Sunday, November 10, 2024

Texas Creutzfeldt Jakob Disease CJD TSE Prion Surveillance Data Update November 2024

Texas Creutzfeldt Jakob Disease CJD TSE Prion Surveillance Data Update November 2024

Texas Human Prion Disease

Texas Recent Trends in Human TSE Prion Disease

Prion disease surveillance monitors the occurrence of prion diseases in the United States, but it also monitors for the emergence of vCJD and other potentially preventable new prion diseases, as well as for rare classic forms of prion diseases that are attributable to medical procedures. Prion disease surveillance also helps assess the efficacy of ongoing U.S. prevention measures.

CJD has been a NOTIFIABLE CONDITION in Texas since 1998, and it was likely under-reported and misdiagnosed for many years. For 15 years now, Texas has carried out enhanced surveillance (passive and active surveillance) for CJD, including sporadic, genetic/familial, and acquired (iatrogenic and variant) CJD. The success of this program is demonstrated by the identification and confirmation of sporadic (sCJD), genetic/familial (gCJD or fCJD) and variant CJD (vCJD), as well as other cases of human prion diseases such as Variably Protease Sensitive Prionopathy (VPSPr), Fatal Familial Insomnia (FFI), sporadic Fatal Insomnia (sFI), and Gerstmann-Sträussler-Scheinker (GSS) syndrome.

From 2013-2022 Texas reported 337 confirmed, probable and possible cases,

of which there were 308 sCJD,

20 fCJD,

1 vCJD,

1 FFI,

1 sFI,

1 GSS and

5 VPSPr cases.

Texas also investigates higher priority suspect cases, such as suspect cases in persons <55 years old, as vCJD is rarely found in individuals >55 years old, reported suspected clusters, suspected iatrogenic cases, and suspected cases and cases with risk factors that could expose them to other prion diseases, such as chronic wasting disease of deer, elk, moose, and other cervids.

In 2014, Texas had the 4th US variant CJD case; the person was likely exposed to the infectious agent before moving to the United States. A full description of the case can be found at: https://stacks.cdc.gov/view/cdc/30921 or https://www.cdc.gov/eid/article/21/5/pdfs/14-2017.pdf

Maheshwari A, Fischer M, Gambetti P, Parker A, Ram A, Soto C, Concha-Marambio L, Cohen Y, Belay ED, Maddox RA, Mead S, Goodman C, Kass JS, Schonberger LB,Hussein HM. Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications. Emerg Infect Dis. 2015 May;21(5):750-9. doi:10.3201/eid2105.142017. PubMed PMID: 25897712; PubMed Central PMCID: PMC4412247.

The Centers for Disease Control and Prevention (CDC) reports a worldwide (including the United States) CJD incidence rate of approximately 1-2 cases per million population per year, and from 2016-2020 the average annual rate in the United States was approximately 5 cases per million population per year in persons 55 years of age or older.

In Texas, the average rate of deaths per million population due to CJD over the past 10 years (2013-2022) is 1.2 cases per million population per year. 

The average rate over two consecutive 5-year periods, 2013-2017 & 2018-2022, are 0.86 & 1.46 (cases per million population per year), respectively. 

There has been an increase in CJD cases over the last five years, and this can be attributed to several factors. 

The population of Texas is increasing but also aging, the availability of the RT-QuIC test (a new more sensitive ante-mortem CSF test) in 2015, as well as increased awareness of this testing, ability of physicians to order this CSF testing through commercial laboratories that forward specimens to the National Prion Disease Pathology Surveillance Center (NPDPSC), and confirmatory testing on brain tissue availability at NPDPSC, have all increased the surveillance for human prion diseases in Texas (increased testing, reporting, and antemortem diagnosis of CJD). 

The intensity of surveillance methods can also influence the reported incidence of CJD, and other prion diseases, and Texas conducts enhanced human prion disease surveillance. 

CDC also updated their criteria for classifying a CJD case, and this was implemented in Texas in 2019. 

The combination of a positive RT-QuIC CSF result with neuropsychiatric symptoms allows for decreased reliance on the presence of specific neurologic symptoms to classify a case of CJD. 

