Case Report
Sporadic Fatal Insomnia in an Adolescent
Jennifer L. Blase, MPHa, Laura Cracco, PhDb, Lawrence B. Schonberger, MDa,
Ryan A. Maddox, PhDa, Yvonne Cohen, BSb, Ignazio Cali, MSb, and Ermias D. Belay,
MDa + Author Affiliations
aDivision of High-Consequence Pathogens and Pathology, National Center for
Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and
Prevention, Atlanta, Georgia; and bNational Prion Disease Pathology Surveillance
Center, Case Western Reserve University, Cleveland, Ohio Abstract The occurrence
of sporadic prion disease among adolescents is extremely rare. A prion disease
was confirmed in an adolescent with disease onset at 13 years of age. Genetic,
neuropathologic, and biochemical analyses of the patient’s autopsy brain tissue
were consistent with sporadic fatal insomnia, a type of sporadic prion disease.
There was no evidence of an environmental source of infection, and this patient
represents the youngest documented case of sporadic prion disease. Although
rare, a prion disease diagnosis should not be discounted in adolescents
exhibiting neurologic signs. Brain tissue testing is necessary for disease
confirmation and is particularly beneficial in cases with an unusual clinical
presentation.
Monday, February 24, 2014
Sporadic Fatal Insomnia in an Adolescent
http://sporadicffi.blogspot.com/2014/02/sporadic-fatal-insomnia-in-adolescent.html
a response to this case from the Doctors that treated ;
Comment on Sporadic Fatal Insomnia in an Adolescent
a.. Michael Tennison, Professor of Neurology and Pediatrics
b.. Heather Baudet M.D. Ph.D., Joseph Muenzer M.D. Ph.D.
UNC Chapel Hill
We read with interest the report by Blas? et al. as we were the physicians who actually cared for the child. We learned that this manuscript was in press upon contacting the National Prion Disease Pathology Surveillance Center (NPDPSC) to include them in our own manuscript. We were disappointed and surprised that the CDC "case
More...
We read with interest the report by Blas? et al. as we were the physicians who actually cared for the child. We learned that this manuscript was in press upon contacting the National Prion Disease Pathology Surveillance Center (NPDPSC) to include them in our own manuscript. We were disappointed and surprised that the CDC "case investigation" did not include speaking with the physicians who treated the patient. It is difficult to extract nuances of a case from a medical record without firsthand knowledge of the patient. The restrictions of space allow us to correct only some of the errors in the manuscript. The presenting symptom was diplopia, which in retrospect is characteristic of sporadic fatal insomnia (sFI). Subsequently, the most prominent symptoms were non-specific incoordination and cognitive changes with very prominent pseudobulbar affect and anxiety. There was a stepwise deterioration after two head injuries followed by strabismus surgery. A major abrupt decline then occurred with a H1N1 illness. Hyperkinetic is imprecise and ballismus is an incorrect characterization of the movements which were appendicular and gait cerebellar ataxia. The authors inaccurately state that no evaluation of insomnia or dysautonomia was performed. In fact, the family reported that the patient had difficulty sleeping only toward the end of his life despite direct questioning at each visit and declined a sleep study when offered. The child did develop incontinence as an autonomic issue but had no cardiovascular or vasomotor changes. We believe no reasonable clinician would stop pursuing alternative diagnoses based solely on polysomnogram or PET results. Head injury was never "diagnostically deceptive" to the neurology team. Could head injury, like surgery and severe influenza, be a trigger for a step-wise deterioration in sFI? A mitochondrial disorder was initially considered as the patient had a de novo POLG1 gene variant (p.V742M), but an outside mitochondrial expert agreed with the pursuit of alternate explanations, including prion-related disorders. The authors fail to note that two of the treating physicians independently raised the possibility of a prion disease. Therefore, the statement that a prion diagnosis was not pursued is false. In March of 2011 after further deterioration, the NPDPSC declined free-of-charge PRNP sequencing stating that the clinical picture was inconsistent with prion disease. We then pursued in-house analysis of the PRNP gene, specifically looking for the permissive methionine at codon 129 as well as the D178N mutation known to cause fatal familial insomnia. Brain biopsy would have potentially been diagnostic and was considered. We felt that the likelihood of a treatable diagnosis being discovered on brain biopsy was small and elected to defer to autopsy. Without aggressive pursuit of a prion diagnosis, amongst others, and counseling about the need for autopsy, a diagnosis would have never been reached. Rather than procuring our notes via the state health department and using our observations and assessments without citation, we believe including the treating physicians who actually saw the patient in the investigation and manuscript would have avoided inaccuracies and inferior science.
Conflict of Interest:
None declared
Published April 28, 2014
http://pediatrics.aappublications.org/content/133/3/e766.abstract/reply#pediatrics_el_59985
Seems (to me at least), Belay et al are always trying to sway the verdict (or diagnosis), again.
for one thing, the statement;
‘’There was no evidence of an environmental source of infection,’’
HOW hard did they look (cjd questionnaire, and questions pertaining to this questionnaire, if there was one at all), and how far back in this adolescents case history did they go back?
