Teen Boy Dies from Fatal Prion Disease

This teen was diagnosed with Fatal Familial Insomnia based on lesions found in his brain on autopsy when he died at 16 - but he did not have apparent insomnia OR the gene associated with the disease. His doctors want to put the word out for other doctors to watch for prion diseases in young people with very unusual neurological diseases that cannot be explained by any other cause.

Teen Boy Is Youngest to Have Rare Fatal Brain Disorder

For three years, a teen boy in North Carolina developed progressively worsening movement, speech and memory problems, but doctors remained unable to determine the cause of his deteriorating condition.

It was only after his death at age 16 that they found the answer: The boy had an extremely rare brain disorder called sporadic fatal insomnia, which is caused by prions, or abnormally folded proteins. The boy is the youngest person known to have had this disease, according to a new report of the tragic case.

...If a doctor is "faced with a patient with very a unusual neurological disease that cannot be explained by any other cause, then they should start considering the possibility of prion diseases, even if the patient is very young," Belay said.

Also see:

DARPA Funds Project to See How Meds Trigger Prion Diseases

New Prion Disease Found: Causes Diarrhea and Neuropathy

reply to post by soficrow

They need to check his surgical history (including dental procedures) and his diet.

He either ingested the prions or they were introduced into his body invasively.

The diet thing will be the tough one. He could have ingested neural tissue and never realized it.

reply to post by butcherguy


Ya, unless they can tell us how he contracted it, all they are doing is scaring the crap out of us. What a horrible thing foe this poor kid and his family.

butcherguy
reply to post by soficrow

 

They need to check his surgical history (including dental procedures) and his diet.

He either ingested the prions or they were introduced into his body invasively.

The diet thing will be the tough one. He could have ingested neural tissue and never realized it.

Prions can be inherited (genetically or epigenetically), "acquired" or even made in the body if conditions are right (wrong). Studies a few years ago showed that any general surgery anywhere in the body (eg., kneecaps) would result in prion disease about 20 years down the road (of special interest to athletes and might help explain the current epidemic of dementia in professional athletes [not just from concussions]).

In any event, prion diseases normally take decades to manifest - about 20 to 30 years. The interesting thing about this case from a medical perspective is that it was not genetic and the kid was just not old enough for the usual decades-long incubation from ingestion/external exposure. ...The teen's medical team are calling this case "sporadic" meaning they don't know how he got it - but no one has ruled out epigenetic inheritance. If it wasn't inherited we're looking at a strain that seems to be very rapidly progressive.

reply to post by kosmicjack

Ya, unless they can tell us how he contracted it, all they are doing is scaring the crap out of us.

I do not agree. I firmly, absolutely and emphatically believe knowledge is always a good thing. Of course, it is also a good thing to learn how to process information rationally and without emotion. That said, prion diseases do not need to be contracted - with the right exposure, we can create and culture the little suckers inside our own cells and bodies. Maybe living downstream or downwind from a chemical plant will do the trick. Maybe absorbing too much radiation from medical tests. Maybe partying a bit too hearty - or taking the wrong meds. All these things and more could result in our developing our own personal prion disease that we can pass on to our children (epigenetically). The good news is that epigenetic inheritance does NOT affect DNA and tends to disappear after a few generations if it's not beneficial.

Hot dogs, hamburgers, steaks, sloppy joes, meatballs..

what do they all have in common?

RIP kiddo

Now I feel guilty sophi, came in earlier today and gave this a S@F, did not realize you were pretty much talking to the wall. Are we going to be able to battle this in the future with nanotechnology or something or is this another harbinger of doom? I think we are all just a bit less than our game if it is accepted and admitted. All but me of course... I think...

reply to post by kosmicjack


I think they withhold information to keep from scaring the poop out of people. I mean we cant have everyone moving into perma bubble environments can we?

reply to post by gardener


What?! You didn't check my signature?

Prions Found in Plants


Check it out.

antar

Are we going to be able to battle this in the future with nanotechnology or something or is this another harbinger of doom?

reply to post by kosmicjack

 

I think they withhold information to keep from scaring the poop out of people. I mean we cant have everyone moving into perma bubble environments can we?

