Friday, January 18, 2013

An autopsied case of Creutzfeldt-Jakob disease with mutation in the prion protein gene codon 232 and type 1+2 prion protein

CASE REPORT



An autopsied case of Creutzfeldt-Jakob disease with mutation in the prion protein gene codon 232 and type 1+2 prion protein



Yasushi Iwasaki1,*, Fuji Yokoi2, Shinsui Tatsumi1, Maya Mimuro1, Katsushige Iwai3, Tetsuyuki Kitamoto4, Mari Yoshida1



Article first published online: 16 JAN 2013



DOI: 10.1111/neup.12013



© 2013 Japanese Society of Neuropathology



Keywords:



coarse-type PrP deposition; codon 232; Creutzfeldt-Jakob disease; prion protein gene mutation; type 1+2 PrP



A 68-year-old Japanese man gradually showed abnormal behavior and gait disturbance with bradykinesia. Slowly progressive dementia, including memory disturbance and disorientation, was also observed. Cerebral cortical hyperintensity on diffusion-weighted MRI was observed 6 months after onset. The patient progressed to an akinetic mutism state with mild myoclonus, and atypical periodic sharp-wave complexes were observed by electroencephalogram 13 months after onset. He was clinically suspected of having atypical CJD and died after 19 months total disease duration. The brain weighed 1160 g and showed mild atrophy of the cerebrum and cerebellum with ventricular dilatation. Spongiform changes with varying vacuole size and gliosis was extensive in the cerebral cortex and basal ganglia. Neuron loss in the cerebral cortex, basal ganglia and thalamus was relatively mild. The cerebellum showed mild spongiform changes of the molecular layer and mild neuron loss in the Purkinje cell layer. PrP immunostaining showed mainly coarse-type combined with diffuse synaptic-type PrP deposition in the cerebral gray matter. Some perivacuolar-type PrP deposition was also present. Numerous plaque-type PrP depositions were observed in the molecular layer of the cerebellum. Analysis of the PrP gene revealed a methionine-to-arginine (Met-to-Arg) substitution at codon 232 (M232R) with Met homozygosity at codon 129. Western blot analysis of protease-resistant PrP indicated type 2 dominant PrP combined with type 1. Genetic CJD with M232R substitution in the PrP gene has only been reported in Japan. Although two clinical phenotypes (rapid-type and slow-type) were suggested in the M232R CJD cases (despite the presence of the same PrP genotype), the pathological and molecular backgrounds have not been well understood because there have only been a few autopsied case reports. This is the first case report of M232R CJD presenting with 1 + 2 PrP.









Monday, January 14, 2013



Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe








Thursday, January 17, 2013



TSE guidance, surgical, dental, blood risk factors, Part 4 Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings (updated January 2013)









Friday, January 18, 2013



NPDPSC Gambetti et al pleading for more autopsies on suspect CJD cases Dear Clinician:





























































TSS

NPDPSC Gambetti et al pleading for more autopsies on suspect CJD cases Dear Clinician:


NPDPSC Gambetti et al pleading for more autopsies on suspect CJD cases Dear Clinician:
 
 
The National Prion Disease Pathology Surveillance Center (www.cjdsurveillance.com) Supported by the Centers for Disease Control and Prevention (CDC)
 
 
 
Dear Clinician:
 
 
 
The National Prion Disease Pathology Surveillance Center (NPDPSC) monitors the occurrence of prion disease through characterization of brain tissue pathology from cases clinically suspected of Creutzfeldt-Jakob disease (CJD). As the director of the NPDPSC, I want to make you aware of the following, which may be helpful in your work with current and future cases of suspected CJD:
 
 
 
* The 14-3-3 and Tau tests performed on cerebrospinal fluid do not definitively diagnose CJD and other prion diseases.
 
 
 
* Autopsy provides the only method to firmly diagnose CJD and to fully classify the etiology of CJD (sporadic, familial, or acquired by infection as in iatrogenic, or variant CJD). Autopsy also allows for the identification of atypical cases and possible novel sources of infection such as chronic wasting disease of elk and deer.
 
 
 
* Autopsies help to further our understanding of CJD. Tissues acquired at autopsy by the Center are made available to laboratories to do prion research.
 
 
 
* Ideally, autopsies should be performed within 24 hours post mortem. However, NPDPSC has successfully diagnosed cases autopsied up to seven days post mortem.
 
 
 
* NPDPSC offers a free autopsy coordination service. We make all of the arrangements, including locating the pathologist, arranging for transportation if necessary, and coordinating with the funeral home. NPDPSC will cover all costs associated with the autopsy.
 
