cows to cows. However, according to an internal USDA document, the agency dismissed the ban because "the cost to the livestock and tendering industries would be substantial."[1] Clearly, this governmental agency has placed the financial interests of the influential, multibillion-dollar livestock industry ahead of public health.
However, a ban on this procedure may not be the answer to the problem. Even in Great Britain, where a mandatory ban has been in effect since 1989, some farmers have illicitly been feeding their cattle rendered animal parts. This disturbing reality has always been the case. Any ban is totally dependent on individual and industrial compliance.
More information from this article can be found at this link: http://www.shepherds-rod-message.org/health/mad.html
These links below contain lots of information of the government's role in monitoring this disease.
http://www.gao.gov/new.items/d06157r.pdf
http://www.gao.gov/new.items/d05101.pdf
http://www.gao.gov/new.items/d02183.pdf
http://www.ratical.org/co-globalize/MaeWanHo/bse.txt
I'm still resting and enjoying some time home with my family. I hope you all are enjoying your 4th of July weekend! Tomorrow will be my long run of 8 miles so I will do some yoga today to get my muscles flexible and ready to go. Wish me luck!
I've been doing more research on CJD and I would like you to read this article about the link between CJD & BSE (Mad Cow's Disease)and also check out the links below to see what the government is doing to monitor the issue. I was shocked to read the findings. I don't want all of you to think that I'm totally believing that my dad's CJD diagnosis is a direct result of eating contaminated meat. I know that the autopsy results say his form was "sporadic", but that really doesn't help at all. I know that there isn't always an explanation for the turns that life takes, but there is information out there that may help me find out more. Before I was just in a daze that my dad truly passed away and now I'm taking the initiative to understand what is being found or said about CJD. This is such a scary disease and I don't want anybody to have to go through what my dad endured. If we are all at risk and there are measures we can take lower that, then we need to know! Please read the information below.
Sincerely,
Lacy
MAD COW DISEASE Written by, Suzanne Sutton in 1997
Mad Cow Disease, known scientifically as bovine spongiform encephalopathy (BSE) in cattle, is an incurable spongy degenaration of the brain and central nervous system. A similar complex disease in humans, known as Creutzfeldt-Jakob Disease (CJD), acts like an accelerated form of Alzheimer's, and is characterized by an irreversible degeneration of brain tissue-holes formed in the brain, disabling and finally killing the victim. The fear of this unusually cruel and fatal neurological disease has sent shock waves throughout Europe and around the globe, creating one of the biggest consumer panics ever experienced in the industrialized world.
Q. How do cattle and humans contract this disease?
A. For decades British and North American farmers have been feeding their beef and dairy cattle, which are of course herbivores, cheap protein supplements made from things which include sheep brains, spinal cords, and other animal parts. Sheep, as any farmer will testify, have for centuries carried scrapie--a fatal, degenerative brain disease, which is remarkably similar to Mad Cow Disease and CJD. It is feared that this disease can be transmitted to humans who eat meat from infected cattle.
Since 1989 Britain has banned sheep offal (the ground remains of the dead animal) from cattle feed. Indeed, all mammal tissue has been banned from all agricultural feed in that country, and, furthermore, the World Health Organization is now endorsing a ban for all countries. However, in the United States this practice continues up to the present time as a routine process, designed to boost milk and meat production. Indeed, offal from sheep, cattle and other animals, as well as animal feces, is routinely fed to American food animals (cattle, pigs, poultry and fish) in the form of rendered pellets, powder or meal. In addition, massive quantities of blood meal, bone meal and other animal byproducts find their way into food animal's feed. It is grossly unnatural and dangerous to feed blood and other animal parts to cattle, which are natural vegetarians. Animal diseases may very well be passed on in the process.
Various diseases may also be transmitted to human beings who eat infected animals. Indeed, from feed, to cow, to the human brain, appears to be the progression of Mad Cow Disease, which has leaped across the species barrier to become a varian of CJD.
