UA-cam timestamps by Les 01:36 guest definition prion disease limited to misfolded prion protein other neurogenerative diseases seem to involve other misfolded proteins 02:46 diseases with misfolded prion protein 04:01 the 4 ways humans get the disease Prof Vincent Lecture on topic: ua-cam.com/video/DJq-tFLrypU/v-deo.html incubation period measured in years 08:07 What makes patients seek a doctor 09:15 diagnosing prion vs other neurological diseases 13:43 what treatments or help is available 17:12 What is the incidence? 18:09 Surveillance 20:40 BSE 37:22 Chronic Wasting disease spreads easily but does it spread to people 39:59 British beef spreading prion disease and how that was stopped 42:25 how to Support this show Website timestamps by Jolene
I think this conversation highlights the importance of allowing organisms to eat their species-appropriate diet. Ruminants should eat grass/hay/other cellulose and not processed repurposed animal leftovers or grain for that matter. When ruminants eat what they are meant to eat, their waste will replace the topsoil (that is disappearing at an alarming rate). All farm animals should eat what they are meant to as well as be able to room around in nature interacting with it and eating what they need from it. This is the basis of regenerative farming.
Geez, I wanted to say Holy Cow after watching this, but my teenage grandchildren warned against it, due to the video subject matter, so I will say Holy Moly. I love these videos immensely, I learn more from Vincent and his team than I’ve learned in 30 years of working in the medical field, and a special shout-out to Daniel Griffin, and of course our lovely Amy! You gentlemen (and ladies) are truly our heroes for bringing us all of this timely information. If nothing else, it lends itself to the age-old advice to enjoy life, it can be cut short in so many, many ways! Vincent’s work is a great cause to contribute to, I hope that everyone will consider a generous donation! We need more science in our world!!
Anyone who talks about the prions is a legend as this field has so many less number of researchers and less volunteers, these guys are really impressive!
I'm in Scotland, UK and was given many blood transfusions in 1997, the Scottish blood Bank will not allow me to give blood still and have no plans to change this. There are still putting protections in.
As a US deer hunter, I've been interested/ worried about "mad deer disease". I suspect a practice that may contribute to its spread: some individual hunters, clubs, commercial hunting operations place "mineral blocks" out as nutrition supplements, to grow larger antlers. Mineral block ingredient lists often include "bone meal", and I am concerned that infectious prions could survive processing and be spread. Mineral blocks are also used in US cattle industry.
I could be wrong, but in deer, isn't it called "chronic wasting disease"? Also, I was under the impression that BSE resulted from ground sheep in cattle feed, not salt licks. I looked up the ingredients in mineral blocks, which are "Salt, Calcium Sulfate, Iron Oxide, Manganous Oxide, Zinc Oxide, Ferrous Carbonate, Calcium Carbonate, Copper Oxide, Calcium Iodate, Cobalt Carbonate, Sodium Selenite and Molasses Products", no bone meal. Another commercial salt lick had yeast, not molasses, but no bone meal. I looked at instructions for DIY salt licks for hunters, some contained cement or mineral oil, but no bone meal, mostly they were made of salt and molasses. Where did you get the idea, that their is bone meal in salt licks?
In the UK there was a clustering of cases in a village called Quenenborough in Leicestershire. The village had a traditional butcher who processed the whole beef carcass so that there was contamination of bone marrow, neural, lymphatic and splenic tissue ( all of which contain high levels of prions) via saws and knives into other cuts of meat- particularly minced meat . I suspect dear hunters processing the carcass just take the meat without opening the brain or grinding up the bones, therefore their risk would be somewhat lower. There are a number of research projects in the USA where very high levels of prions have been found in the soil in some areas associated with CWD. Cause or effect? Update: i have just read the CDC guidance. They state that you should never eat wasted , sick or road kill cervids. And cases in farmed or captive animals are higher than in the wild population. States with high numbers of cases should be testing for CWD, and meat should be tested before consumption. If no test is available, you probably shouldn’t eat the meat because a study has been done where macaque monkeys were fed infected meat and developed prion neuropathy. Transmission of CWD from cervids to humans is denied, because it hasn’t been proven. BUT for me the fact that a species jump from deer to primates ( monkeys) means there is no evidence that it DOESN’T transmit. Personally, i wouldn’t eat ANY cervid meat of US, Canadian, Norwegian or S Korean origin.
@@lulabellegnostic8402 so the study you are likely referring to actually found that macaques were not susceptible but that 100% of spider monkeys inoculated intracebrally were and 92% inoculated orally were. The study suggested that this means we are likely not at risk as we are more closely related to macaques. Still, CWD is a scary thing and I think caution should be taken.
