Brazil Creutzfeldt Jakob Disease CJD 547 confirmed cases in 16 years

Brazil Creutzfeldt Jakob Disease CJD 547 confirmed cases in 16 years

Lito: Understand what Creutzfeldt-Jakob disease is; Brazil had 547 confirmed cases in 16 years.

There are four known forms of CJD: sporadic, hereditary, iatrogenic, and the new variant (vCJD). According to a survey by the Ministry of Health, Brazil registered 547 confirmed cases of the disease between 2005 and 2021 — a period that corresponds to the first 17 years since CJD became part of the list of mandatory notifications. By Poliana Casemiro 21/08/2026 08:54 Updated13 hours ago

Lito Souza is diagnosed with a neurodegenerative disease, says his wife. The wife of influencer Lito Sousa, from the Aviões e Música channel, said in a video that he was diagnosed with Creutzfeldt-Jakob disease . In the video, published this Friday (21), Lito's wife, Mila, commented that the diagnosis took a while to be confirmed, but that in recent weeks the influencer lost part of his motor skills and received the news of the disease. Creutzfeldt-Jakob disease (CJD) is a rare, neurodegenerative, progressive, and incurable human prion disease. The condition typically begins with dementia, memory loss, and tremors, and progresses with other neurological signs affecting different regions of the brain simultaneously. There are four known forms of CJD: sporadic, hereditary, iatrogenic, and the new variant (vCJD).

Pilot Lito Sousa, from the YouTube channel Aviões e Músicas, confirms Creutzfeldt-Jakob disease diagnosis — Photo: Reproduction/Youtube According to a survey by the Ministry of Health, Brazil recorded 547 confirmed cases of the disease between 2005 and 2021 — a period that corresponds to the first 17 years since CJD became part of the list of mandatory notifications.

SEE ALSO Lito Sousa's illness is not the same as 'mad cow disease'; understand the difference.

Suffering from a rare and incurable disease, Lito Sousa explained the 'cycle of life' to his 7-year-old son, says his wife. Lito Sousa is diagnosed with an incurable degenerative disease, says his wife. A rare, fast-moving, and incurable disease. CJD is a prion disease — caused by an abnormal alteration of a protein called a prion, which multiplies in the brain and destroys neurons, leaving a pattern of holes in the brain tissue that gives rise to the term "spongiform." The disease is rare, progressive, and, to date, there is no treatment capable of reversing or halting its progression. According to neurologist José Bauab, the prion settles inside the neuron and progressively intoxicates it. The effect is not limited to the isolated cell: the toxicity also compromises the glia, the tissue responsible for supporting and sustaining neurons. "The protein particles enter the neuron, intoxicate it, and exhibit absolutely destructive and toxic behavior within the cell," describes Bauab.

In Brazil, the disease mainly affects older people: the 55 to 74 age group accounted for 60.2% of reported cases, with an average age of 66 among suspected cases — a different profile from Lito's case, since the sporadic form usually affects people between 60 and 80 years old. The literature used by the Ministry of Health indicates that approximately 90% of patients die between six months and one year after the onset of symptoms, with an average survival of five months—a fact that reinforces the severity of the condition reported by Lito's family. Among the notifications analyzed, there were 290 records of death due to the illness, although the report points out flaws in the completion and monitoring of the data.

What do the national data show?

The Ministry of Health study analyzed 1,576 notifications of suspected cases registered in the Notifiable Diseases Information System (SINAN). Among these cases, 547 were confirmed. The highest concentration of cases occurred in the South, Southeast, and Northeast regions. São Paulo leads in the number of confirmed cases (202), followed by Minas Gerais (57) and Paraná (44). According to the epidemiological bulletin, the cases are divided into:

São Paulo: 202 cases

Minas Gerais: 57 cases

Paraná: 44 cases

Rio de Janeiro: 38 cases

Rio Grande do Sul: 35 cases

Has the disease increased in the country?

The number of reported cases has grown throughout the historical series, with a more significant increase starting in 2012 — which, according to the ministry, may reflect greater sensitivity in epidemiological surveillance, and not necessarily more actual cases. The peak was in 2019, with 174 records (11% of the total), while 2020 and 2021, during the Covid-19 pandemic, saw a decrease in reported cases. DCJ is not the same thing as "mad cow disease". Sporadic CJD — the likely form investigated in the most recent suspected cases in Rio de Janeiro — should not be confused with variant CJD (vCJD), linked to the consumption of beef contaminated with bovine spongiform encephalopathy (BSE), also known as "mad cow disease." The Ministry of Health states that there have never been any recorded cases of vCJD in Brazil since 2005, nor any deaths attributed to the variant in the country.