This has permitted the classification of CJD cases that previously may not have been counted. 

The change in the criteria for classifying a CJD case is evidence of the incorporation of scientific data into surveillance system approaches to count cases more accurately. 

In 2019, the number of CJD cases and the overall rate of CJD increased, however, in the majority of cases brain tissue was examined and there were no unusual findings. 

Also, only four of the 2019 cases were <55 years of age, all of whom had autopsies, and the brain tissue analysis did not reveal any unusual neuropathology. 

In 2020, the number of CJD cases and the overall rate of CJD decreased from 2019. This may be an influence from the COVID-19 pandemic and decreased seeking of healthcare; however, the rate did not significantly decrease like some other infectious diseases reported to public health. 

The number of cases that had brain tissue examined also decreased in 2020, and there was a higher number of cases in individuals <55 years of age. 

In 2020, nine cases were <55 years of age, of which three cases had brain tissue examined and there were no unusual findings, and six cases did not have brain tissue examined. 

In 2021, the number of CJD cases and overall rate of CJD increased but did not surpass the 2019 case count or rate. 

The percentage of cases that had brain tissue examined increased from 34% in 2020 to 44% in 2021, and none of the cases were <55 years of age. 

There was only one case in 2021 that was <55 years of age, and brain tissue was not examined. 

In 2022, the number of CJD cases and overall rate of CJD increased. 

The percentage of cases that had brain tissue examined decreased from 44% in 2021 to 38% in 2022, and three of the cases were <55 years of age with no unusual findings. 

There were four additional cases in 2022 that were <55 years of age, and brain tissue was not examined. 

Decreased confirmation of CJD via brain tissue analysis over the last several years could be an influence of the COVID-19 pandemic and response, increased trust by physicians in RT-QuIC CSF results and less reliance on autopsy results, decreased consent to autopsy by the patient or family, or other factors. 

Also, fluctuations in the rates of rare diseases, data obtained via CJD surveillance systems, and the demographics of cases is expected from year to year. 

The rate of CJD decreased from 2019 to 2020 and then increased from 2020 to 2021 and again from 2021 to 2022. 

However, small changes in case counts can lead to larger changes in rates, and with increased surveillance in Texas, increased awareness, and ease of ante-mortem testing using a better test, an average rate closer to 1.5 cases per million population per year may be more representative of CJD cases in Texas, which still falls within the worldwide rate of 1-2 cases per million population per year. There have also been efforts to increase autopsy rates, and the rate of cases that have brain tissue analysis will be monitored over time. All other CJD data will continue to be evaluated on an ongoing basis.

Phone

512-776-7676 Fax

512-776-7616 Email

Feedback.IDCU@dshs.texas.gov Mailing Address

Texas Department of State Health Services Emerging and Acute Infectious Disease Unit P.O. Box 149347, Mail Code: 1960 Austin , TX 78714-9347 Physical Address

Emerging and Acute Infectious Disease Unit Moreton Building, Suite M-631, Mail Code: 1960 1100 West 49th Street Austin , TX 78756-3199


Texas Creutzfeldt Jakob Disease CJD Surveillance Data


Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022

TUESDAY, MAY 24, 2022

Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022


Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022

Tue, May 24, 2022 11:06 am

DSHS EAIDU.DataRequests EAIDU.DataRequests@dshs.texas.govHide

To Terry Singeltary flounder9@verizon.net

Cc DSHS EAIDU.DataRequests EAIDU.DataRequests@dshs.texas.gov

Hello Mr. Singletary,

Thank you for your inquiry. The CJD data you requested through 2020 is available below. This data will also be available on the Texas Department of State Health Services CJD data website in the next few days.