Cattle with the disease show symptoms of staggering, drooling, aggression, and confused behavior, appearing to have gone "mad." Afflicted humans show symptoms similar to Alzheimer's disease--dementia, confusion, convulsions, loss of speech, sight, and hearing, and ending with a coma and death. This disease, one of the most mysterious known to human beings, is always fatal and there is no treatment for it. The incubation period seems to be four to thrty years.
The causative agent appears to be a deformed molecule called a "prion," (pronounced PREE-on), a mysterious and abnormal infectious protein. This strange-acting, never-before-seen infectious agent, which is neither a bacteria nor a virus, is distinct from anything encountered before--an infectious agent that defies the accepted rules of nature. Smaller than the tiniest virus, they do not contain nucleic acid which makes up the RNA and DNA that carry the genetic codes of normal viruses, bacteria, plants, cows, humans and virtually all other living things. Yet they are able to replicate and spread, but do not activate an immune response. Unfortunately, they are highly resistant to heat, UV light, radiation and most common chemical disinfectants.
Q. Surely the proper authorities in this country are taking actions to prevent this disease from gaining a foothold here. Is not this the case?
A. Tragically, this is not so. The very practice that apparently caused and fostered Mad Cow Disease in England--feeding cattle processed remains of other animals--is commonplace in America. The image of contented cows grazing on sweet grass and hay should forever be dispelled. No "Green Acres" here! Ground up carcasses of sheep, cows and other animals, including their tonsils, intestines, spinal cords, brains, spleens, and so on, are a regular part of the daily bill of fare of food animals, which are mass produced by intensive, risky, pro-duction-driven, farming methods. The poor aimals are crowded and confined by the thousands on factory-style farms. These, and other horrors of modern animal food production, give rise to various chronic, insidious, and complex groups of diseases.
Recently, the United States Department of Agriculture (USDA) considered a ban on feeding cows to cows. However, according to an internal USDA document, the agency dismissed the ban because "the cost to the livestock and tendering industries would be substantial."[1] Clearly, this governmental agency has placed the financial interests of the influential, multibillion-dollar livestock industry ahead of public health.
However, a ban on this procedure may not be the answer to the problem. Even in Great Britain, where a mandatory ban has been in effect since 1989, some farmers have illicitly been feeding their cattle rendered animal parts. This disturbing reality has always been the case. Any ban is totally dependent on individual and industrial compliance.
More information from this article can be found at this link: http://www.shepherds-rod-message.org/health/mad.html
These links below contain lots of information of the government's role in monitoring this disease.
http://www.gao.gov/new.items/d06157r.pdf
http://www.gao.gov/new.items/d05101.pdf
http://www.gao.gov/new.items/d02183.pdf
http://www.ratical.org/co-globalize/MaeWanHo/bse.txt
Thursday, July 3, 2008
Yeah...I didn't fall!!
I want to start of by saying a personal thank you to the person who made a generous donation. It was such a pleasant surprise when I checked the account balance. That definitely brought a smile to my face. Thank you!
Today I did some cross-training and instead of running 6 miles, I rollerbladed about 12. My roommates and I were all home at the same time which never really happens. So we decided to go to rollerblade together. Well...they were going more leisurely and I wanted to go faster to put in a good work out. They ended up just going around Lake Harriet and taking a dip in the water. I proceeded to go to Lake Calhoun and I ended up going around it 2 times. I was on a roll. I felt like I was flying! However, I was a little in pain towards the end because I could feel a blister coming on strong. It is just a little bitty one so I think I'll be ok. I'm most thankful that I didn't fall. I'm not very good at using my brakes and it freaks me out when I reach the bumps at the end of a sidewalk. I always feel like I'm going to fall flat on my face and make a fool of myself in front of traffic. How embarrassing. I was lucky this time and made it home in one piece. Now I have two days of rest and I sunday I will run 8 miles. I hope it is a nice day. Wish me luck. Please keep on checking the blog because I will still be posting information to keep you all up to date. Thank you for your support. I hope you have a great 4th of July weekend!