Mad cow disease — Photo: Art Department/G1 Diagnosis and management

There is no treatment that reverses CJD; care focuses on managing symptoms, supporting the patient, and controlling complications—the same palliative treatment mentioned in Lito's case. Diagnosis combines clinical evaluation with imaging tests, such as magnetic resonance imaging (MRI), electroencephalography (EEG), and computed tomography (CT) scans, in addition to the RT-QuIC test, performed on cerebrospinal fluid, which has high specificity for confirming suspected cases.

https://g1.globo.com/saude/noticia/2026/08/21/entenda-o-que-e-a-doenca-creutzfeldt-jakob-doenca-de-piloto-lito-que-causa-demencia-e-perda-dos-movimentos.ghtml

“DCJ is not the same thing as "mad cow disease". Sporadic CJD — the likely form investigated in the most recent suspected cases in Rio de Janeiro — should not be confused with variant CJD (vCJD), linked to the consumption of beef contaminated with bovine spongiform encephalopathy (BSE), also known as "mad cow disease." The Ministry of Health states that there have never been any recorded cases of vCJD in Brazil since 2005, nor any deaths attributed to the variant in the country.”

***> SEE BRAZIL BSE, SCRAPIE, CJD, HISTORY FILES AT THE BOTTOM…TSS

*** Grant Agreement number: 222887 ***

*** Project acronym: PRIORITY ***

*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***

Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014 Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es Project website¡ Error! Marcador no definido. address: www.prionpriority.eu

PRIORITY, PROJECT FINAL REPORT

*** 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species ***

snip...

http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf

see full text;

Block D: Prion epidemiology Studies on atypical scrapie were identified as a key element of this block, given the potential risk associated to this agent. We studied the permeability of Human, bovine and porcine species barriers to atypical scrapie agent transmission. Experiments in transgenic mice expressing bovine, porcine or human PrPC suggest that this TSE agent has the intrinsic ability to propagate across these species barriers including the Human one. Upon species barrier passage the biological properties and phenotype of atypical scrapie seem to be altered. Further experiments are currently ongoing (in the framework of this project but also in other projects) in order to: 

(i) characterize the properties of the prion that emerged from the propagation of atypical scrapie in tg Hu; 

(ii) to confirm that the phenomena we observed are also true for atypical scrapie isolates other than the ones we have studied. In parallel, studies in sheep have concluded that:

*** Atypical scrapie can be transmitted by both oral and intracerebral route in sheep with various PRP genotypes

*** Low but consistent amount of infectivity accumulates in peripheral tissue (mammary gland, lymph nodes, placenta, skeletal muscles, nerves) of sheep incubating atypical scrapie.

*** The combination of data from all our studies leads us to conclude that:

*** Atypical scrapie passage through species barriers can lead to the emergence of various prions including classical BSE (following propagation in porcine PRP transgenic mice).

*** Atypical scrapie can propagate, with a low efficacy, in human PrP expressing mice. This suggests the existence of a zoonotic potential for this TSE agent.

snip...

We advance our main conclusions and recommendations, in particular as they might affect public policy, including a detailed elaboration of the evidence that led to them. Our main recommendations are:

a. The issue of re-introducing ruminant protein into the food-chain The opinion of the members of Priority is that sustaining an absolute feed ban for ruminant protein to ruminants is the essential requirement, especially since the impact of non-classical forms of scrapie in sheep and goats is not fully understood and cannot be fully estimated.

Therefore, the consortium strongly recommends prohibiting re-introduction of processed ruminant protein into the food-chain.

Arguments in support of this opinion are:

• the large (and still uncharacterized) diversity of prion agents that circulate in animal populations;

• the uncertainties related to prion epidemiology in animal populations;

• the unknown efficacy of industrial processes applied to reduce microbiological risk during processed animal protein (PAP) production on most prion agents;

• the intrinsic capacity of prions to cross interspecies transmission barriers;

• the lack of sensitive methodology for identifying cross contamination in food.

• the evolution of natural food chains in nature (i.e. who eats whom or what) has generated an efficient barrier preventing, to some extent, novel prion epidemies and that this naturally evolved ecology should be respected.