I think this statement says it all ;
‘’In March of 2011 after further deterioration, the NPDPSC declined free-of-charge PRNP sequencing stating that the clinical picture was inconsistent with prion disease.’’
nuff said. ...it’s what they do$$$
i am reminded of a few things deep throat told me 15 years or so ago, and it holds true today ;
*** 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 at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work 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 agreat 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 UPDATE...TSS
Sporadic Fatal Insomnia in an Adolescent
http://sporadicffi.blogspot.com/2014/02/sporadic-fatal-insomnia-in-adolescent.html
a response to this case from the Doctors that treated ;
Comment on Sporadic Fatal Insomnia in an Adolescent
a.. Michael Tennison, Professor of Neurology and Pediatrics
b.. Heather Baudet M.D. Ph.D., Joseph Muenzer M.D. Ph.D.
UNC Chapel Hill
We read with interest the report by Blas? et al. as we were the physicians who actually cared for the child. We learned that this manuscript was in press upon contacting the National Prion Disease Pathology Surveillance Center (NPDPSC) to include them in our own manuscript. We were disappointed and surprised that the CDC "case
More...
We read with interest the report by Blas? et al. as we were the physicians who actually cared for the child. We learned that this manuscript was in press upon contacting the National Prion Disease Pathology Surveillance Center (NPDPSC) to include them in our own manuscript. We were disappointed and surprised that the CDC "case investigation" did not include speaking with the physicians who treated the patient. It is difficult to extract nuances of a case from a medical record without firsthand knowledge of the patient. The restrictions of space allow us to correct only some of the errors in the manuscript. The presenting symptom was diplopia, which in retrospect is characteristic of sporadic fatal insomnia (sFI). Subsequently, the most prominent symptoms were non-specific incoordination and cognitive changes with very prominent pseudobulbar affect and anxiety. There was a stepwise deterioration after two head injuries followed by strabismus surgery. A major abrupt decline then occurred with a H1N1 illness. Hyperkinetic is imprecise and ballismus is an incorrect characterization of the movements which were appendicular and gait cerebellar ataxia. The authors inaccurately state that no evaluation of insomnia or dysautonomia was performed. In fact, the family reported that the patient had difficulty sleeping only toward the end of his life despite direct questioning at each visit and declined a sleep study when offered. The child did develop incontinence as an autonomic issue but had no cardiovascular or vasomotor changes. We believe no reasonable clinician would stop pursuing alternative diagnoses based solely on polysomnogram or PET results. Head injury was never "diagnostically deceptive" to the neurology team. Could head injury, like surgery and severe influenza, be a trigger for a step-wise deterioration in sFI? A mitochondrial disorder was initially considered as the patient had a de novo POLG1 gene variant (p.V742M), but an outside mitochondrial expert agreed with the pursuit of alternate explanations, including prion-related disorders. The authors fail to note that two of the treating physicians independently raised the possibility of a prion disease. Therefore, the statement that a prion diagnosis was not pursued is false. In March of 2011 after further deterioration, the NPDPSC declined free-of-charge PRNP sequencing stating that the clinical picture was inconsistent with prion disease. We then pursued in-house analysis of the PRNP gene, specifically looking for the permissive methionine at codon 129 as well as the D178N mutation known to cause fatal familial insomnia. Brain biopsy would have potentially been diagnostic and was considered. We felt that the likelihood of a treatable diagnosis being discovered on brain biopsy was small and elected to defer to autopsy. Without aggressive pursuit of a prion diagnosis, amongst others, and counseling about the need for autopsy, a diagnosis would have never been reached. Rather than procuring our notes via the state health department and using our observations and assessments without citation, we believe including the treating physicians who actually saw the patient in the investigation and manuscript would have avoided inaccuracies and inferior science.
Conflict of Interest:
None declared
Published April 28, 2014
http://pediatrics.aappublications.org/content/133/3/e766.abstract/reply#pediatrics_el_59985
Seems (to me at least), Belay et al are always trying to sway the verdict (or diagnosis), again.
for one thing, the statement;
‘’There was no evidence of an environmental source of infection,’’
HOW hard did they look (cjd questionnaire, and questions pertaining to this questionnaire, if there was one at all), and how far back in this adolescents case history did they go back?
I think this statement says it all ;
‘’In March of 2011 after further deterioration, the NPDPSC declined free-of-charge PRNP sequencing stating that the clinical picture was inconsistent with prion disease.’’
nuff said. ...it’s what they do$$$
i am reminded of a few things deep throat told me 15 years or so ago, and it holds true today ;
*** 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 at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work 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 agreat 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 UPDATE...TSS
I have often wondered if sporadic FFI, considering there is no link to any
family gene, if sporadic FFI could be a by-product of iatrogenic FFI, i.e.
friendly fire?