Much love,
Lacy
Today I did some cross-training and instead of running 6 miles, I rollerbladed about 12. My roommates and I were all home at the same time which never really happens. So we decided to go to rollerblade together. Well...they were going more leisurely and I wanted to go faster to put in a good work out. They ended up just going around Lake Harriet and taking a dip in the water. I proceeded to go to Lake Calhoun and I ended up going around it 2 times. I was on a roll. I felt like I was flying! However, I was a little in pain towards the end because I could feel a blister coming on strong. It is just a little bitty one so I think I'll be ok. I'm most thankful that I didn't fall. I'm not very good at using my brakes and it freaks me out when I reach the bumps at the end of a sidewalk. I always feel like I'm going to fall flat on my face and make a fool of myself in front of traffic. How embarrassing. I was lucky this time and made it home in one piece. Now I have two days of rest and I sunday I will run 8 miles. I hope it is a nice day. Wish me luck. Please keep on checking the blog because I will still be posting information to keep you all up to date. Thank you for your support. I hope you have a great 4th of July weekend!
Much love,
Lacy
Wednesday, July 2, 2008
This is very informative!
GRAND ROUNDS PRESENTATION
Pathology's Dr. Nitya Ghatak Presents
Prion Diseases
Nitya Ghatak, MD, Professor of Neuropathology at Virginia Commonwealth University presented Prion Diseases: From Scrapie to Mad Cow, February 20 at the Pathology Grand Rounds.
"Without question," Dr. Ghatak began, "prion theory is one of the most revolutionary ideas in the study of infectious diseases. It captures the imagination. How can a protein, a substance that has no RNA nor DNA, infect individuals between and across the species barrier by itself?" Some scientists, he added, don't believe it can. They are interested in prions as markers of an infectious agent, possibly a slow virus, that has yet to be identified.
"I'd like to walk you through the history behind prion disease theory," he said. Then I'll bring you up-to-date, sharing the current thinking about prions today."
The major characters in this history, he explained, are goats, sheep, mankind and cows. The goats, the sheep and mankind all got sick. In 1959 it was discovered their stories overlapped when a research veterinarian, Dr. William Hadlow, made the observation that their brain changes were similar.
The goats and sheep had scrapie, a well known neurological disease described by Europeans in the 1750s. The sick animals showed behavioral changes, then progressively worsening ataxia (tremors), general neurological degeneration and death.
The people had two rare diseases with similar symptoms. One described in the 1920s--by German physicians Hans Gerhard Creutzfeldt and Alfons Jakob--was aptly named Creutzfeldt-Jakob disease (CJD). The second, described in the 1950s, was called "Kuru," meaning "to tremble."
Creutzfeldt-Jakob had a wide range of manifestations. Dr. Ghatak pointed out, "The same disease was called by different names, depending on the form that was present." CJD was rare. Chances of getting it were described as one in a million. It was sporadic and uniformly fatal, usually within weeks or months. Most of its victims were older than fifty years of age. No one had any idea of its cause.
CJD victims showed behavioral changes, rapidly developing dementia, ataxia, blindness, coma, then death. During autopsy neurologists found major changes in the victim's brains. They were spongiform. "Literally, full of little holes," said Dr. Ghatak. There was a loss of neurons and reactive astrocytes were present. But, no inflammation, as one might expect from an infectious agent. Varying neurologic signs and symptoms were present depending on which part of the brain was involved.
Then they found Kuru, he continued, an exotic disease of only one people in the world--the Fore people of Papua, New Guinea. Its victims were the women and children who ritualistically consumed human brain in a funereal practice honoring the dead. Its symptoms were behavioral changes, ataxia, progressive neurological degeneration and death within a year.
Dr. Vincent Zigas a medical officer in New Guinea and Dr. D. Carleton Gajdusek a pediatrician and virologist who later won the Nobel Prize for his work, were the first to study this disease called "the laughing death" after one of its symptoms. In 1959, Dr. Igor Klatzo of the National Institutes of Health explained the neuropathology of Kuru and added an observation not yet documented, the presence of amyloid in the form of congophilic plaques in the brains of Kuru victims.