Texas Creutzfeldt-Jakob Disease Case Counts by Classification, Transmission Type, Disease Type and Year (2011-2020)

Classification Transmission Type Disease Type 2011 2012 2013 2014 2015 2016 2017 2018 2019 2020 Total Counts

Confirmed Acquired vCJD 1 1

Confirmed Familial fCJD 3 1 2 4 1 11

Confirmed Familial FFI 1 1

Confirmed Sporadic sCJD 12 10 10 11 14 18 15 21 24 13 148

Confirmed Sporadic sFI 1 1 2

Confirmed Sporadic VPSPr 1 1 1 3

Probable Familial fCJD 1 1 1 1 1 5

Probable Sporadic CJD 5 10 4 10 4 14 8 11 17 26 109

Possible Sporadic sCJD 1 1 2

Total Counts 18 22 14 27 20 33 25 36 46 41 282

vCJD: variant Creutzfeldt-Jakob disease; fCJD: Familial Creutzfeldt-Jakob disease; FFI: Fatal Familial Insomnia; sCJD: sporadic Creutzfeldt-Jakob disease; sFI: sporadic Fatal Insomnia; VPSPr: variably protease-sensitive prionopathy


Unfortunately, the 2021 CJD data cannot be released at this time, as 2021 data is provisional and still undergoing the verification process and will then be certified by the state epidemiologist. After this process is complete, the CJD website will be updated with the 2021 data.

Thank you again,

EAIDU Data Request Mailbox

Emerging and Acute Infectious Disease Unit

Texas Department of State Health Services

EAIDU.DataRequests@dshs.texas.gov 

END

From: Terry Singeltary <flounder9@verizon.net>

Sent: Tuesday, May 10, 2022 2:12 PM

To: DSHS EAIDU.DataRequests <EAIDU.DataRequests@dshs.texas.gov>

Cc: DSHS hivstd <hivstd@dshs.texas.gov>

Subject: Creutzfeldt Jakob Disease TSE Prion disease DATA UPDATE REQUEST

Creutzfeldt Jakob Disease TSE Prion disease DATA UPDATE REQUEST I have been trying to get the latest stats on creutzfeldt jakob disease in Texas for over a year, and it's always, something like; 'we hope to update the CJD data base soon'. the last time i requested cjd stats was; MONDAY, JUNE 14, 2021

Texas Health and Human Services The Department of State Health Services Creutzfeldt Jakob Disease TSE Prion Report 2021? still waiting? with great urgency, i request this information, especially since cwd tse prp seems to be heading for a zoonosis zoonotic call soon... Thank You! kind regards, terry 

SUNDAY, MAY 08, 2022 

USA National Prion Disease Pathology Surveillance Center Surveillance Update April 11th, 2022 


TUESDAY, APRIL 05, 2022 

Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014 


TUESDAY, MAY 10, 2022

Concordance of CSF RT-QuIC across the European Creutzfeldt-Jakob Disease surveillance network


TUESDAY, MAY 24, 2022

Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022


MONDAY, JUNE 14, 2021

Texas Health and Human Services The Department of State Health Services Creutzfeldt Jakob Disease TSE Prion Report 2021?

Texas last CJD update was 2018...

Texas Creutzfeldt-Jakob Disease Case Counts by Type of Disease and Year (2009-2018)

Classification Transmission

Type Disease Type

2009 2010 2011 2012 2013 2014 2015 2016 2017 2018

Total Counts Confirmed Acquired vCJD 1 1

Familial fCJD 2 2 3 1 2 10

FFI 2 2

Sporadic sCJD 10 16 12 10 10 11 14 18 15 21 137

sFI 1 1 2

VPSPr 1 1 1 3

Probable Familial fCJD 1 1 1 1 4

Sporadic sCJD 6 8 5 10 4 10 4 14 8 11 80

Possible Sporadic sCJD 2 1 1 4

Total Counts 20 28 18 22 14 27 20 33 25 36 243

vCJD: variant Creutzfeldt-Jakob Disease; fCJD: familial Creutzfeldt-Jakob Disease; FFI: Fatal Familial Insomnia; sCJD: sporadic Creutzfeldt-Jakob Disease; sFI: sporadic Fatal Insomnia; VPSP: variably protease-sensitive prionopathy


SNIP...SEE FULL TEXT;

MONDAY, JUNE 14, 2021 

Texas Health and Human Services The Department of State Health Services Creutzfeldt Jakob Disease TSE Prion Report 2021?