The consortium is also hesitant to introduce processed ruminant proteins into fish food considering the paucity of data on prion infections in fishes and sea animals including those of mammalian origin, and the risk of establishing an environmental contamination of the oceans that cannot be controlled.

b. Atypical prion agents and surveillance Atypical prion agents (see below) will probably continue to represent the dominant form of prion diseases in the near future, particularly in Europe.

*** Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models.

*** Similarly, there are now some data that seem to indicate that the atypical scrapie agent can cross various species barriers.

*** Moreover, the current EU policy for eradicating scrapie (genetic selection in affected flocks) is ineffective for preventing atypical scrapie.

*** The recent identification of cell-to-cell propagation and the protein-encoded strain properties of human neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, suggest that they bear the potential to be transmissible even if not with the same efficiency as CJD. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of their transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. In that context it would appear valuable

• to develop knowledge related to the pathogenesis and inter-individual transmission of atypical prion agents in ruminants (both intra- and inter-species)

• to improve the sensitivity of detection assays that are applied in the field towards this type of agent

• to maintain a robust surveillance of both animal and human populations

c. The need for extended research on prions

Intensified searching for a molecular determinants of the species barrier is recommended, since this barrier is a key for many important policy areas - risk assessment, proportional policies, the need for screening of human products and food. In this respect, prion strain structural language also remains an important issue for public health for the foreseeable future. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research. Prions maintain a complex two-way relationship with the host cell and fundamental research is needed on mechanisms for their transmission, replication and cause of nervous system dysfunction and death.

Early detection of prion infection, ideally at preclinical stage, also remains crucial for development of effective treatment strategies in humans affected by the disease.

Position of the Priority consortium

Nearly 30 years ago, the appearance in the UK of Bovine Spongiform Encephalopathy (BSE) quickly brought the previously obscure “prion diseases” to the spotlight. The ensuing health and food crises that spread throughout Europe had devastating consequences. In the UK alone, there were more than 36,000 farms directly affected by BSE and the transmission of BSE prions to humans via the food chain has caused over 200 people in Europe to die from variant Creutzfeldt-Jakob disease (vCJD) (http://www.cjd.ed.ac.uk

Origins of prion epidemies

Classical BSE now appears to be under control, with 18 EU Member States having achieved the World Organisation for Animal Health (Office International Epizooties) „negligible risk‟ status (May 2014; http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/list-of-bse-risk-status/), and the remaining MS assessed as „controlled‟ risk. Of note, research, including EU-funded research, has played a key role in this success: while the origin of the infection was never defined, the principle driver of the epidemic was identified as prions in Meat and Bone Meal (MBM). Tests based on prion protein-specific antibodies were developed, allowing detection of infected animals, and a better understanding of disease pathogenesis and the distribution of infectivity in edible tissues; experimental investigation of transmission barriers between different species allowed a rational estimation of risks, etc. All of this led to the implementation of rational and effective policies, such as the MBM ban to protect the animal feed chain, and the Specified Risk Material (SRM) regulations to protect the human food chain.

In spite of this progress, prions are still a threat.

Epidemiological re-assessment indicates that the ∼10 year incubation period separating the peaks of the BSE and the vCJD epidemics is probably too short. In addition, results from a large number of human tonsil and appendix analyses in the UK suggest that there may be a high number of asymptomatic individuals who are positive for the disease-associated conformer prion protein PrPSc. While vCJD is the only form of human prion disease that has been consistently demonstrated to have lymphoreticular involvement, there has been no systematic investigation of lymphoid tissue in cases with other prion diseases.

The human prion problem

The clinical cases of vCJD identified to date have all shared a common PrP genotype (M129M), although one pre-clinical case was confirmed as an M129V heterozygote, and it has been mooted that perhaps only the M129M proportion of the population is susceptible. However, in the UK appendix study, PrP accumulation was described in samples representing every codon 129 genotype, raising the possibility that genotype does not confer resistance but instead modulates incubation period. Apart from the two UK studies, the lymphoid tissues of non-CJD patients have not been examined for the presence of PrPSc, so, these cases may not solely represent pre-clinical vCJD, but also other forms of prion disease.