 
 
* Pre-arranging the autopsy with NPDPSC can alleviate some of the stress on the family and can result in a more rapid and efficient autopsy process. NPDPSC staff are available to make autopsy arrangements at any time after the diagnosis of suspected CJD has been made.
 
 
 
* Our autopsy network includes major health centers throughout the United States. In most cases, an autopsy can be arranged and completed within 24 hours, including any necessary transportation. In the event of any delays (usually caused by weekend or holiday hours), the family will always be informed of the status.
 
 
 
* Embalming and viewing are still possible after a cranial autopsy is performed. NPDPSC staff members are available to consult with funeral homes to insure that families can still have their desired memorial services.
 
 
 
* Because of IRB regulations, results are reported to a clinician designated by the family. This facilitates answering questions and discussing any appropriate next steps with family members.
 
 
 
* If the autopsy shows that the patient does not have CJD, NPDPSC can assist families who would like further evaluation and a final diagnosis.
 
 
 
To make autopsy arrangements, or for more information about the autopsy program, please contact our autopsy coordination team at 1-216-368-0587.
 
 
 
Thank you for helping us to further our understanding of this significant health concern.
 
 
 
Sincerely,
 
 
 
Pierluigi Gambetti, M.D.
 
 
 
Professor and Director
 
 
 
National Prion Disease Pathology Surveillance Center
 
 
 
Case Western Reserve University
 
 
 
MAILING ADDRESS
 
 
 
Institute of Pathology
 
 
 
Division of Neuropathology
 
 
 
Case Western Reserve University
 
 
 
2085 Adelbert Road
 
 
 
Cleveland, Ohio 44106-4907
 
 
 
Telephone -
 
 
 
 
 
 
 
 
 
>>>Autopsy provides the only method to firmly diagnose CJD and to fully classify the etiology of CJD (sporadic, familial, or acquired by infection as in iatrogenic, or variant CJD). Autopsy also allows for the identification of atypical cases and possible novel sources of infection such as chronic wasting disease of elk and deer.<<<
 
 
 
IT could also mean that it’s the only way the USDA INC PRION UNIT can keep all human TSE prion disease NOT TIED WITH ANY LINK TO THE LIVESTOCK INDUSTRY, and keep it all linked to a spontaneous mutation, from nothing, except maybe a genetic TSE that is NOT linked to any gene mutation, and to continue to ignore the possibility being linked to maybe an atypical BSE such as the atypical hgBSE, hBSE, lBSE, or IBNC BSE $$$
 
 
 
AFTER what deep throat told me back in 2001-2002, and when the USDA INC. came out and announced that Aretha Vinson did not have mad cow disease, after her doctors diagnosed she did, and after all that mess in Korea, I knew then I could never trust the USDA INC PRION UNIT CWRU again, and there have been plenty other questionable nvCJD victims, all changed to sporadic CJD, and when that could not explain it away, another fake name would be called in i.e. sporadic FFI, sporadic GSS, the prionpathies, the prionopathies, or VPSP Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY, anything but everything that is not, and connot be tied to a zoonotic TSE prion disease in the USA, considering the USA has more documented TSE prion disease in the wild and livestock than any other country documented in the world, excluding the TSE prion disease documented at the zoos across the world, I could no longer in my heart trust them. but what the hell else do you do. like deep throat said, they hold all the cards, and they will use each and every one of them.
 
 
WHAT happened with Aretha Vinsons case, and after year after year, after more than a decade of trying to track human TSE CJD prion disease in the USA, and the excuses used by the USDA INC PRION UNIT, time and time again, CONFIDENTIALITY CLAUSE, CONFIDENTIALITY CLAUSE, CONFIDENTIALITY CLAUSE, WE CANNOT TELL YOU! WE CAN NEVER BREACH THE CONFIDENTIALITY CLAUSE. BUT, Gambetti et al can run to the cattle industry and break such confidentiality claus $$$ that’s o.k. ? no, it’s not o.k. ....TSS
 
 
 
 
 
Portsmouth woman did not die of mad cow-related condition, ___USDA___ says
 
 
 
By Nancy Young The Virginian-Pilot © May 7, 2008
 
 
 
Preliminary test results indicate that a 22-year-old Portsmouth woman who died in April did not have an illness that has been associated with eating contaminated beef, a U.S. Department of Agriculture official said this week.
 
 
 
"The epidemiologic characteristics of the illness and preliminary results of the neuropathologic testing of brain tissue obtained at autopsy indicate that the patient did not die of" a variant of Creutzfeldt-Jakob Disease (vCJD), which has been associated with mad cow disease, said Richard Raymond, USDA undersecretary for food safety. He was speaking Sunday to a group of Korean and American reporters in Washington.
 