ALSO, SINCE the Alabama mad cow atypical BSE was Associated with Prion
Protein Gene Mutation (g-h-BSEalabama), and since we do know that these FFI is
transmissible, why then is it so far fetched to then believe that the FEED FROM
AN ATYPICAL BSE CASE WITH A PRION PROTEIN GENE MUTATION COULD NOT THEN BE PASSED
VIA FEED AND IN FOOD TO HUMANS, AS VPSPr PRIONPATHY, AND OR SPORADIC FFI OR
SPORADIC GSS ???
see ;
> Here we report the successful transmission of the disease to
experimental animals, placing FFI within the group of infectious cerebral
amyloidoses...
Letters to Nature Nature 376, 434 - 435 (03 August 2002);
doi:10.1038/376434a0
First experimental transmission of fatal familial insomnia
Jun Tateishi*, Paul Brown‡, Tetsuyuki Kitamoto*, Zahirul M. Hoque*, Raymond
Roos§, Robert Wollman∥, Larisa Cervenàkovà ‡ & D. Carleton Gajdusek‡
* Department of Neuropathology, Neurological Institute, Kyushu University,
Fukuoka 812, Japan ‡ Laboratory of CMS Studies, National Institute of
Neurological Disorders and Stroke, National Institutes of Health, Bethesda,
Mayland 20892, USA Departments of Neurology § and Pathology∥, University of
Chicago Medical Center, Chicago, Illinois 60637, USA
ORIGINALLY described by Lugaresi et al. in 1986 (ref. 1), fatal familial
insomnia (FFI) is a rare inherited neurological disease characterized by the
subacute progression of intractable insomnia and other autonomic abnormalities,
cerebellar and pyramidal signs, myoclonus and dementia; neuropathologically, the
major feature is severe neuronal loss with associated gliosis in the ventral and
mediodorsal thalamic nuclei. The disease has been related to the group of
spongiform encephalopathies by virtue of the presence of low levels of
proteinase-resistant amyloid protein (PrPres) in the brain2á¤-4, and of a
pathogenic single-allele mutation at codon 178 of the PRNP gene that encodes
PrF68 (refs 2, 5). Here we report the successful transmission of the disease to
experimental animals, placing FFI within the group of infectious cerebral
amyloidoses.
------------------
References
The Lancet, Volume 346, Issue 8974, Pages 569 - 570, 26 August 1995
doi:10.1016/S0140-6736(95)91405-6Cite or Link Using DOI Transmission of
fatal familial insomnia to laboratory animals Original TextJohn Collinge a,
MarkS. Palmer , KatieC.L. Sidle , Ian Gowland , Rosella Medori , James Ironside
, Peter Lantos
Expedited Publication
A subtype of sporadic prion disease mimicking fatal familial insomnia
P. Parchi, MD, S. Capellari, MD, S. Chin, MD, PhD, H.B. Schwarz, MD, N.P.
Schecter, MD, J.D. Butts, MD, P. Hudkins, MD, D.K. Burns MD, J.M. Powers, MD and
P. Gambetti, MD + Show Affiliations Address correspondence and reprint requests
to Dr. Pierluigi Gambetti, Division of Neuropathology, Institute of Pathology,
Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106.
doi: 10.1212/WNL.52.9.1757 Neurology June 1, 1999 vol. 52 no. 9 1757
Abstract Full Text Full Text (PDF) Also available: Figures Only PPT Slides
of All Figures
Abstract
Objective: To establish a variant of sporadic prion disease as the sporadic
form of fatal familial insomnia (FFI).
Background: FFI is a recently described prion disease characterized
clinically by severe sleep impairment, dysautonomia, and motor signs, and
pathologically by atrophy of thalamic nuclei, especially the medial dorsal and
anterior ventral, and of the inferior olive. FFI is linked to the D178N mutation
coupled with the methionine codon at position 129 in the prion protein gene
(PRNP). It is also identified by the properties of the abnormal prion protein
(PrPSc), which has the relative molecular mass of 19 kDa, corresponding to the
so-called type 2, and a marked underrepresentation of the unglycosylated form
relative to the diglycosylated and monoglycosylated forms.
Methods: Clinical, pathologic, PrPSc, and PRNP data from 5 subjects with a
sporadic prion disease phenotypically similar to FFI were collected and
analyzed.
Results: All 5 subjects had a disease clinically similar and
histopathologically virtually identical to FFI. PrPSc type 2 was present in all
subjects in amount and distribution similar to those of FFI. However, the PrPSc
did not show the striking underrepresentation of the unglycosylated isoform of
the protein that is characteristic of FFI. Moreover, none of the subjects had
the D178N PRNP mutation but all were homozygous for methionine at codon 129.
Conclusion: This condition is likely to represent the sporadic form of FFI
and the term “sporadic fatal insomnia” is proposed. Received February 23, 1999.
Accepted April 3, 1999.
Sunday, March 31, 2013
*** Creutzfeldt Jakob Disease CJD worlds youngest documented victim, 11
years old, shall we pray
HOW TO TURN A POTENTIAL MAD COW VICTIM IN THE USA, INTO A HAPPENSTANCE OF
BAD LUCK, A SPONTANEOUS MUTATION FROM NOTHING.