Recent experiments in highly susceptible mouse models indicate the presence of infectivity in blood or blood components at late disease stages in sporadic CJD. The significance of this experimental finding for humans has to be explored in more detail and, at the present time, there is no evidence for the transmission of prions via blood in sporadic CJD. However a likely scenario is that all those with signs of infection or abnormal PrP accumulation in peripheral tissue could have infective blood, posing the risk for transmission via blood products, which has been clearly demonstrated in experimental models, and confirmed in several cases of vCJD in man. Altogether, these data clearly demonstrate the potential risk of a second wave of vCJD, particularly when the number people identified with lymphoid accumulation of PrPSc (16/32,411) gives a prevalence estimate in the UK of 493 per million, much higher than the number of clinical cases seen to date.

The animal prion problem

An increasing number of reports on cases of “atypical” BSE in cattle throughout the EU and beyond may lead to a new epidemic, particularly since we still do not understand all factors determining the species barrier. Ovine scrapie is another concern, because it could mask ovine BSE, presumably transmissible to humans. Scrapie is endemic and not likely to be eradicated soon, although current control measures are effective at greatly reducing disease incidence. Atypical forms, which may be spontaneous, are not affected by these control measures and these forms of disease will persist in the global animal population. The low prevalence of these disease forms makes effective surveillance very challenging. However, there is a clear risk attendant on ignoring these cases without an understanding of their possible zoonotic potential, particularly when most forms of human disease have no established aetiology. In summary, atypical cases of BSE and scrapie presently clearly outnumber classical cases in cattle and sheep in all member states.

We will highlight the state-of-the-art knowledge and point out scientific challenges and the major questions for research. Strategic objectives and priorities in Europe in the future for research that aims to control, eliminate or eradicate the threat posed by prions to our food and health are also indicated.

The Priority project has focused on 4 themes, namely the structure, function, conversion and toxicity of prions; detection of prions; mechanisms of prion transmission and spreading and epidemiology of prion diseases. This paper summarizes the opinions/positions reached within these themes at the end of the project.

http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf

see;

https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html

https://transmissiblespongiformencephalopathy.blogspot.com/2026/04/us-report-scrapie-cwd-cattle-sheep-pigs.html

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

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

No 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.

https://efsa.onlinelibrary.wiley.com/doi/epdf/10.2903/sp.efsa.2020.EN-1946

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.

http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160

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

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/

PRION 2016 TOKYO

Saturday, April 23, 2016

SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016 Prion. 10:S15-S21. 2016 ISSN: 1933-68961933-690X WS-01:

Prion diseases in animals and zoonotic potential

***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.

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

Title: 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.

http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160

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

Ru G1 ., Pocchiari M2 ., Bertolini S. 1, Pite L.1 , Puopolo M.2 , Ladogana A.2 , Perrotta M.G.3 , Meloni D

1 . (1) National reference center for the study and research on animal encephalopathies and comparative neuropathologies (CEA). Experimental Zooprophylactic Institute of Piemonte, Liguria and Valle d'Aosta, Torino, Italy. (2) Department of Cellular Biology and Neuroscience, Istituto Superiore di Sanità, Roma, Italy. (3) Office 3 National center for the fight and emergency against animal diseases. Ministry of Health, Roma, Italy.

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.

Materials and Methods: National data from prion disease surveillance in humans (sporadic CJD) and small ruminants (CS) in Italy were utilized. A descriptive geographic analysis was conducted for each disease individually. Subsequently, an ecological study was performed to compare the occurrence of both diseases at the district and regional levels. Standardized incidence ratios (SIR), adjusted for confounders, were calculated for CJD and CS by district and region, respectively, representing the outcome and proxy of exposure. Considering a possible long incubation period of CJD, two study periods were analysed: 2010-2014 for CJD and 2002-2006 for CS. Eight alternative linear regression models were developed using SIR in humans as the dependent variable and SIR in sheep as the independent variable. These models varied in the scale of SIR data (continuous vs. categorical), geographical level (district vs. region), and the potential past exposure of sheep in specific areas to a known source of infection (via a contaminated vaccine).

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.