 
 
"The U.S. Centers for Disease Control and Prevention has just provided us with that information, and I felt it was important to share with you today," he told the group. The comment appears on a statement on the safety of the U.S. beef supply that is available on the USDA's Food Safety and Inspection Service Web site.
 
 
 
Aretha Vinson died April 9 at Bon Secours Maryview Medical Center in Portsmouth. She had a degenerative brain condition that can be caused by a wide variety of things, and concerns were raised that she might have had vCJD. The state health department, CDC and the University of Virginia were involved in testing to determine the cause of death and whether it indicated a public health concern.
 
 
 
"There's no threat to the general public," said Michelle Peregoy, a Virginia Department of Health spokeswoman. She would not say more, citing patient confidentiality issues.
 
 
 
Nancy Young, (757) 446-2947, nancy.young@pilotonline.com
 
 
 
 
 
 
 
Virginia State University student Aretha Vinson has contracted a rare brain disorder called Creutzfeldt-Jakob Disease, a variant of Mad Cow Disease. Virginia state health officials are investigating how Vinson contracted the disease. Doctors suspect Vinson contracted the disease after having gastric bypass surgery, possibly from tainted medical instruments. Vinson's family says her health worsened after having gastric bypass surgery three months prior to the diagnosis.
 
 
 
 
 
UPDATE: Vinson passed away at 5:30pm on Wednesday, April 9, 2008.


 
 
 
 
 
 
 
The program, which now faces charges for playing up the possibility that the woman died of vCJD, said, “The CDC last Thursday announced the cause of death of Aretha Vinson, who died of symptoms similar to vCJD.” It quoted the CDC as saying although the suspected case received international media attention, the National Prion Disease Pathology Surveillance Center determined that the cause of death was not due to vCJD, a finding, it pointed out, that was similar to an announcement by the Department of Agriculture.


 



 
22 year old sporadic CJD ???
 
 
 
 
 
we will never know ;


 
In a telephone interview with the Chosun Ilbo, CDC spokesman Dave Daigle on Monday said the centers posted the announcement after performing their own checkup once the NPDPSC finished its investigation. He added that because the CDC only provide information on diseases, they have no plans to make a separate press release on the issue including the result of the investigation. ...
 


 
 
 
 
 
 
 
Now, the cdc et al usually hide behind patient confidentiality to hide cjd cases. but in this 22 years old, confidentiality was not the case, she was well known around the world, and the cdc et al chose to hide her final diagnosis. why, because it was another young victim in the USA with sporadic CJD ???
 
 
 
 
 
SEE FULL TEXT ;
 
 
 
Tuesday, June 17, 2008
 
 
 
Portsmouth woman did not die of mad cow-related condition, USDA says
 
 
 
UPDATE Updated Jun.17, 2008 08:34 KST
 
 
 
 
 
 
 
 
 
Wednesday, January 13, 2010
 
 
 
High Court Rules In Favor of PD Notebook 01-13-2010 21:21
 
 
 
High Court Rules In Favor of PD Notebook
 
 
 
snip...see full text ;
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
snip...skroll down towards bottom of the link below ;


 
 
 
 
 
 
 
 
Greetings everyone et al,


 
 
you all know how I feel about the USDA INC. NPDPSC PRION UNIT CWRU. I have pretty much been outspoken about my feelings there, and why in my opinion they are there. in my opinion, I also believe that the USDA et al have been systematically, and in secret, culling the USA cattle herd of mad cow disease, and this has been an ongoing for years, since the mad cows that were attempted to be covered up, and then they got caught, and the other 9,200 plus that were used with the least likely test to find. but, I feel compelled that I still must post this anyway. ...
 
 
 
 
kind regards,
 


 
terry
 
 
 
 
 
Monday, January 14, 2013
 
 
 
Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe
 
 
 
 
snip...
 
 
 
 
DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)


 
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
 
 
 
 
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
 
 
 
 
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
 
 
 
 
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"


 
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
 
 
 
 
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
 
 
 
 
END...TSS


 
snip...see full text ;
 
 
 
 
 
 
 
see full text and more here ;
 
 
 
 
 
Monday, January 14, 2013
 
 
 
Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe
 
 
 
 
 
 
 
 
Thursday, January 17, 2013
 
 
 
TSE guidance, surgical, dental, blood risk factors, Part 4 Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings (updated January 2013)
 
 
 
 
 
 
 
 
Thursday, January 17, 2013
 
 
 
Canada, U.S. agree on animal-disease measures to protect trade, while reducing human and animal health protection
 
 
 
 
 
 
 
TSS