FRIDAY, DECEMBER 24, 2021

Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021


THURSDAY, JULY 13, 2017

TEXAS CREUTZFELDT JAKOB DISEASE CJD TSE PRION


HUMAN MAD COW DISEASE nvCJD TEXAS CASE NOT LINKED TO EUROPEAN TRAVEL CDC

Sunday, November 23, 2014

*** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in June 2014 confirmed as USA case NOT European the patient had resided in Kuwait, Russia and Lebanon. The completed investigation did not support the patient's having had extended travel to European countries, including the United Kingdom, or travel to Saudi Arabia. The specific overseas country where this patient’s infection occurred is less clear largely because the investigation did not definitely link him to a country where other known vCJD cases likely had been infected.



SUNDAY, OCTOBER 13, 2013

Prion Disease Cases in Texas by Year, 2003-2012


SUNDAY, OCTOBER 13, 2013

CJD TSE Prion Disease Cases in Texas by Year, 2003-2012

Prion Disease Cases in Texas by Year, 2003-2012

Last updated September 17, 2013


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.

=====================


Sunday, July 11, 2010

CJD or prion disease 2 CASES McLennan County Texas population 230,213 both cases in their 40s

2 mysterious cases of disease in McLennan County a rarity, but no cause for alarm

By Cindy V. Culp Tribune-Herald staff writer

Friday July 9, 2010

Two likely cases of a mysterious, fatal brain disorder have been reported in McLennan County — a statistical anomaly considering that only one in 1 million people worldwide is affected by the condition in any given year.

Adding to the peculiarity is that the noncontagious disorder belongs to the same family as Creutzfeldt-Jakob disease.

One of its forms is believed to be triggered by people eating meat from cattle infected with mad cow disease.

As frightening as that might sound, officials said residents shouldn’t be alarmed.

One of the local cases definitely is not the type associated with mad cow disease, and there is no evidence the other one is, either. More importantly, the disorder cannot be transmitted from person to person, officials said.

“To have potentially two cases this close together is statistically unusual,” said Dr. Farley Verner, an infectious disease specialist who advises the Waco-McLennan County Public Health District. “But because of the type of disorder it is, and because of what we know about how it develops, it’s not a worrisome coincidence. It’s just a coincidence.”

Because of privacy laws, health officials can release only limited details about the local cases. Both were reported in May.

The first case involved a 49-year-old man from McGregor, Hammad Akram, the health district’s epidemiologist, said. The man has since died.

Initial results from an autopsy show he had some type of human prion disease, a family of diseases involving an abnormal protein.

Creutzfeldt-Jakob disease, or CJD, is the most common type of human prion disease. The autopsy ruled out CJD, however, Akram said.

The second case involves a Waco woman in her late 40s, Akram said. Her symptoms point to CJD, but since the only way to confirm the disease is to study brain tissue after death, that diagnosis is not confirmed, he said.

No apparent link

There is no apparent link between the two local victims, Akram said.

Prion disease usually occurs in people older than age 60.

Doctors give patients a “working diagnosis” of human prion disease based on certain symptoms, combined with results from a blood test, Farley said.

The symptoms are similar to those of other neurological conditions: confusion, difficulty remembering recent events, loss of feeling in certain body parts, balance problems, difficulty walking and muscle jerks and spasms.

If a physician rules out other causes for such symptoms, a blood test can be done that indicates whether the person has a genetic mutation associated with human prion disease. The test cannot confirm it, but positive results make the diagnosis more likely, Verner said.

The name of the disease category comes from a protein called a prion.

People have normal prions, which are concentrated in the brain. But in some instances, there is abnormal prion protein, which causes normal prions to be converted to abnormal form.

That destroys brain tissue and is eventually fatal. The process can take years, but most people die within three months to a year of having symptoms.

There are three main categories of human prion disease — sporadic, familial and acquired.

Sporadic cases start spontaneously, without a clearly identifiable cause. They account for about 85 percent of all human prion disease, according to the National Prion Disease Pathology Surveillance Center.

Familial cases are inherited and are caused by a defect in the prion protein gene, the center said.