OR WAS IT $$$
Case report Sporadic fatal insomnia in a young woman: A diagnostic
challenge: Case Report
Karen M Moody , Lawrence B Schonberger , Ryan A Maddox , Wen-Quan Zou ,
Laura Cracco and Ignazio Cali
BMC Neurology 2011, 11:136doi:10.1186/1471-2377-11-136
Published:
31 October 2011
Abstract (provisional)
Background
Sporadic fatal insomnia (sFI) and fatal familial insomnia (FFI) are rare
human prion diseases.
Case presentation
We report a case of a 33-year-old female who died of a prion disease for
whom the diagnosis of sFI or FFI was not considered clinically. Following death
of this patient, an interview with a close family member indicated the patient's
illness included a major change in her sleep pattern, corroborating the reported
autopsy diagnosis of sFI. Genetic tests identified no prion protein (PrP) gene
mutation, but neuropathological examination and molecular study showed
protease-resistant PrP (PrPres) in several brain regions and severe atrophy of
the anterior-ventral and medial-dorsal thalamic nuclei similar to that described
in FFI.
Conclusions
In patients with suspected prion disease, a characteristic change in sleep
pattern can be an important clinical clue for identifying sFI or FFI;
polysomnography (PSG), genetic analysis, and nuclear imaging may aid in
diagnosis.
snip...
Case presentation
Clinical findings In February 2007, the Centers for Disease Control and
Prevention (CDC) and the National Prion Disease Pathology Surveillance Center
(NPDPSC) notified the Texas Department of State Health Services (DSHS) of a
32-year-old woman with an 18-month history of progressive neurological symptoms
suggestive of CJD. (Table 1) Based on the medical record and her neurologist,
her illness began in August 2005 with attention deficits and progressive memory
loss. In June 2006, she demonstrated anisocoria and bizarre behavior, including
talking incoherently to herself, and she was then referred to psychiatry. On a
mini-mental state examination, she scored abnormally low in the measure of
attention and calculation and she had reduced ability to repeat the names of
three unrelated objects [14]. Later in 2006 she was described as being in
constant motion, having unfocused hand gestures, and continued difficulty with
ambulation. She was reported as alert, but confused, sad, and having difficulty
with her thought process. Physicians caring for the case patient discussed the
possibility of several diagnoses such as viral encephalopathy, paranoid
schizophrenia, and subacute sclerosing panencephalitis, yet the overall etiology
remained unclear. By February 2007, the patient was unable to ambulate and
became bed-bound. She continued to demonstrate bizarre behavior, inability to
follow commands, and unintelligible speech. The patient expired in June 2007, 22
months after the onset of illness.
Over the course of her illness, she had EEGs, magnetic resonance imaging
(MRI) studies, and cerebrospinal fluid (CSF) tests. The EEG study performed in
July 2006 showed generalized slowing with bilateral periodic lateralized
epileptiform discharges. A second EEG performed two to three weeks later was
unsuccessful due to excessive movements of the patient. In April 2006, an MRI
study was negative for intracranial abnormalities. Another MRI study was
completed in February 2007 and it showed supratentorial parenchymal atrophy with
no other acute intracranial findings. CSF studies performed in March 2007 were
normal, including the amount of the 14-3-3 protein determined.
Because of the age of the patient and the potential for variant or
iatrogenic CJD, in July 2007 an investigator from the DSHS (KMM) interviewed a
family member to obtain additional information about the patient's travel
history, past medical history, and the symptoms of the present illness. The
patient had a history of travel outside the continental United States to Puerto
Rico during 1995-96 where she had lived approximately one year. Her surgical
history included two back surgeries for internal disc disruption and
degenerative disc disease. An anterior lumbar discectomy with interbody fusion
at L4-5 was performed in November 2000 utilizing cadaver donated bone and in
August 2001 another fusion was performed at L5-S1 utilizing autologous bone. The
donor of cadaver bone was pre-screened minimizing the possibility of iatrogenic
transmission. There was no familial history of progressive neurological disease
or dementia-like illness. The family member also confirmed the clinical history
including the onset in August 2005 of progressive memory loss and, in February
2006, bizarre behavior that included the patient's sitting in a chair for hours
making noises that progressively got louder.
Following preliminary autopsy results, the NPDPSC requested the DSHS
re-interview the family to ask specifically about the patient's pattern of
sleep. When questioned about insomnia, the family member recalled that the
patient had experienced disturbed sleep at the time of her disease onset. The
family member also reported that the patient's sleep pattern progressively
deteriorated throughout her illness. Some nights, for example, the patient did
not sleep. On other nights when she did appear to be sleeping, her sleep was
intermittent. During nights that the patient did not sleep, she would roam the
house at all hours, unable to calm down. By August of 2006, four hours was the
maximum amount of sleep the patient would get in one stretch and at times she
would go two to three days without sleep. Medications were prescribed to help
her sleep but they were not beneficial.
