USA: Young man diagnosed with unknown origin CJD in middle TN; Similar case in Canada

[Emily]

He's only 32, apparently no history in family.

https://www.newschannel5.com/news/le...ad-cow-disease

Posted: 9:18 PM, Dec 17, 2018
Updated: 8:51 PM, Dec 17, 2018
By: Emily Luxe
...

Danielle said she doesn’t know how Tony got CJD. Doctors told her it was rare he was diagnosed at such a young age.

 

[alert]

The young age does tend to argue against this being sporadic CJD, and the lack of family history probably argues against genetic. As the article indicates, I wonder if he had any previous surgeries that would raise the risk of him accidentally contracting it in that fashion.

I'm assuming variant CJD (aka mad cow disease) is probably ruled out simply by the location of the case, unless he had a history of living overseas for a prolonged period of time.

Variant CJD can be distinguished from classical based on studying the prions in his brain, but of course, that cannot be done on a living patient.

 

[Emily]

I think you are right that vCJD is not likely.

 

[Emily]

https://people.com/human-interest/tony-gibson-human-mad-cow-disease/

[h=1]Tennessee Dad of 4 Dies After Contracting Human Form of Mad Cow Disease[/h] Char Adams


February 05, 2019 02:26 PM


A Tennessee father of four has died less than a year after being diagnosed with what doctors call the human form of mad cow disease...

 

[Emily]

Mr. Gibson is reported below as having worked as a welder/ironworker:

https://www.foxnews.com/health/tenne...hopes-for-cure

I remembered Mark Purdy's theories about manganese being one of two factors in the evolution of prion disease in cows Britian:

https://www.monbiot.com/2000/11/23/m...and-manganese/

Later research supported a link:

http://www.sciencemag.org/news/2006/...ows-and-metals

CDC does not specifically mention prion disease but they do warn about manganese neurotoxicity being a threat to welders:

https://www.cdc.gov/niosh/topics/welding/default.html

Mrs. Gibson has generously donated her husband's tissues to the research community. I think this is really important since CJD reports are increasing and Mr. Gibson was so young. I suspect an environmental/work-related link. If that is the case, then other lives might be saved.

Death from CJD is excruciating for the victim and loved ones. Mrs. Gibson lost someone to ALS previously and said her husbands suffering was even worse. Here is a video of her singing to him at the end:

https://www.facebook.com/danielle.cl...user_video_tab

The People article has a link to a Gofundme account for the family.

 

[Emily]

Another tragic case in Canada. Ms. Lauman was also in her 30's so both these victims were born in the 1980's.

https://globalnews.ca/news/4933165/e...dementia-dies/

Young Edmonton mother with rare form of dementia passes away
By Caley Ramsay Online Supervisor Global News

..Last summer, Lauman was diagnosed with Creutzfeldt-Jakob disease (CJD), a rapidly progressing dementia. The 35-year-old’s diagnosis came shortly after she found out she was pregnant with her second child.
Watch below: Sabrina Lauman was one of the world’s youngest people ever to be diagnosed with CJD. Su-Ling Goh reports (Sept. 4, 2018)

There is a report of something like this noticed in Britian in 2014 with a case in a 36 yr old man. In this case vCJD was involved but was harder to diagnose and his genetics indicated a broader range of persons were susceptible, however there seems to be a trigger needed so fortunately not all prion carriers get ill.

https://www.newscientist.com/article...ase-in-the-uk/

Some new research from Slovakia points toward manganese again, even in familial cases the people who get sick have manganese out of balance with copper.
https://www.researchgate.net/publica...sible_Cofactor

I think I remember reading that Gajdusek believed that an environmental factor was involved in the Kuru epidemic. I wondered if mining in the area was involved.