I really think we are way wrong to keep thinking in terms of "battling" diseases, making "war" on them and so on. What saves my sanity regarding prions is recognizing that they are an evolutionary mechanism. ...We keep changing our environment with chemical, radiation and other contaminations and the little darlings just keep dancing trying to help us adapt to the mess we made.

Unfortunately, the absolute worst environment would be a perma bubble one - talk about a petrie dish. And one day I WILL do my rant about prions in space. lol.

reply to post by soficrow


Mad cow was known to manifest itself in victims in England in as few ten years after ingestion. I had good reason to read up on it, I ate beef in the UK in 1990.

reply to post by butcherguy

Mad cow was known to manifest itself in victims in England in as few ten years after ingestion.

Thanks for the reminder. To clarify - the rate of progression in prion diseases (time to manifestation) depends on exposure levels and point of entry, as well as "competition" from other prion strains in the body. Also, my pet peeve is that few acknowledge the illnesses caused by prion infections in various tissues as they make their way from the gut to the brain after ingestion.

In this case - if this 16-year-old ingested enough prions to shorten the "incubation" to 10 years, then his initial exposure occurred at 6 years of age. In that case, his family or school mates are also infected and will have symptoms. ...But I don't think epigenetic inheritance has been ruled out.

reply to post by soficrow


Yes. I still wonder what is down the road for me. In addition to consuming UK beef, I ate beef brains once about 35 years ago. I don't think there is a big risk on that one though, the steer involved was grass/corn fed with no commercial feeds or supplements.

reply to post by butcherguy

Yes. I still wonder what is down the road for me. In addition to consuming UK beef, I ate beef brains once about 35 years ago.

The focus on beef is entirely inappropriate. Prions are everywhere - in our meds and plants, in our soil and water. The list goes on.

Not to feed anyone's paranoia, I actually came online to post the following news.


The Patient: A young 38 year-old mom.

Another fast progression with no explanation. Her symptoms started in November with a cough and vertigo. Christmas Eve, stroke symptoms sent her to hospital, but doctors found no evidence of stroke. By mid-January, she had slurred speech, a loss of motor skills, deteriorating vision, memory loss and was unable to walk. Days later she developed encephalitis (swelling of the brain). Diagnosed as a prion disease within 3 months.

Family hopes for miracle after young mom struck by disease

After months of debilitating symptoms with no diagnosis, doctors this week say Sandi Tucker Kennedy, a 38-year-old wife and mom of four young children ages 2 to 9, is believed to have a prion disease, a family of rare progressive neurodegenerative disorders that take over the neurological system.

“She's actually very young for it,” …

“The neurological condition is going to continue. It's a rapid-moving disease,” Tucker said. “The prognosis is four to 12 months from the onset of symptoms and she's had symptoms for four months now.”

...Got any references for 3 month progression?

reply to post by soficrow

The focus on beef is entirely inappropriate.

My focus is really more on neural tissue being consumed or transferred via invasive surgical procedures. I have thought about beef brains specifically because I consumed them. I did not eat pork, sheep, chicken or squirrel brains, so I do not have a personal interest their risk. I mention squirrel brains because eating them has been linked to prion disease in Kentucky... NYTimes

I believe the people with the highest risk of prion diseases that cause encephalopathy are people that consume neural tissue from mammals. I can't cite studies, but this is the feel I have for it.

reply to post by butcherguy

My focus is really more on neural tissue being consumed or transferred via invasive surgical procedures. ...I believe the people with the highest risk of prion diseases that cause encephalopathy are people that consume neural tissue from mammals. I can't cite studies, but this is the feel I have for it.

Yes, prion-infected neural tissue transferred via surgery near the brain would confer the highest risk of prion-related encephalopathies; this has to do with the point of entry as well as the strain. However, even if prion-infected neural tissue carries the highest levels of infectious prions compared to other tissues (which does not apply to all prion strains), ingested prions still must travel a long and complicated path to get from the gut to the brain.

The case of the 38 year-old mom cited above may be explained by ingestion, but I doubt the teen case can be. I suspect the 16 year-old originally was infected via epigenetic inheritance, consider airborne infection unlikely, think a more rapidly progressive strain must be considered - but most strongly suspect a super-imposed infection (probably flu) allowed already present prions to use the immune system to spread in the body.