I was working as a junior dr in Cambridge ( UK) in ‘87 on the neurology ward. We had a patient ( who worked at a nearby academic animal research institute) who was ataxic and myoclonic. Investigations back then were limited, and everything came back negative. I was chatting to the professor in charge of the case, and he said ‘ we’ll probably never get a diagnosis, even after post mortem. But i for one have stopped eating lamb’. I asked why. He said ‘scrapie’. So off i went to the library. I too stopped eating lamb.
Very interesting interview! I gather the rather distressing disease name “fatal familial insomnia” has been changed? I did not hear it mentioned in the discussion.
28:00 Native Americans used brains as an emulsifying agent in tanning animal skins. Brain tanned leather is something exquisite to the touch. I wonder if prion diseases were found amongst this population, prior to Europeans.
Hi Vincent Very nice episode I think protein folding is a very interesting tool to explain such neural disease as explained by your guest Also i think Dr. Susan lindqest Explained how chaperone protein Hsp90 playes an important role in the cascade of developing the disease. Dr Shreiner
@V. Great topic and guest. Although we may be eating less meat it is rather concerning that animal organ tissue like brain could end up being used as an emulsifying agent...consumers wouldn't know or question it. We also rely heavily on best practices being used at slaughter houses. One would hope that the future might hold more promise regarding arresting the production of abnormal folding proteins once the disease has been diagnosed thus halting/delaying its progression.
(Previous comment ctd.) If deer can pick up CWD prions from the landscape, what's to prevent cattle, which we eat, from picking up the same prions and developing the same sort of disease, which then becomes a wildfire in the human population? It seems to me only a matter of time before this becomes quite a disaster.
Well.... We've known about the routes of communication and infection for a couple decades at least, and we successfully reduced the incidence by simply changing standards and regulations. As the researcher stated, there is spontaneous CJD and yet it doesn't become a dumpster fire problem. Also, as the researcher stated, our bodies have detection and protection mechanisms that likely get less reliable with age. Finally, the researcher also said, explicitly, that prion disease is very difficult to transmit; wasting disease is easy.
My Father died of sporadic CJD at the age of 63 10 years ago. I was told by members of family that he had the pathology test to verify he had not died of genetic CJD but they lied. So I will never know if it was genetic CJD he died of and consequently I worry for my kids and for me. In fact I wonder if this fella diagnosed my Father. His name rings a bell.
It’s happening to me but they are calling it Somatisism whick is bull crap. Constant pressure in the head, off balance pins and needles all over. Head to toe. On morphine patch.
neurodegenerative diseases are devastating. some of them such as ataxia is quite rare so there is not as much research monies as there are for cancer etc. its very difficult for the whole family especially when it is hereditary and your children are at risk
Hi I would like to declare that this is a very slow developing disease so i think from a clinical point of view it is difficult to affect children or even young men Dr Shreiner.
I am concerned about possible implications of chronic wasting disease amongst deer especially in the American west. It's my understanding that the prions associated with this disease are freely shed by infected animals, which may be why the disease is so widespread among c e r v i d s
Fatal Familial insomnia is a prion, and when you think of the word Familial (Family) and prions can't be destroyed and are Transmissible, might that suggest prions are inherited at birth, also Jaundice is a sign of liver damage, something doesn't sound right.