The collective of these different diseases gave rise to the concept of transmissible spongiform encephalitis or TSE. Interestingly, in the 1960s, since the subject of transmissibility was very popular among researchers and clinicians at the time, Dr. Ghatak and his colleagues at the Albert Einstein College of Medicine, Bronx, NY, sent tissue from two CJD cases they had to Dr. Gajdusek. They never heard back, Dr. Ghatak said.
It was in 1982 that Dr. Stanley Prusiner, Professor of Neurology, Virology, and Biochemistry at the University of California San Francisco developed Prion Theory for which he later won the Nobel Prize. He isolated what he believed to be the transmissible agent from hamster brains infected with scrapie--a misfolded protein particle. He named it PRION for proteinaceous infectious particles.
According to the theory, the prion protein, PRNP, found normally in animals in the form of PrPc, becomes conformationally modified into its isoform PrPsc, considered "infectious." The conformational changes make it resistant to enzyme digestion. Its "infectious" character arises when one prion acts as a template for the creation of more prions presumably with the help of a pathological chaperone. These prions aggregate and form amyloid giving rise to the typical symptoms.
Finally, as the history goes--the cows enter the picture. They became major players when another form of TSE, bovine spongiform encephalitis (BSE), commonly called "mad cow disease," showed up in the United Kingdom in 1985. The cows had been given high-protein feed made from the ground-up carcasses of sheep. Like other TSEs, BSE caused a change in temperament, abnormal posture, lack of coordination, difficulty in rising and finally death for the cows.
The tragedy is that BSE crossed the species barrier into humans causing, "A peculiar kind of CJD named variant CJD (vCJD)," said Dr. Ghatak. Its victims were atypically young. In fact, the connection was first made in a 19 year old man diagnosed with it. It sparked a wave of fear that led to the slaughter of 4.5m cattle in the UK and across Europe. A total of 153 people had died by 2003. All but 10 of the victims were from the UK.
The symptoms of vCJD are familiar part of the story by now. It first presents as a psychiatric problem, explained Dr. Ghatak, with dementia, anxiety, and depression. Then symptoms of progressive neurodegeneration appear including ataxia, coma and death within one year of onset. The incubation period can be long--as long as 20 to 30years. Pathologically, PrPsc accumulation is found in lymphoid tissue including the tonsils. The brain is spongiform and shows neuronal loss and amyloid plagues.
Phenotypes of CJD are modified by 2 factors, the genotype of PRNP polymorphic codon 129, and the type of PrPsc, either Type 1 and Type 2. So far, all tested cases of vCJD have been homozygous for methionine.
Today, CJDs are generally classified as familial, sporadic, iatrogenic, or variant. Familial, or inherited, forms include fatal familial insomnia (FFI) and Gertsman-Straussler-Scheinker (GSS) Syndrome. At least twenty different mutations are known. Sporadic CJD (sCJD) accounts for 90% of the cases. In the recent past, iatrogenic CJD arose in patients who had received tissue grafts, particularly cornea and dura, and human growth hormone.
The mystery remains, said Dr. Ghatak, but evidence is building that TSE's are not caused by prions acting alone. "There's something," he said, "about the conversion process from PrPc to PrPsc at the cell membrane...
For more information about his work, you may contact Dr. Ghatak at nrghatak@vcu.edu.
Source: http://www.pathology.vcu.edu/news/grand51.html
Pathology's Dr. Nitya Ghatak Presents
Prion Diseases
Nitya Ghatak, MD, Professor of Neuropathology at Virginia Commonwealth University presented Prion Diseases: From Scrapie to Mad Cow, February 20 at the Pathology Grand Rounds.
"Without question," Dr. Ghatak began, "prion theory is one of the most revolutionary ideas in the study of infectious diseases. It captures the imagination. How can a protein, a substance that has no RNA nor DNA, infect individuals between and across the species barrier by itself?" Some scientists, he added, don't believe it can. They are interested in prions as markers of an infectious agent, possibly a slow virus, that has yet to be identified.
"I'd like to walk you through the history behind prion disease theory," he said. Then I'll bring you up-to-date, sharing the current thinking about prions today."