Genetic analysis Sequencing of the PrP gene open reading frame revealed
methionine homozygosity at codon 129, with no pathogenic mutation.
snip...
see full text ;
===================
Clinical findings In February 2007, the Centers for Disease Control and
Prevention (CDC) and the National Prion Disease Pathology Surveillance Center
(NPDPSC) notified the Texas Department of State Health Services (DSHS) of a
32-year-old woman with an 18-month history of progressive neurological symptoms
suggestive of CJD. (Table 1) Based on the medical record and her neurologist,
her illness began in August 2005 with attention deficits and progressive memory
loss. In June 2006, she demonstrated anisocoria and bizarre behavior, including
talking incoherently to herself, and she was then referred to psychiatry.
=====================
AND THAT MY FRIENDS, IS HOW YOU EXPLAIN SOMETHING AWAY INTO NOTHING. IT'S
THE USDA ET AL MAD COW WAY $$$
how many times have we seen this happen? time and time again.
sporadic FFI or nvCJD Texas style ???
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
nvCJD Clinical course
The clinical course of disease in the ten patients was distinct from that
usually seen in sporadic CJD (table 2). Nine had behavioural changes as an early
clinical feature and were referred to a psychiatrist. In four patients, an early
symptom was dysaesthesiae and in another, pain in the feet persisted throughout
the illness. Nine patients developed ataxia early in the course of the disease.
While all patients developed progressive dementia, in only two was memory
impairment part of initial clinical presentation. Seven of the patients
developed myoclonus, often late in the course of the disease, and three had
choreoathetosis. None of the cases had the electroencephalographic (EEG)
features usually associated with CJD.
LET'S take a closer look at this new prionpathy or prionopathy, and then
let's look at the g-h-BSEalabama mad cow.
This new prionopathy in humans? the genetic makeup is IDENTICAL to the
g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like
this, ......wait, it get's better. this new prionpathy is killing young and old
humans, with LONG DURATION from onset of symptoms to death, and the symptoms are
very similar to nvCJD victims, OH, and the plaques are very similar in some
cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets
even better, the new human prionpathy that they claim is a genetic TSE, has no
relation to any gene mutation in that family. daaa, ya think it could be related
to that mad cow with the same genetic make-up ??? there were literally tons and
tons of banned mad cow protein in Alabama in commerce, and none of it
transmitted to cows, and the cows to humans there from ??? r i g h t $$$
ALABAMA MAD COW g-h-BSEalabama
In this study, we identified a novel mutation in the bovine prion protein
gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United
States of America. This mutation is identical to the E200K pathogenic mutation
found in humans with a genetic form of CJD. This finding represents the first
report of a confirmed case of BSE with a potential pathogenic mutation within
the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most
likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K
mutation.
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and
VPSPr PRIONPATHY
(see mad cow feed in COMMERCE IN ALABAMA...TSS)
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS
Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic
in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the
UK epidemic had most likely originated from such a mutation and argued against
the scrapierelated assumption. Such rare potential pathogenic PRNP mutations
could occur in countries at present considered to be free of BSE, such as
Australia and New Zealand. So it is important to maintain strict surveillance
for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many
countries still feed ruminant proteins to pigs). Removal of specified risk
material, such as brain and spinal cord, from cattle at slaughter prevents
infected material from entering the human food chain. Routine genetic screening
of cattle for PRNP mutations, which is now available, could provide additional
data on the risk to the public. Because the point mutation identified in the
Alabama animals is identical to that responsible for the commonest type of
familial (genetic) CJD in humans, it is possible that the resulting infective
prion protein might cross the bovine-human species barrier more easily. Patients
with vCJD continue to be identified. The fact that this is happening less often
should not lead to relaxation of the controls necessary to prevent future
outbreaks.
Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary
Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: maf12@cam.ac.uk Jürgen
A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier
Hall, Manhattan, Kansas 66506-5601, USA
NATURE|Vol 457|26 February 2009
P.9.21
Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim
McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre,
Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of
Calgary, Canada
Background: Three BSE types (classical and two atypical) have been
identified on the basis of molecular characteristics of the misfolded protein
associated with the disease. To date, each of these three types have been
detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16
Canadian BSE cases based on the biochemical properties of there associated
PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and
relative proteinase K sensitivity of the PrPres from each of the 16 confirmed
Canadian BSE cases was determined using modified Western blot analysis.
Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type
and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and
changes in glycosylation similar to other atypical BSE cases. PK digestion under
mild and stringent conditions revealed a reduced protease resistance of the
atypical cases compared to the C-type cases. N terminal- specific antibodies
bound to PrPres from H type but not from C or L type. The C-terminal-specific
antibodies resulted in a shift in the glycoform profile and detected a fourth
band in the Canadian H-type BSE.
Discussion: The C, L and H type BSE cases in Canada exhibit molecular
characteristics similar to those described for classical and atypical BSE cases
from Europe and Japan. This supports the theory that the importation of BSE
contaminated feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these
countries.
what about that ALABAMA MAD COW, AND MAD COW FEED THERE FROM IN THAT STATE
???