Funded by: Italian Ministry of Health Grant number: Realizzazione del programma epidemiologico finalizzato a dare evidenza del potenziale zoonotico delle TSE animali diverse dalla BSE. Prot. N. 0018730-17/07/2015-DGSAFCOD_UO-P ''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.'' Meeting-book-final-version prion 2023 Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.

https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf

Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene

Waqas Tahir , Sandor Dudas , Renee Anderson , Jianmin Yang , Sarah Bogart , Kristina Santiago-Mateo, Yuanmu Fang & Roberta Quaghebeur Pages 36-49 | Received 20 Feb 2025, Accepted 22 May 2025, Published online: 04 Aug 2025 Cite this article https://doi.org/10.1080/19336896.2025.2511933

ABSTRACT

Bovine Spongiform Encephalopathy (BSE) is a fatal neurodegenerative disease in cattle which can be either classical BSE (C-BSE) or atypical BSE (including H-BSE and L-BSE). Here, we report the results of our analyses of an H-BSE case found in Canada in 2021, indicating restriction of the pathological agent (PrPSc) mainly to the central nervous system with no or occasional weak involvement of peripheral tissues. Importantly, a non-synonymous mutation at codon 211 of the PRNP gene was detected and confirmed to be present as a germline mutation. This is the first case of BSE in Canada with a predisposing E211K mutation. Snip… Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions. Supplemental material Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene KEYWORDS: Atypical BSE Bovine Spongiform Encephalopathycentral nervous systemE211K mutationprion diseasesprion protein genesynonymous mutation

https://www.tandfonline.com/doi/full/10.1080/19336896.2025.2511933#d1e1606

Thursday, August 20, 2026

United Kingdom - Bovine spongiform encephalopathy - Immediate notification GENERAL INFORMATION COUNTRY/TERRITORY OR ZONE

https://woahoie.blogspot.com/2026/08/united-kingdom-bovine-spongiform.html

https://prpsc.proboards.com/thread/231/united-kingdom-spongiform-encephalopathy-confirmed

FRIDAY, APRIL 17, 2026

Ireland Central Veterinary Research Laboratory confirmed a case of atypical BSE on April 9, 2026

https://bse-atypical.blogspot.com/2026/04/ireland-central-veterinary-research.html

Research and analysis Annual report to Parliament on the monitoring programme for transmissible spongiform encephalopathies (TSEs) – 2024 and 2025 Published 2 June 2026

https://www.gov.uk/government/publications/monitoring-programme-for-tses-annual-report-2024-and-2025/annual-report-to-parliament-on-the-monitoring-programme-for-transmissible-spongiform-encephalopathies-tses-2024-and-2025

https://efsaopinionbseanimalprotein.blogspot.com/2026/06/research-and-analysis-annual-report-to.html

USA Report, Scrapie, CWD, BSE, TSE, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026 April 2026

https://fdabse589.blogspot.com/2026/04/usa-report-scrapie-cwd-bse-tse-cattle.html

https://www.researchgate.net/publication/403956772_USA_Report_Scrapie_CWD_BSE_TSE_Cattle_Sheep_Pigs_Cervid_Humans_Zoonotic_2026

Mad cow disease: Could it be here? 2001 revisited 2026

https://prpsc.proboards.com/thread/230/mad-disease-2001-revisited-2026

https://bovineprp.blogspot.com/2026/08/mad-cow-disease-could-it-be-here-2001.html

“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”

see my full report here; Saturday, April 25, 2026

***> USDA Statement BSE Surveillance Information Center Update 2026 <***

https://oieusdabseprp.blogspot.com/2026/04/usda-statement-bse-surveillance.html

United States of America - Scrapie - Immediate notification WAHIS

https://wahis.woah.org/#/in-review/7742

USDA APHIS statement

https://www.aphis.usda.gov/news/program-update/usda-confirms-detection-classical-scrapie-slaughtered-sheep

Oregon statement

https://apps.oregon.gov/oregon-newsroom/OR/ODA/Posts/Post/Classical-Scrapie-Confirmed-in-Oregon-Sheep-Through-Routine-Surveillance

Scrapie Update

https://scrapie-usa.blogspot.com/2026/08/usda-confirms-detection-of-classical.html