Acquired cases are transmitted by infection, which can occur if a person receives a transplant infected with prion disease or undergoes surgery where contaminated instruments are used, according to the center.

Another avenue of infection is when someone eats contaminated beef, the center said. That’s where the connection to mad cow disease comes in.

Only three cases linked to contaminated beef have been found in the United States, according to health officials. In all three cases, the victims are thought to have been infected while living overseas.

In Texas, about 120 people died from human prion disease between 2000-08, according to state data.

Last year, there were 19 probable or confirmed cases of sporadic CJD and two familial CJD cases statewide.

McLennan County has not had any human prion disease cases in the past decade, according to state records. Verner said he can only recall two or three cases in the 25 years he has been here.

cculp@wacotrib.com

757-5744

http://www.wacotrib.com/news/98085839.html


> "It’s just a coincidence.”

http://www.wacotrib.com/news/98085839.html


r i g h t. $$$

cjd = one-in-a-million ???

McLennan County, Texas population 2008 230,213

http://en.wikipedia.org/wiki/McLennan_County,_Texas

CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8

>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<


CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER



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 ;



Monday, September 26, 2011

Variably Protease-Sensitive Prionopathy, Prionpathy, Prionopathy, FFI, GSS, gCJD, hvCJD, sCJD, TSE, PRION, update 2011


Snip…see;

TUESDAY, JUNE 1, 2010

USA cases of dpCJD rising with 24 cases so far in 2010


MONDAY, MARCH 29, 2010

CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

URGENT, PLEASE NOTE ;

>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas.

She left 6 Kids and a Husband. The Purpose of this web is to give information in Spanish to the Hispanic community, and to all the community who want's information about this terrible disease.- Physician Discharge Summary, Parkland Hospital, Dallas Texas Admit 
Date: 12/29/2009 Discharge Date: 1/20/2010 

Attending Provider: Greenberg, Benjamin Morris; General Neurology Team: General Neurology Team Linda was a Hispanic female with no past medical history presents with 14 months of increasing/progressive altered mental status, generalized weakness, inability to walk, loss of appetite, inability to speak, tremor and bowel/bladder incontinence. She was, in her usual state of health up until February, 2009, when her husband notes that she began forgetting things like names and short term memories. He also noticed mild/vague personality changes such as increased aggression. In March, she was involved in a hit and run MVA, although she was not injured. The police tracked her down and ticketed her. At that time, her son deployed to Iraq with the Army and her husband assumed her mentation changes were due to stress over these two events. Also in March, she began to have weakness in her legs, making it difficult to walk. Over the next few months, her mentation and personality changes worsened, getting to a point where she could no longer recognized her children. She was eating less and less. She was losing more weight. In the last 2-3 months, she reached the point where she could not walk without an assist, then 1 month ago, she stopped talking, only making grunting/aggressive sounds when anyone came near her. She also became both bowel and bladder incontinent, having to wear diapers. Her '"tremor'" and body jerks worsened and her hands assumed a sort of permanent grip position, leading her family to put tennis balls in her hands to protect her fingers. The husband says that they have lived in Nebraska for the past 21 years. They had seen a doctor there during the summer time who prescribed her Seroquel and Lexapro, Thinking these were sx of a mood disorder. However, the medications did not help and she continued to deteriorate clinically. Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. The husband says that he does not know any fellow workers with a similar illness. He also says that she did not have any preceeding illness or travel.

http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8



FRIDAY, OCTOBER 23, 2009

Creutzfeldt-Jakob Disease Surveillance Texas Data for Reporting Years 2000-2008


SUNDAY, DECEMBER 16, 2007

Creutzfeldt-Jakob Disease Surveillance in Texas 2000-2006


Creutzfeldt-Jakob Disease in Northeast Texas,

J.A. Rawlings,*1 K.A. Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of Health, 1Austin and 2Tyler, Texas

Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform encephalopathy, is caused by prions composed of proteinaceous material devoid of nucleic acid. CJD occurs sporadically (generally 1 case/1,000,000 population per year) in older patients (average age of 65) and is characterized by rapidly progressive dementia, accompanied by severe muscle spasms and incoordination. Death usually occurs within 3 to 12 months (average 7 months). CJD activity in Texas, which has a population of nearly 19 million, appeared to be typical. The statewide death rate for 1995 and 1996 was just under 1/1,000,000. In April of 1997, the Texas Department of Health became aware of an increased number of possible CJD cases in a 23-county area of NE Texas with a population of just over one million. After review of medical and pathology records, four patients were identified with definite classic CJD and three were identified with probable CJD. Dates of death for the eight patients were from April, 1996 through mid-July 1997. The patients were from 46 through 65 years of age; four were male and three were female. A case-control study to identify risks for CJD in NE Texas has been initiated.




FRIDAY, MAY 03, 2024 

National Prion Disease Pathology Surveillance Center Cases Examined1 April 8th 2024 


SUNDAY, JULY 07, 2024

Updated global epidemiology atlas of human prion diseases June 2024


TUESDAY, DECEMBER 12, 2023

CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023


SUNDAY, NOVEMBER 26, 2023

The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis


MONDAY, APRIL 24, 2023

2023 CDC REPORTS CJD TSE Prion 5 cases per million in persons 55 years of age or older


MONDAY, DECEMBER 18, 2023 

Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020


SUNDAY, NOVEMBER 26, 2023

The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis



February 14, 2001

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Terry S. Singeltary, Sr

Author Affiliations

JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.



26 MARCH 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

Terry S. Singeltary, retired (medically)

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?


This old study by Gibbs et Al should have been a wake up call back in 1994…terry 

1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.



Sunday, November 10, 2024

Understanding prions and the implications of iatrogenic Alzheimer’s disease


TUESDAY, OCTOBER 01, 2024 

Texas TAHC TPWD Confirm 213 More Cases of CWD TSE PrP 1008 Positive To Date


THURSDAY, OCTOBER 31, 2024

Texas Chronic Wasting Disease Detected in Kerr County Deer Breeding Facility




2023 US Mad Cow Case Confirmed

Wednesday, May 24, 2023 

***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification




SATURDAY, MAY 20, 2023 

***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE



MAY 19, 2023


2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;

***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;

Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023

''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...



1985

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle 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... 




Bovine Spongiform Encephalopathy BSE TSE Prion Origin USA?, what if?

Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research

Title: Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues

Author item CASSMANN, ERIC - Oak Ridge Institute For Science And Education (ORISE) item MOORE, SARA - Oak Ridge Institute For Science And Education (ORISE) item SMITH, JODI - Iowa State University item Greenlee, Justin 

Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/14/2019 Publication Date: 11/29/2019 Citation: Cassmann, E.D., Moore, S.J., Smith, J.D., Greenlee, J.J. 2019. 

Sheep are susceptible to the agent of TME by intracranial inoculation and have evidence of infectivity in lymphoid tissues. 

Frontiers in Veterinary Science. 6:430. https://doi.org/10.3389/fvets.2019.00430. DOI: https://doi.org/10.3389/fvets.2019.00430 

Interpretive Summary: Prion diseases are protein misfolding diseases that are transmissible between animals. The outcome of prion infection is irreversible brain damage and death. Transmission can occur between animals of the same or different species, however, transmission between different species is usually less efficient due to the species barrier, which results from differences in the amino acid sequence of the prion protein between the donor and recipient species. The present work evaluated whether transmissible mink encephalopathy (TME) can infect sheep. Our results demonstrate that sheep are susceptible to the TME agent and that the TME agent has similar properties to the agent of L-type atypical bovine spongiform encephalopathy (L-BSE). This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.