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and
VPSPr PRIONPATHY
*** (see mad cow feed in COMMERCE IN ALABAMA...TSS)
BANNED MAD COW FEED IN COMMERCE IN ALABAMA
Date: September 6, 2006 at 7:58 am PST PRODUCT
a) EVSRC Custom dairy feed, Recall # V-130-6;
b) Performance Chick Starter, Recall # V-131-6;
c) Performance Quail Grower, Recall # V-132-6;
d) Performance Pheasant Finisher, Recall # V-133-6.
CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R
Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter
dated July 19, 2006. Firm initiated recall is complete.
REASON
Dairy and poultry feeds were possibly contaminated with ruminant based
protein.
VOLUME OF PRODUCT IN COMMERCE 477.72 tons
DISTRIBUTION AL
______________________________
PRODUCT Bulk custom dairy pre-mixes,
Recall # V-120-6 CODE None RECALLING FIRM/MANUFACTURER Ware Milling Inc.,
Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete.
REASON Possible contamination of dairy animal feeds with ruminant derived meat
and bone meal.
VOLUME OF PRODUCT IN COMMERCE 350 tons
DISTRIBUTION AL and MS
______________________________
PRODUCT
a) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet, 50 lb.
bags, Recall # V-121-6;
b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet, 50 lb. bags,
Recall # V-122-6;
c) Tucker Milling, LLC #31232 Game Bird Grower, 50 lb. bags, Recall #
V-123-6;
d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50
lb bags, Recall # V-124-6;
e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall #
V-125-6;
f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall #
V-126-6;
g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall #
V-127-6
CODE All products manufactured from 02/01/2005 until 06/20/2006 RECALLING
FIRM/MANUFACTURER Recalling Firm: Tucker Milling LLC, Guntersville, AL, by
telephone and visit on June 20, 2006, and by letter on June 23, 2006.
Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall
is ongoing.
REASON Poultry and fish feeds which were possibly contaminated with
ruminant based protein were not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE 7,541-50 lb bags
DISTRIBUTION AL, GA, MS, and TN
END OF ENFORCEMENT REPORT FOR AUGUST 9, 2006
###
Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125
TONS Products manufactured from 02/01/2005 until 06/06/2006
Date: August 6, 2006 at 6:16 pm PST PRODUCT
a) CO-OP 32% Sinking Catfish, Recall # V-100-6;
b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall #
V-101-6;
c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6;
d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;
e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50
lb. bag, Recall # V-105-6;
g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%,
Recall # V-106-6;
h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to
20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall #
V-107-6;
i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall #
108-6;
j) CO-OP LAYING CRUMBLES, Recall # V-109-6;
k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall #
V-110-6;
l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6;
m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE
Product manufactured from 02/01/2005 until 06/06/2006
RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL,
by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is
complete.
REASON Animal and fish feeds which were possibly contaminated with ruminant
based protein not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE 125 tons
DISTRIBUTION AL and FL
END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006
###
MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006
09:22 71.248.128.67
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II
______________________________
PRODUCT
a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals,
Recall # V-079-6;
b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg),
Recall # V-080-6;
c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED,
Recall # V-081-6;
d) Feather Meal, Recall # V-082-6 CODE
a) Bulk
b) None
c) Bulk
d) Bulk
RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL,
by telephone on June 15, 2006 and by press release on June 16, 2006. Firm
initiated recall is ongoing.
REASON
Possible contamination of animal feeds with ruminent derived meat and bone
meal.
VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons
DISTRIBUTION Nationwide
END OF ENFORCEMENT REPORT FOR July 12, 2006
###
2007
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN
COMMERCE USA 2007
Date: March 21, 2007 at 2:27 pm PST
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II
PRODUCT
Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried,
Recall # V-024-2007
CODE
Cattle feed delivered between 01/12/2007 and 01/26/2007
RECALLING FIRM/MANUFACTURER
Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.
Firm initiated recall is ongoing.
REASON
Blood meal used to make cattle feed was recalled because it was cross-
contaminated with prohibited bovine meat and bone meal that had been
manufactured on common equipment and labeling did not bear cautionary BSE
statement.
VOLUME OF PRODUCT IN COMMERCE
42,090 lbs.
DISTRIBUTION
WI
___________________________________
PRODUCT
Custom dairy premix products:
MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX
Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL
PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST
PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN
Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J -
PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN,
BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT
Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007
CODE
The firm does not utilize a code - only shipping documentation with
commodity and weights identified.
RECALLING FIRM/MANUFACTURER
Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm
initiated recall is complete.
REASON
Products manufactured from bulk feed containing blood meal that was cross
contaminated with prohibited meat and bone meal and the labeling did not bear
cautionary BSE statement.
VOLUME OF PRODUCT IN COMMERCE
9,997,976 lbs.
DISTRIBUTION
ID and NV
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
Saturday, August 4, 2012
Final Feed Investigation Summary - California BSE Case - July 2012
What irks many scientists is the USDA’s April 25 statement that the rare
disease is “not generally associated with an animal consuming infected feed.”
The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown,
one of the world’s experts on this type of disease who retired recently from the
National Institutes of Health.