TUESDAY, SEPTEMBER 07, 2021

Atypical Bovine Spongiform Encephalopathy BSE OIE, FDA 589.2001 FEED REGULATIONS, and Ingestion Therefrom

https://bse-atypical.blogspot.com/2021/09/atypical-bovine-spongiform.html

US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025

From the first full year of reporting CJD TSE in the US in 2000, where 90 cases of CJD was reported that year, to today, where in September 2025, the number of CJD cases reported in the last full year reporting, which would have been 2024, the number of CJD cases for 2024 was 249 cases. So, from the first full year 2000 CJD cases were 90 cases confirmed in that year, to 2024, where 2024 CJD statistics rose to 249 confirmed CJD cases in a single year. A dramatic increase in deaths, from figures that don’t seem to be dramatic. But thes figures today, they are not from “better surveillance”, that dog don’t hunt no more. They have been saying this for over 25 years, year after year, well it’s time to call it for what it is, Human Transmissible Spongiform Encephalopathy TSE Prion cases are rising, and it’s NOT because of better surveillance, or just a “happenstance of bad luck, that 85%+ of all human cases, sporadic CJD, including VPSPr, just happen spontaneously, no, it’s because of unknown environmental factors, and or iatrogenic factors, imho…terry

US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025

https://prionunitusaupdate.blogspot.com/2025/10/us-national-prion-disease-pathology.html

Brazil TSE Prion

THURSDAY, NOVEMBER 11, 2021

Brazil investigating two possible cases of mad cow disease in humans

https://creutzfeldt-jakob-disease.blogspot.com/2021/11/brazil-investigating-two-possible-cases.html

SATURDAY, NOVEMBER 13, 2021

Brazil Creutzfeldt Jakob Disease CJD TSE Prion Update 2021

https://creutzfeldt-jakob-disease.blogspot.com/2021/11/brazil-creutzfeldt-jakob-disease-cjd.html

SATURDAY, SEPTEMBER 4, 2021

Brazil Confirms TWO More Cases of Mad Cow Disease BSE States of Mato Grosso and Minas Gerais

https://animalhealthreportpriontse.blogspot.com/2021/09/brazil-confirms-two-more-cases-of-mad.html

SATURDAY, JUNE 01, 2019

Brazil reports another cases of mad cow disease atypical BSE TSE Prion

https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html

TUESDAY, MARCH 26, 2019

Joint Statement from President Donald J. Trump USA and President Jair Bolsonaro Brazil FOREIGN POLICY BSE TSE Prion aka mad cow disease

https://bseusa.blogspot.com/2019/03/joint-statement-from-president-donald-j.html

FRIDAY, NOVEMBER 03, 2017

First case of V180I rare mutation in a Brazilian patient with Creutzfeldt-Jakob disease

http://creutzfeldt-jakob-disease.blogspot.com/2017/11/first-case-of-v180i-rare-mutation-in.html

TUESDAY, SEPTEMBER 27, 2016

Classical Scrapie Diagnosis in ARR/ARR Sheep in Brazil Acta Scientiae Veterinariae, 2015. 43(Suppl 1): 69.

http://scrapie-usa.blogspot.com/2016/09/classical-scrapie-diagnosis-in-arrarr.html

MONDAY, AUGUST 1, 2016

USDA Announces Reopening of Brazilian Market to U.S. Beef Exports and the Potential for Transmissible Spongiform Encephalopathy TSE prion disease

http://madcowusda.blogspot.com/2016/08/usda-announces-reopening-of-brazilian.html

MONDAY, MAY 5, 2014

Brazil BSE Mad Cow disease confirmed OIE 02/05/2014

http://bovineprp.blogspot.com/2014/05/brazil-bse-mad-cow-disease-confirmed.html

Monday, May 5, 2014

Brazil 2nd BSE Mad Cow disease confirmed OIE 02/05/2014

http://bovineprp.blogspot.com/2014/05/brazil-bse-mad-cow-disease-confirmed.html

Thursday, April 24, 2014

Brazil investigates possible BSE mad cow case

http://bovineprp.blogspot.com/2014/04/brazil-investigates-possible-bse-mad.html

WEDNESDAY, JANUARY 29, 2014

Another Suspect case of Creutzfeldt-Jakob disease investigated in Brazil

http://creutzfeldt-jakob-disease.blogspot.com/2014/01/another-suspect-case-of-creutzfeldt.html

THURSDAY, SEPTEMBER 26, 2013

Brazil evaluate the implementation of health rules on animal by-products and derived products SRM BSE TSE PRION aka MAD COW DISEASE

http://bse-atypical.blogspot.com/2013/09/brazil-evaluate-implementation-of.html

Wednesday, December 19, 2012

Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil

http://bse-atypical.blogspot.com/2012/12/scientific-report-of-european-food.html

Friday, December 07, 2012

ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012

http://bse-atypical.blogspot.com/2012/12/atypical-bse-brazil-2010-finally.html

Terry S. Singeltary Sr.