The major characters in this history, he explained, are goats, sheep, mankind and cows. The goats, the sheep and mankind all got sick. In 1959 it was discovered their stories overlapped when a research veterinarian, Dr. William Hadlow, made the observation that their brain changes were similar.
The goats and sheep had scrapie, a well known neurological disease described by Europeans in the 1750s. The sick animals showed behavioral changes, then progressively worsening ataxia (tremors), general neurological degeneration and death.
The people had two rare diseases with similar symptoms. One described in the 1920s--by German physicians Hans Gerhard Creutzfeldt and Alfons Jakob--was aptly named Creutzfeldt-Jakob disease (CJD). The second, described in the 1950s, was called "Kuru," meaning "to tremble."
Creutzfeldt-Jakob had a wide range of manifestations. Dr. Ghatak pointed out, "The same disease was called by different names, depending on the form that was present." CJD was rare. Chances of getting it were described as one in a million. It was sporadic and uniformly fatal, usually within weeks or months. Most of its victims were older than fifty years of age. No one had any idea of its cause.
CJD victims showed behavioral changes, rapidly developing dementia, ataxia, blindness, coma, then death. During autopsy neurologists found major changes in the victim's brains. They were spongiform. "Literally, full of little holes," said Dr. Ghatak. There was a loss of neurons and reactive astrocytes were present. But, no inflammation, as one might expect from an infectious agent. Varying neurologic signs and symptoms were present depending on which part of the brain was involved.
Then they found Kuru, he continued, an exotic disease of only one people in the world--the Fore people of Papua, New Guinea. Its victims were the women and children who ritualistically consumed human brain in a funereal practice honoring the dead. Its symptoms were behavioral changes, ataxia, progressive neurological degeneration and death within a year.
Dr. Vincent Zigas a medical officer in New Guinea and Dr. D. Carleton Gajdusek a pediatrician and virologist who later won the Nobel Prize for his work, were the first to study this disease called "the laughing death" after one of its symptoms. In 1959, Dr. Igor Klatzo of the National Institutes of Health explained the neuropathology of Kuru and added an observation not yet documented, the presence of amyloid in the form of congophilic plaques in the brains of Kuru victims.
The collective of these different diseases gave rise to the concept of transmissible spongiform encephalitis or TSE. Interestingly, in the 1960s, since the subject of transmissibility was very popular among researchers and clinicians at the time, Dr. Ghatak and his colleagues at the Albert Einstein College of Medicine, Bronx, NY, sent tissue from two CJD cases they had to Dr. Gajdusek. They never heard back, Dr. Ghatak said.
It was in 1982 that Dr. Stanley Prusiner, Professor of Neurology, Virology, and Biochemistry at the University of California San Francisco developed Prion Theory for which he later won the Nobel Prize. He isolated what he believed to be the transmissible agent from hamster brains infected with scrapie--a misfolded protein particle. He named it PRION for proteinaceous infectious particles.
According to the theory, the prion protein, PRNP, found normally in animals in the form of PrPc, becomes conformationally modified into its isoform PrPsc, considered "infectious." The conformational changes make it resistant to enzyme digestion. Its "infectious" character arises when one prion acts as a template for the creation of more prions presumably with the help of a pathological chaperone. These prions aggregate and form amyloid giving rise to the typical symptoms.
Finally, as the history goes--the cows enter the picture. They became major players when another form of TSE, bovine spongiform encephalitis (BSE), commonly called "mad cow disease," showed up in the United Kingdom in 1985. The cows had been given high-protein feed made from the ground-up carcasses of sheep. Like other TSEs, BSE caused a change in temperament, abnormal posture, lack of coordination, difficulty in rising and finally death for the cows.
The tragedy is that BSE crossed the species barrier into humans causing, "A peculiar kind of CJD named variant CJD (vCJD)," said Dr. Ghatak. Its victims were atypically young. In fact, the connection was first made in a 19 year old man diagnosed with it. It sparked a wave of fear that led to the slaughter of 4.5m cattle in the UK and across Europe. A total of 153 people had died by 2003. All but 10 of the victims were from the UK.