"(The agency) has no foundation on which to base that statement.”
“We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an
official with the USDA during the Clinton Administration now at Mississippi
State.
In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the
origins of atypical cases of BSE,” she said
Saturday, May 26, 2012
Are USDA assurances on mad cow case 'gross oversimplification'?
Sunday, December 15, 2013
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
Thursday, June 23, 2011
Experimental H-type bovine spongiform encephalopathy characterized by
plaques and glial- and stellate-type prion protein deposits
Thursday, July 21, 2011
A Second Case of Gerstmann-Sträussler-Scheinker Disease Linked to the G131V
Mutation in the Prion Protein Gene in a Dutch Patient Journal of Neuropathology
& Experimental Neurology:
August 2011 - Volume 70 - Issue 8 - pp 698-702
PLEASE NOTE *
Over the next 8-10 weeks, approximately 40% of all the adult mink on the
farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (> 95 %) downer or
dead dairy cattle...
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus
Macaque
"BSE-L in North America may have existed for decades"
Sunday, June 26, 2011
Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque
Monday, May 11, 2009
Rare BSE mutation raises concerns over risks to public health
Wednesday, July 28, 2010
Atypical prion proteins and IBNC in cattle DEFRA project code SE1796 FOIA
Final report
IBNC
"All of the 15 cattle tested showed that the brains had abnormally
accumulated prion protein."
Saturday, February 28, 2009
NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15
cattle tested showed that the brains had abnormally accumulated PrP" 2009
SEAC 102/2
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
Friday, May 13,
2011 EFSA Joint Scientific Opinion on any possible epidemiological or
molecular association between TSEs in animals and humans
Sunday, May 01, 2011
STUDY OF ATYPICAL BSE 2010 Annual Report May 2011
Tuesday, November 08, 2011
Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob
Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4,
2011
Original Paper
Conclusions:These findings raise doubt about the possibility of a reliable
CJD surveillance only based on mortality data.
Friday, November 04, 2011
Diagnostic accuracy of cerebrospinal fluid protein markers for sporadic
Creutzfeldt-Jakob disease in Canada: a 6-year prospective study Research
article
Monday, September 26, 2011
Variably Protease-Sensitive Prionopathy, Prionpathy, Prionopathy, FFI, GSS,
gCJD, hvCJD, sCJD, TSE, PRION, update 2011
Monday, June 27, 2011
Comparison of Sheep Nor98 with Human Variably Protease-Sensitive
Prionopathy and Gerstmann-Sträussler-Scheinker Disease
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease
update July 10, 2008 Friday, June 20, 2008
Sunday, August 10, 2008
A New Prionopathy OR more of the same old BSe and sporadic CJD
Sunday, August 24, 2008
Sporadic Fatal Insomnia with Unusual Biochemical and Neuropathological
Findings
Here we go folks. AS predicted. THIS JUST OUT ! as i predicted, more BSe.
...
Tuesday, August 03, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the
prion protein
Monday, August 9, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the
prion protein or just more PRIONBALONEY ?
snip...see full text ;
O.K. let's compare some recent cases of this prionpathy in other countries
besides Gambetti's first 10 recently, that he claims is a spontaneous event,
from a genetic disorder, that is not genetic, but sporadic, that is related to
no animal TSE in North America, or the world. ...
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary...
2009
Tuesday, August 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009
2011
EFSA Journal 2011 The European Response to BSE: A Success Story
This is an interesting editorial about the Mad Cow Disease debacle, and
it's ramifications that will continue to play out for decades to come ;
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
snip...
EFSA and the European Centre for Disease Prevention and Control (ECDC)
recently delivered a scientific opinion on any possible epidemiological or
molecular association between TSEs in animals and humans (EFSA Panel on
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical
BSE prions as the only TSE agents demonstrated to be zoonotic so far but the
possibility that a small proportion of human cases so far classified as
"sporadic" CJD are of zoonotic origin could not be excluded. Moreover,
transmission experiments to non-human primates suggest that some TSE agents in
addition to Classical BSE prions in cattle (namely L-type Atypical BSE,
Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic
wasting disease (CWD) agents) might have zoonotic potential.
snip...
see follow-up here about North America BSE Mad Cow TSE prion risk factors,
and the ever emerging strains of Transmissible Spongiform Encephalopathy in many
species here in the USA, including humans ;
Monday, September 26, 2011
L-BSE BASE prion and atypical sporadic CJD
Thursday, August 4, 2011
Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis,
Date aired: 27 Jun 2011
Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215
cases in 2009, the highest recorded year to date. sporadic CJD is on a steady
rise, and has been since 1996.
I also urge you to again notice these disturbing factors in lines 5 and 6
;
5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive
cases;
6 Includes 23 (22 from 2010) cases with type determination pending in which
the diagnosis of vCJD has been excluded.