My ongoing concern about epidemics and pandemics is precisely that: prions use the immune system to spread in the body. Given that infection with prions and other misfolded proteins is widespread, deaths are just the tip of the iceberg - the real problem is the burden of chronic disease unleashed in survivors.
NOTE: Protein misfolding can result in several different types of diseases, including diseases that are infectious, genetic, epigenetic, chronic, degenerative, and almost always debilitating.


Also of interest:

* Longer exposures to prion-containing aerosols are strongly correlated with shortened incubation periods; with inhalation, exposure time is more important than exposure level - in contrast to ingestion - when prion-contaminated foods are eaten, the amount or number of prions affects the speed and degree of disease development.

* Airborne prion infection is independent of the immune system - in contrast to ingestion - when prion-contaminated foods are eaten, the prions use the immune system to spread in the body.

reply to post by butcherguy


Great link about squirrels - 1997, before the gag orders. In any event, looks like I was on the right track with the super-imposed infection idea - people with subclinical prion disease can be levelled by any infection affecting the brain: infection causes inflammation; inflammation attracts prions, accelerates the infection rate and causes rapid progression.

Targeting of prion-infected lymphoid cells to the central nervous system accelerates prion infection

...inflammation did not change the virulence properties of the initial prion strain, but rather accelerated the targeting of the infectious agent to vital areas, resulting in a shorter disease.

Also, check out this 2007 paper:

...individuals incubating prion disease may be highly susceptible to CNS inflammation. ...During the years or decades of prion disease incubation, especially with low or subclinical prion titers, at-risk individuals are certain to encounter diverse pathological insults, such as viral and bacterial infections, autoimmune disease, or neoplastic processes, all of which may have an inflammatory component. These individuals may develop severe neurological diseases not immediately associated with prion infections due to the dissociation of the putative clinical findings from the accumulation of PrPSc. If so, then the effects of the bovine spongiform encephalopathy epidemic on the population should be looked upon differently.

From your link:

Dr. Frank Bastian, a neuropathologist at the University of South Alabama in Mobile, said he knew of similar cases of Creutzfeldt-Jakob disease in Alabama, Mississippi and West Virginia.

Not saying you're wrong about eating neural tissue, just sayin' it's a very small part of the big picture.

I read your other thread as well and am trying to understand this. Prions exist regardless but sometimes get too overwhelming for our bodies to handle? Somewhat like oxidation but more of a "character" or "intelligence to it". If so - what can we do to reverse this problem? Because I've had some pretty severe cognitive issues and it's a truly terrifying thing at my age - there is no way I have even early onset Alzheimer's but my doc - in just the last couple of months, suspects autoimmune. I have none of this in my family history and have been super healthy my whole life.

I heard you say eat a variety of foods and keep the body alkaline (I think you said this). What more can be done - just in case.

Dianec
I read your other thread as well and am trying to understand this. Prions exist regardless but sometimes get too overwhelming for our bodies to handle?

Proteins misfold in response to environmental change, and sometimes, become infectious. It's a fact of life. Our bodies get overwhelmed if a) we ingest lots of food contaminated with prions, or b) get exposed to lots of chemical and other contaminants, change our internal 'environment' and trigger lots of misfolding.

...Inflammation changes the process because prions congregate at sites of inflammation, speeding the prion disease process.

If so - what can we do to reverse this problem?

Eat a varied diet with food from different sources/areas, and minimize your exposure to chemical and other contaminants. Try not to get sick; if you have inflammation, deal with it preferably by natural means.

I heard you say eat a variety of foods and keep the body alkaline (I think you said this). What more can be done - just in case.

I did not say to keep the body alkaline and have no comments on so-called alkaline diets.

It's odd they said it was a case of Sporadic Fatal Insomnia when all documented cases of FFI/SFI the person once showing signs normally don't live more then 6-18 months. The longest case was a man who lived for 36 months after showing signs. They also lack to mention if the kid did have the severe insomnia were FFI/SFI get it's name from since it damages the part of the brain that regulates sleep making it litarrly impossible to sleep even chemically induced, the person might look "asleep" but they are still awake in all reality. It sounds like while the scarring was like SFI unless they are hiding the details of him not sleeping it sounds more like CJB or a variant of it which can cause similar scarring. Either way like you mentioned and the article for someone that young to develop a prion disease is extremely rare. Not a fun thing to have to go though or witness a loved one to go through that's for sure.