"I appreciate the conversation", as well. However, still vage of an (brain-degenerative-disease) explanation, I'm glad word is out again & conversations, colaborations and more research can be picked up & worked on for bringing more clarity & sollutions (in case "funding" hit the road, Jack). I mean: is a keep on 'rolling', I guess... 😒 Now: Anyone forwarding this, same conversation to ex-MD's in my neigbourhood? Seemingly, here, we still live in the 'middleages' (not even; during those times, "dr.'s" might not have had awfully much knowleadge & machines/tools but there still was some kind of empathy, some sort of curiosity 'thirst' & therefor a more listening ear to the patient instead of arrogant attitudes of stetoscopes, eye-light-flashes and oh, now new: SPO2 finger measurement? Or was it that this sort of patients-discrimination has been in place at all money times?). PSQ: The "sporadic" type of encephalitis might be brought to you(men) by a 'druplet from the sky'? 🤔 Since the cigaar smoking random & entropic theories, I wouldN't be surprised as of what else happens in the cogniac-brown shoes walking-lobies... 😔
The Food Safety and Inspection Service (FSIS) has amended the Federal meat inspection regulations to prohibit the use of penetrative captive bolt stunning devices that deliberately inject air into the cranial cavity of cattle. This rulemaking responds to the findings of a risk assessment on bovine spongiform encephalopathy (BSE) conducted by the Harvard Center for Risk Analysis (referred to as the Harvard study) and is part of a series of actions that the USDA is taking to strengthen its BSE prevention programs. The Harvard study found that, owing to already ongoing Federal programs, the U.S. is highly resistant to the introduction and spread of the disease. Even so, the USDA response to BSE has always been proactive and preventive. Therefore, FSIS is taking this action to address the potential risk posed by stunning devices that may force visible pieces of brain, known as macro-emboli, into the circulatory system of stunned cattle. So, along with "Do not eat your dead" (Kuru Prevention League) we've got a regulatory response from the days of dairy farmers hobbling up a BSE infected animal and passing it off at the slaughter house as a parlor splitter which then moved that animal into the meat supply to humans and / or canines. (Parlor Splitter: infrequently, a very heavy dairy cow might slip on a wet, slippery milking parlor floor and fall injured, legs flailed out, muscles torn and unable to stand unless hobbled, which allowed enough time to get the beast to slaughter. A BSE victim resembles a "parlor splitter" due brain degenerations and thus hobbled, had been passed off to omnivores food chain. I do not know many vegans who changed into keto-carnivores after learning about milk pus, slaughter house practices, blood gutters or reading James Agee's "A Mother's Tale." 🎉 Cheers! 🍻
It's a cellular protein that in 99.999 percent of cases functions perfectly fine but I'm a very rare case it can misfold and other prions follow suit and fold incorrectly and this results in a build up of protein clumps on the brain that cannot be cleared
UA-cam timestamps by Les
01:36 guest definition prion disease limited to misfolded prion protein
other neurogenerative diseases seem to involve other misfolded proteins
02:46 diseases with misfolded prion protein
04:01 the 4 ways humans get the disease
Prof Vincent Lecture on topic:
ua-cam.com/video/DJq-tFLrypU/v-deo.html
incubation period measured in years
08:07 What makes patients seek a doctor
09:15 diagnosing prion vs other neurological diseases
13:43 what treatments or help is available
17:12 What is the incidence?
18:09 Surveillance
20:40 BSE
37:22 Chronic Wasting disease spreads easily but does it spread to people
39:59 British beef spreading prion disease and how that was stopped
42:25 how to Support this show
Website timestamps by Jolene
I think this conversation highlights the importance of allowing organisms to eat their species-appropriate diet. Ruminants should eat grass/hay/other cellulose and not processed repurposed animal leftovers or grain for that matter. When ruminants eat what they are meant to eat, their waste will replace the topsoil (that is disappearing at an alarming rate). All farm animals should eat what they are meant to as well as be able to room around in nature interacting with it and eating what they need from it. This is the basis of regenerative farming.
Wow, what a great interviewee is Richard Knight: accurate, approachable and fun. This was a great episode. Thanks, TWIV!
Geez, I wanted to say Holy Cow after watching this, but my teenage grandchildren warned against it, due to the video subject matter, so I will say Holy Moly. I love these videos immensely, I learn more from Vincent and his team than I’ve learned in 30 years of working in the medical field, and a special shout-out to Daniel Griffin, and of course our lovely Amy! You gentlemen (and ladies) are truly our heroes for bringing us all of this timely information. If nothing else, it lends itself to the age-old advice to enjoy life, it can be cut short in so many, many ways! Vincent’s work is a great cause to contribute to, I hope that everyone will consider a generous donation! We need more science in our world!!
Wonderfully different. I became interested in prion diseases after reading an article about them in the NY Times. How awful. Thanks for the update.
Anyone who talks about the prions is a legend as this field has so many less number of researchers and less volunteers, these guys are really impressive!
This was great introduction to prion diseases. short and to the point.
I'm in Scotland, UK and was given many blood transfusions in 1997, the Scottish blood Bank will not allow me to give blood still and have no plans to change this. There are still putting protections in.
As a US deer hunter, I've been interested/ worried about "mad deer disease". I suspect a practice that may contribute to its spread: some individual hunters, clubs, commercial hunting operations place "mineral blocks" out as nutrition supplements, to grow larger antlers. Mineral block ingredient lists often include "bone meal", and I am concerned that infectious prions could survive processing and be spread. Mineral blocks are also used in US cattle industry.
I could be wrong, but in deer, isn't it called "chronic wasting disease"? Also, I was under the impression that BSE resulted from ground sheep in cattle feed, not salt licks. I looked up the ingredients in mineral blocks, which are "Salt, Calcium Sulfate, Iron Oxide, Manganous Oxide, Zinc Oxide, Ferrous Carbonate, Calcium Carbonate, Copper Oxide, Calcium Iodate, Cobalt Carbonate, Sodium Selenite and Molasses Products", no bone meal. Another commercial salt lick had yeast, not molasses, but no bone meal. I looked at instructions for DIY salt licks for hunters, some contained cement or mineral oil, but no bone meal, mostly they were made of salt and molasses. Where did you get the idea, that their is bone meal in salt licks?