The symptoms of vCJD are familiar part of the story by now. It first presents as a psychiatric problem, explained Dr. Ghatak, with dementia, anxiety, and depression. Then symptoms of progressive neurodegeneration appear including ataxia, coma and death within one year of onset. The incubation period can be long--as long as 20 to 30years. Pathologically, PrPsc accumulation is found in lymphoid tissue including the tonsils. The brain is spongiform and shows neuronal loss and amyloid plagues.
Phenotypes of CJD are modified by 2 factors, the genotype of PRNP polymorphic codon 129, and the type of PrPsc, either Type 1 and Type 2. So far, all tested cases of vCJD have been homozygous for methionine.
Today, CJDs are generally classified as familial, sporadic, iatrogenic, or variant. Familial, or inherited, forms include fatal familial insomnia (FFI) and Gertsman-Straussler-Scheinker (GSS) Syndrome. At least twenty different mutations are known. Sporadic CJD (sCJD) accounts for 90% of the cases. In the recent past, iatrogenic CJD arose in patients who had received tissue grafts, particularly cornea and dura, and human growth hormone.
The mystery remains, said Dr. Ghatak, but evidence is building that TSE's are not caused by prions acting alone. "There's something," he said, "about the conversion process from PrPc to PrPsc at the cell membrane...
For more information about his work, you may contact Dr. Ghatak at nrghatak@vcu.edu.
Source: http://www.pathology.vcu.edu/news/grand51.html
Tuesday, July 1, 2008
Lost Between the Cracks....
It was a long day and a long evening at the library, but here I am eager to write to all of you about something that has been on my mind.
Last week I was feeling very sad about my dad's passing. This journey of grief is definitely a rollercoaster. There are days that I feel so good and there are days that I feel so low. I think about my dad almost everyday, but doing this blog has given me some discontent about the situation. Even though it feels like just yesterday, my dad passed away 8 months ago now. I will never completely jump over this hurdle of missing my dad, but I'm slowly coming out of the stage of unconsciousness. Before it was all just a bad dream and now, the dark clouds are starting to clear and give way to light.
So the more I sit down to investigate about CJD with this blog the more I feel like my dad was lost between the cracks. I do believe that this was God's plan for my dad, but I also believe that He gave us free will. This free will can lead us to make decisions that don't come from God and therefore, perpetuate death and destruction in the world. This is clearly represented with war, pollution, murder and among many others, disease. This is my own personal belief.
With this in mind, I've been feeling trapped behind all the unknown and conflicting information about CJD. This especially hit close to home, when I got to thinking about how we still haven't received my dad's autopsy results. My step-mom is the power of attorney so really the whole thing is out of my hands. However, I went ahead and did some investigation on my own. Several sources that I found stated that autopsy results and testing for CJD should take no more than 10 weeks. It has obviously been way longer than that and that makes me feel uneasy and confused.
I called my step-mom to discuss with her about my feelings and she filled me in on the situation. It turns out that the CJD survelliance center and my dad's doctor have been sending her through hoops. When she contacted the surveillance center they said that the autopsy results can only be given to the patient's doctor. From there, my step-mom understood that the results would be faxed to my dad's doctor. She went several weeks and never heard a word from Dr. Hill. She called him and he stated that he never received a fax. She then called the surveillance center and they supposedly said that they don't fax the results; they only send them by mail. Can you believe this run around? This was my dad's life; it should've been handled with the utmost care!
My step-mom realized after talking to us that this all didn't add up and it wasn't fair. She pressed the issue more and finally, recieved some confirmed information this Monday. He stated that my dad's autopsy and test results were finished back in March, but the fax that the surveillance center supposedly sent never made its way to his office. Can you believe this?? Well, he confirmed that my dad was diagnosed with Sporadic CJD. This calmed my worries and at the same time, made me feel very suspicious. I'm beyond thankful that it is not the familial variant, but now I'm full of many questions.