Technical Abstract: Transmissible mink encephalopathy (TME) is a food borne prion disease. Epidemiological and experimental evidence suggests similarities between the agent of TME and L-BSE. This experiment demonstrates the susceptibility of four different genotypes of sheep to the agent of TME by intracranial inoculation. The four genotypes of sheep used in this experiment had polymorphisms corresponding to codons 136 and 171 of the prion gene: VV136QQ171, AV136QQ171, AA136QQ171, and AA136QR171. All intracranially inoculated sheep without comorbidities (15/15) developed clinical scrapie and had detectable PrPSc by immunohistochemistry, western blot, and enzyme immunoassay (EIA). The mean incubation periods in TME infected sheep correlated with their relative genotypic susceptibility. There was peripheral distribution of PrPSc in the trigeminal ganglion and neuromuscular spindles; however, unlike classical scrapie and C-BSE in sheep, ovine TME did not accumulate in the lymphoid tissue. To rule out the presence of infectious, but proteinase K susceptible PrPSc, the lymph nodes of two sheep genotypes, VV136QQ171 and AA136QQ171, were bioassayed in transgenic ovinized mice. None of the mice (0/32) inoculated by the intraperitoneal route had detectable PrPSc by EIA. Interestingly, mice intracranially inoculated with RPLN tissue from a VV136QQ171 sheep were EIA positive (3/17) indicating that sheep inoculated with TME harbor infectivity in their lymph nodes. Western blot analysis demonstrated similarities in the migration patterns between ovine TME and the bovine TME inoculum. Overall, these results demonstrate that sheep are susceptible to the agent of TME, and that the tissue distribution of PrPSc in TME infected sheep is distinct from classical scrapie.




Previous work has shown that the Stetsonville, WI outbreak of TME could have been precipitated by feeding mink a downer cow with atypical BSE; therefore, it very well may have originated from a cow with L-BSE. The agent of TME appears to remain stable, and it has a high transmission efficiency after a sequence of interspecies transmission events. Although C-BSE is the archetypal foodborne TSE, our findings indicate that L-BSE and bTME have greater transmission efficiencies in bovinized mice. Previous work has demonstrated that L-BSE also is more virulent than C-BSE in mice expressing the human prion protein [46, 55]. Although the documented incidence of L-BSE is low, the propensity of L-BSE and the TME agent to cross species barriers support the continued monitoring for atypical BSE.


***>This work supports the ideas that L-BSE is a possible source for TME in mink and that the practice of feeding cattle with neurologic disease to mink should be avoided. This information is important to farmers who raise cattle, sheep, or mink.<***

1985

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle 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... 




Specified Risk Materials DOCKET NUMBER Docket No. FSIS-2022-0027 Singeltary Submission Attachment



Number: 0579-0189APHIS-2021-0004 Singeltary Submission



Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification



APHIS Indemnity Regulations [Docket No. APHIS-2021-0010] RIN 0579-AE65 Singeltary Comment Submission

Comment from Singeltary Sr., Terry

Posted by the Animal and Plant Health Inspection Service on Sep 8, 2022



Texas Mad Cow

In 2005, the second reported case of BSE was identified in a 12-year-old Texas beef cow representing the first native born casein the United States.


NOW before you go off and start repeating BSE TSE Prion science that is almost 50 years old, let's be perfectly clear what science is saying today, and especially what the WAHIS/WOAH/OIE et al are saying about the atypical BSE strains... OIE Conclusions on transmissibility of atypical BSE among cattle

Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.


Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019

34 Scientific Commission/September 2019

3. Atypical BSE

The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.

The Group acknowledged the challenges in demonstrating the zoonotic transmission of atypical strains of BSE in natural exposure scenarios. Overall, the Group was of the opinion that, at this stage, it would be premature to reach a conclusion other than that atypical BSE poses a potential zoonotic risk that may be different between atypical strains.

4. Definitions of meat-and-bone meal (MBM) and greaves

snip...

REFERENCES

SNIP...END SEE FULL TEXT;


''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''

This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.


Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.


Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.


IT is imperative that the USA puts forth immediately a MANDATORY National Animal Identification System and Country Of Origin Labeling System, for the sake of livestock industry and the consumers that consume their products...terry 

We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold longe incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), is the third potentially zoonotic PD (with BSE and L-type BSE), thus questioning the origin of human sporadic cases. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.


2.3.2. New evidence on the zoonotic potential of atypical BSE and atypical scrapie prion strains

Olivier Andreoletti, INRA Research Director, Institut National de la Recherche Agronomique (INRA) – École Nationale Vétérinaire de Toulouse (ENVT), invited speaker, presented the results of two recently published scientific articles of interest, of which he is co-author:

‘Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice’ (MarinMoreno et al., 2020) and ‘The emergence of classical BSE from atypical/Nor98 scrapie’ (Huor et al., 2019).