In the UK there was a clustering of cases in a village called Quenenborough in Leicestershire. The village had a traditional butcher who processed the whole beef carcass so that there was contamination of bone marrow, neural, lymphatic and splenic tissue ( all of which contain high levels of prions) via saws and knives into other cuts of meat- particularly minced meat . I suspect dear hunters processing the carcass just take the meat without opening the brain or grinding up the bones, therefore their risk would be somewhat lower. There are a number of research projects in the USA where very high levels of prions have been found in the soil in some areas associated with CWD. Cause or effect?
Update: i have just read the CDC guidance. They state that you should never eat wasted , sick or road kill cervids. And cases in farmed or captive animals are higher than in the wild population. States with high numbers of cases should be testing for CWD, and meat should be tested before consumption. If no test is available, you probably shouldn’t eat the meat because a study has been done where macaque monkeys were fed infected meat and developed prion neuropathy. Transmission of CWD from cervids to humans is denied, because it hasn’t been proven. BUT for me the fact that a species jump from deer to primates ( monkeys) means there is no evidence that it DOESN’T transmit. Personally, i wouldn’t eat ANY cervid meat of US, Canadian, Norwegian or S Korean origin.
@@lulabellegnostic8402 so the study you are likely referring to actually found that macaques were not susceptible but that 100% of spider monkeys inoculated intracebrally were and 92% inoculated orally were. The study suggested that this means we are likely not at risk as we are more closely related to macaques. Still, CWD is a scary thing and I think caution should be taken.
I was really impressed and was not expecting this epitope to be that cool and useful! Excellent teaching!
Thank you for doing this very interesting and informative interview together, Vincent and Richard.
I was working as a junior dr in Cambridge ( UK) in ‘87 on the neurology ward. We had a patient ( who worked at a nearby academic animal research institute) who was ataxic and myoclonic. Investigations back then were limited, and everything came back negative. I was chatting to the professor in charge of the case, and he said ‘ we’ll probably never get a diagnosis, even after post mortem. But i for one have stopped eating lamb’. I asked why. He said ‘scrapie’. So off i went to the library. I too stopped eating lamb.
Very interesting interview! I gather the rather distressing disease name “fatal familial insomnia” has been changed? I did not hear it mentioned in the discussion.
Very fascinating discussion. One thing that really interests me is how cjd mimics so many other diseases
Cool. I've been interested in this topic since the outbreaks were big news twenty-five or thirty that's ago.... Thanks!
28:00 Native Americans used brains as an emulsifying agent in tanning animal skins. Brain tanned leather is something exquisite to the touch. I wonder if prion diseases were found amongst this population, prior to Europeans.
Fantastic, learned a lot: and I'll never be able to watch 'Bonanza' again without thinking of CWD.
Hi Vincent
Very nice episode
I think protein folding is a very interesting tool to explain such neural disease as explained by your guest
Also i think Dr. Susan lindqest
Explained how chaperone protein Hsp90 playes an important role in the cascade of developing the disease.
Dr Shreiner
@V. Great topic and guest. Although we may be eating less meat it is rather concerning that animal organ tissue like brain could end up being used as an emulsifying agent...consumers wouldn't know or question it. We also rely heavily on best practices being used at slaughter houses. One would hope that the future might hold more promise regarding arresting the production of abnormal folding proteins once the disease has been diagnosed thus halting/delaying its progression.
Look into "natural flavors"
What an interesting subject!
Such an interesting episode!! Thanks!
(Previous comment ctd.) If deer can pick up CWD prions from the landscape, what's to prevent cattle, which we eat, from picking up the same prions and developing the same sort of disease, which then becomes a wildfire in the human population? It seems to me only a matter of time before this becomes quite a disaster.
Well.... We've known about the routes of communication and infection for a couple decades at least, and we successfully reduced the incidence by simply changing standards and regulations. As the researcher stated, there is spontaneous CJD and yet it doesn't become a dumpster fire problem. Also, as the researcher stated, our bodies have detection and protection mechanisms that likely get less reliable with age. Finally, the researcher also said, explicitly, that prion disease is very difficult to transmit; wasting disease is easy.
Fascinating. Thank you.
My Father died of sporadic CJD at the age of 63 10 years ago. I was told by members of family that he had the pathology test to verify he had not died of genetic CJD but they lied. So I will never know if it was genetic CJD he died of and consequently I worry for my kids and for me. In fact I wonder if this fella diagnosed my Father. His name rings a bell.