Why did this whole autopsy take so long? This is a rare disease and this was a person's life, you think they would have been more careful. This just frustrates me. Being that he passed away from the Sporadic variant of CJD means that there is basically no hard core fact as to why it happened (Just like some people suddenly acquire cancer or have a heart attack for no apparent reason). However, all of this talk about Mad Cow's disease and how it has been found in animals here, but there are no confirmed cases of this variant in humans definitely fills me with doubt. It just doesn't make sense. My dad was an avid hunter and ate all sorts of meat, including deer, elk, buffalo, etc. You name it, he ate it.
Are they hiding something from us? (They being the USDA or the government) Are they just saying that it is sporadic so that we don't question about Mad Cow's disease?
We need to know more!! If there is more than just what we see on the surface, than we all are at risk for CJD.
Please continue to spread the word!
-Lacy
Ps. I had the radio on as I was writing this and one of my dad's songs was played "Listen to the music". I totally felt him here with me at that moment. Is he telling me that I'm heading in the right direction with all of this?
Last week I was feeling very sad about my dad's passing. This journey of grief is definitely a rollercoaster. There are days that I feel so good and there are days that I feel so low. I think about my dad almost everyday, but doing this blog has given me some discontent about the situation. Even though it feels like just yesterday, my dad passed away 8 months ago now. I will never completely jump over this hurdle of missing my dad, but I'm slowly coming out of the stage of unconsciousness. Before it was all just a bad dream and now, the dark clouds are starting to clear and give way to light.
So the more I sit down to investigate about CJD with this blog the more I feel like my dad was lost between the cracks. I do believe that this was God's plan for my dad, but I also believe that He gave us free will. This free will can lead us to make decisions that don't come from God and therefore, perpetuate death and destruction in the world. This is clearly represented with war, pollution, murder and among many others, disease. This is my own personal belief.
With this in mind, I've been feeling trapped behind all the unknown and conflicting information about CJD. This especially hit close to home, when I got to thinking about how we still haven't received my dad's autopsy results. My step-mom is the power of attorney so really the whole thing is out of my hands. However, I went ahead and did some investigation on my own. Several sources that I found stated that autopsy results and testing for CJD should take no more than 10 weeks. It has obviously been way longer than that and that makes me feel uneasy and confused.
I called my step-mom to discuss with her about my feelings and she filled me in on the situation. It turns out that the CJD survelliance center and my dad's doctor have been sending her through hoops. When she contacted the surveillance center they said that the autopsy results can only be given to the patient's doctor. From there, my step-mom understood that the results would be faxed to my dad's doctor. She went several weeks and never heard a word from Dr. Hill. She called him and he stated that he never received a fax. She then called the surveillance center and they supposedly said that they don't fax the results; they only send them by mail. Can you believe this run around? This was my dad's life; it should've been handled with the utmost care!
My step-mom realized after talking to us that this all didn't add up and it wasn't fair. She pressed the issue more and finally, recieved some confirmed information this Monday. He stated that my dad's autopsy and test results were finished back in March, but the fax that the surveillance center supposedly sent never made its way to his office. Can you believe this?? Well, he confirmed that my dad was diagnosed with Sporadic CJD. This calmed my worries and at the same time, made me feel very suspicious. I'm beyond thankful that it is not the familial variant, but now I'm full of many questions.
Why did this whole autopsy take so long? This is a rare disease and this was a person's life, you think they would have been more careful. This just frustrates me. Being that he passed away from the Sporadic variant of CJD means that there is basically no hard core fact as to why it happened (Just like some people suddenly acquire cancer or have a heart attack for no apparent reason). However, all of this talk about Mad Cow's disease and how it has been found in animals here, but there are no confirmed cases of this variant in humans definitely fills me with doubt. It just doesn't make sense. My dad was an avid hunter and ate all sorts of meat, including deer, elk, buffalo, etc. You name it, he ate it.
Are they hiding something from us? (They being the USDA or the government) Are they just saying that it is sporadic so that we don't question about Mad Cow's disease?
We need to know more!! If there is more than just what we see on the surface, than we all are at risk for CJD.
Please continue to spread the word!
-Lacy
Ps. I had the radio on as I was writing this and one of my dad's songs was played "Listen to the music". I totally felt him here with me at that moment. Is he telling me that I'm heading in the right direction with all of this?
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