In the first experimental study, H-type and L-type BSE were inoculated into transgenic mice expressing all three genotypes of the human PRNP at codon 129 and into adapted into ARQ and VRQ transgenic sheep mice. The results showed the alterations of the capacities to cross the human barrier species (mouse model) and emergence of sporadic CJD agents in Hu PrP expressing mice: type 2 sCJD in homozygous TgVal129 VRQ-passaged L-BSE, and type 1 sCJD in homozygous TgVal 129 and TgMet129 VRQ-passaged H-BSE.


This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.


***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***

Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.


O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France

Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases).

Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.

*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,

***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),

***is the third potentially zoonotic PD (with BSE and L-type BSE),

***thus questioning the origin of human sporadic cases.

We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.

===============

***thus questioning the origin of human sporadic cases***

===============

***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.

==============

PRION 2015 CONFERENCE


Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed 

PUBLIC SUBMISSION

Comment from Terry Singeltary Sr.

Posted by the Food and Drug Administration on May 17, 2016 Comment

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission 



Transmission of scrapie prions to primate after an extended silent incubation period

*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.

*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.

*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.


***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.

***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.

***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.


***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***

Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.



O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,

***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),

***is the third potentially zoonotic PD (with BSE and L-type BSE),

***thus questioning the origin of human sporadic cases.

==============

PRION 2015 CONFERENCE


Cwd, cattle, sheep, raccoons, oh my

The chronic wasting disease agent from white-tailed deer is highly infectious to humanized mice after passage through raccoons


Prion Conference 2023

Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure

Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA

Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.

Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).

Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.

Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.

"Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material."

=====end

Strain characterization of chronic wasting disease in bovine-PrP transgenic mice

Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.

"Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study."

=====end


cwd scrapie pigs oral routes

***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <***

>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <***

***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18(44%), and the tonsil in 10/25 (40%).

***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.




Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.


Volume 30, Number 8—August 2024

Research

Scrapie Versus Chronic Wasting Disease in White-Tailed Deer

Zoe J. Lambert1, Jifeng Bian, Eric D. Cassmann, M. Heather West Greenlee, and Justin J. Greenlee

Author affiliations: Oak Ridge Institute for Science and Education, Oak Ridge, Tennessee, USA (Z.J. Lambert); US Department of Agriculture, Ames, Iowa, USA (Z.J. Lambert, J. Bian, E.D. Cassmann, J.J. Greenlee); Iowa State University, Ames (Z.J. Lambert, M.H. West Greenlee) Suggested citation for this article

Abstract

White-tailed deer are susceptible to scrapie (WTD scrapie) after oronasal inoculation with the classical scrapie agent from sheep. Deer affected by WTD scrapie are difficult to differentiate from deer infected with chronic wasting disease (CWD). To assess the transmissibility of the WTD scrapie agent and tissue phenotypes when further passaged in white-tailed deer, we oronasally inoculated wild-type white-tailed deer with WTD scrapie agent. We found that WTD scrapie and CWD agents were generally similar, although some differences were noted. The greatest differences were seen in bioassays of cervidized mice that exhibited significantly longer survival periods when inoculated with WTD scrapie agent than those inoculated with CWD agent. Our findings establish that white-tailed deer are susceptible to WTD scrapie and that the presence of WTD scrapie agent in the lymphoreticular system suggests the handling of suspected cases should be consistent with current CWD guidelines because environmental shedding may occur.

snip…

The potential for zoonoses of cervid-derived PrPSc is still not well understood (6,18,45–47); however, interspecies transmission can increase host range and zoonotic potential (48–50). Therefore, to protect herds and the food supply, suspected cases of WTD scrapie should be handled the same as cases of CWD.


Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014)

Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure.

Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased.

Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.


Thursday, October 24, 2024 

***> Lack of traceability in U.S. beef industry is a disgrace


Terry S. Singeltary Sr.