It’s happening to me but they are calling it Somatisism whick is bull crap. Constant pressure in the head, off balance pins and needles all over. Head to toe. On morphine patch.
Great Interview !
neurodegenerative diseases are devastating. some of them such as ataxia is quite rare so there is not as much research monies as there are for cancer etc. its very difficult for the whole family especially when it is hereditary and your children are at risk
Hi
I would like to declare that this is a very slow developing disease so i think from a clinical point of view it is difficult to affect children or even young men
Dr Shreiner.
I am concerned about possible implications of chronic wasting disease amongst deer especially in the American west. It's my understanding that the prions associated with this disease are freely shed by infected animals, which may be why the disease is so widespread among c e r v i d s
I'm worried about transmission to humans..
Wonderful program...I'm not going to eat man made processed glop though.
Gertsmann Straussler Scheinker disease is my absolute favorite
Fascinating and scary. Very interesting -thanks
Thank you for this discussion, sir. Informative and poignant podcast today.
Interesting discussion. Personally, I'd be on the phone to Dignitas as soon as I was confirmed with one of these syndromes. That's just me though
Really interesting and so well presented thankyou.
Fatal Familial insomnia is a prion, and when you think of the word Familial (Family) and prions can't be destroyed and are Transmissible, might that suggest prions are inherited at birth, also Jaundice is a sign of liver damage, something doesn't sound right.
"I appreciate the conversation", as well.
However, still vage of an (brain-degenerative-disease) explanation, I'm glad word is out again & conversations, colaborations and more research can be picked up & worked on for bringing more clarity & sollutions (in case "funding" hit the road, Jack).
I mean:
is a keep on 'rolling', I guess... 😒
Now:
Anyone forwarding this, same conversation to ex-MD's in my neigbourhood?
Seemingly, here, we still live in the 'middleages' (not even; during those times, "dr.'s" might not have had awfully much knowleadge & machines/tools but there still was some kind of empathy, some sort of curiosity 'thirst' & therefor a more listening ear to the patient instead of arrogant attitudes of stetoscopes, eye-light-flashes and oh, now new: SPO2 finger measurement?
Or was it that this sort of patients-discrimination has been in place at all money times?).
PSQ:
The "sporadic" type of encephalitis might be brought to you(men) by a 'druplet from the sky'? 🤔 Since the cigaar smoking random & entropic theories, I wouldN't be surprised as of what else happens in the cogniac-brown shoes walking-lobies... 😔
The Food Safety and Inspection Service (FSIS) has amended the Federal meat inspection regulations to prohibit the use of penetrative captive bolt stunning devices that deliberately inject air into the cranial cavity of cattle. This rulemaking responds to the findings of a risk assessment on bovine spongiform encephalopathy (BSE) conducted by the Harvard Center for Risk Analysis (referred to as the Harvard study) and is part of a series of actions that the USDA is taking to strengthen its BSE prevention programs.
The Harvard study found that, owing to already ongoing Federal programs, the U.S. is highly resistant to the introduction and spread of the disease. Even so, the USDA response to BSE has always been proactive and preventive.
Therefore, FSIS is taking this action to address the potential risk posed by stunning devices that may force visible pieces of brain, known as macro-emboli, into the circulatory system of stunned cattle.
So, along with "Do not eat your dead" (Kuru Prevention League) we've got a regulatory response from the days of dairy farmers hobbling up a BSE infected animal and passing it off at the slaughter house as a parlor splitter which then moved that animal into the meat supply to humans and / or canines.
(Parlor Splitter: infrequently, a very heavy dairy cow might slip on a wet, slippery milking parlor floor and fall injured, legs flailed out, muscles torn and unable to stand unless hobbled, which allowed enough time to get the beast to slaughter. A BSE victim resembles a "parlor splitter" due brain degenerations and thus hobbled, had been passed off to omnivores food chain.
I do not know many vegans who changed into keto-carnivores after learning about milk pus, slaughter house practices, blood gutters or reading James Agee's "A Mother's Tale." 🎉 Cheers!
🍻
@@robertoconnor371 God help us. Karma is not without irony
Nice cold open.
?????? Prions are not viruses are they ????????
No. Misfolded proteins which are encoded by prnp
It's a cellular protein that in 99.999 percent of cases functions perfectly fine but I'm a very rare case it can misfold and other prions follow suit and fold incorrectly and this results in a build up of protein clumps on the brain that cannot be cleared
What a fiddler, winding that string 6 times around his left index..over and over...very distracting!...haha