========end=====tss=====2011
PPS POLITICAL PRION SCIENCE $$$
Creutzfeldt-Jakob Disease Surveillance in Texas
Sunday, July 11, 2010
CJD or prion disease 2 CASES McLennan County Texas population 230,213 both
cases in their 40s
see the continuing rise of sporadic CJD in Texas here ;
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE
RISE IN NORTH AMERICA
Sunday, August 21, 2011
The British disease, or a disease gone global, The TSE Prion Disease
(see video here)
U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?
(see video at bottom)
Sunday, September 6, 2009
MAD COW USA 1997
(SEE SECRET VIDEO)
Tuesday, November 01, 2011
Could we face the return of CJD? Experts fear it may lie dormant in
thousands
MAD COW DISEASE, TEXAS STYLE
Case report Sporadic fatal insomnia in a young woman: A diagnostic
challenge: Case Report TEXAS
HOW TO TURN A POTENTIAL MAD COW VICTIM IN THE USA, INTO A HAPPENSTANCE OF
BAD LUCK, A SPONTANEOUS MUTATION FROM NOTHING.
OR WAS IT $$$
2/12/2014
*********
Response from Linda Detwiler, Assistant Director of the Center for Public
and Corporate Veterinary Medicine (CPCVM) at the Virginia-Maryland Regional
College of Veterinary Medicine, University of Maryland:
I respectfully disagree with your assessment that the current BSE SRM
requirements are not necessary. Unlike your cute hound analogy where you have
definitive proof of zero risk, one cannot say the same about BSE. I could
provide > 5 pages of scientific justification for these requirements but I am
limited by the word count.
So in a nutshell:
• We do not truly know or understand the “real” risk to the public in
regards to vCJD as caused by classical BSE and any risk that may be caused by
atypical BSE.
• Per results of the UK’s appendix survey, it appears that humans may be
susceptible to infection with the BSE agent at doses below what was previously
considered. Currently the central prevalence estimate of vCJD in the UK is very
close to 1 in 2,000 in the age cohort tested.
• The UK has substantial evidence of “subclinical” vCJD and it is not known
whether these people will ever develop the disease and/or if they are a risk to
the blood or organ supply.
• We have less of an understanding of atypical BSE and its potential risk
to humans. All 3 native born cases in the US were atypical. The US surveillance
system is not intended to detect and eliminate such cases from the food supply,
hence after each case was reported, the government reassured the public that the
SRM bans were protective.
• In March 2013, APHIS will begin allowing the importation of certain live
bovines and bovine products from other countries. The risk to the US public is
not limited to the US system alone.
SNIP...SEE FULL TEXT AND MORE HERE ;
Thursday, February 20, 2014
Unnecessary precautions BSE MAD COW DISEASE Dr. William James FSIS VS Dr.
Linda Detwiler 2014
Monday, February 3, 2014
*** Evaluation of the zoonotic potential of transmissible mink
encephalopathy TSE Prion disease
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Sunday, February 16, 2014
Recycling collection to benefit young mother with rare, terminal
disease
Buy This Photo Photo 1 of 1 | Zoom Photo + Top Photo Sandi Kennedy, shown
here with her oldest son, is now home with family and the community is rallying
around her. Courtesy photo By Staff reports February 14, 2014 3:21 PM KENNEBUNK
- The CAN DO program at the Kennebunk Transfer Station will spend the next two
months collecting cans and bottles to help the family of Sandi Kennedy, a young
Kennebunk mother struck by a rare, fatal disease.
"Drop off bottles and cans with a redemption value (Maine State deposit
only) at the Transfer Station on Sea Road," said CAN DO organizer Tom Couming.
"This will continue for at least two months."
Doctors have diagnosed Kennedy, a 38-year-old wife and mom of four young
children ages 2 to 9, with Creutzfeldt-Jakob Disease, an incurable neurological
disorder, part of a family known as prion diseases.
Kennedy is now at her home in Kennebunk, surrounded by family, and will be
on hospice care as doctors have given her a short time to live.
Friends and family members have created a page on YouCaring.com to support
the Kennedy family. Visit youcaring.com and search for "Hope for Sandi." One
hundred percent of any money raised through the page will benefit the
Kennedys.
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
Friday, January 10, 2014
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type
prion disease, what it ???
Monday, February 03, 2014
CREUTZFELDT-JAKOB DISEASE T.S.E. PRION U.K. UPDATE As at 3rd February 2014
WHAT about the sporadic CJD TSE proteins ?
WE now know that some cases of sporadic CJD are linked to atypical BSE and
atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all
it’s sub-types $$$
Creutzfeldt-Jakob Disease CJD cases rising North America updated report
August 2013
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada
seeing an extreme increase of 48% between 2008 and 2010 ***
Sunday, October 13, 2013
*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
Sunday, January 19, 2014
National Prion Disease Pathology Surveillance Center Cases Examined1 as of
January 8, 2014
Wednesday, December 11, 2013
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic
Creutzfeldt-Jakob Disease ***
Friday, February 14, 2014
Creutzfeldt-Jakob disease (CJD) biannual update (February 2014), with
briefing on novel human prion disease National CJD Research and Surveillance
Unit NCJDRSU
TSS
