Ñuble, Chile, region, there are eight to twelve cases per year of Creutzfeldt Jakob Disease CJD TSE Prion
News: Neurologist from the Chillán Hospital and academics from the UCSC lead scientific research on Creutzfeldt-Jakob Disease in Ñuble
October 17, 2025
Thanks to the awarding of the Health Research and Development Fund (FONIS), a group of doctors and scientists, in collaboration with specialists from the Chillán Hospital, are leading an investigation to understand the genetic, clinical and pathological bases of Creutzfeldt-Jakob Disease (CJD), a neurological disease with a high prevalence in the region.
This disease is characterized by progressive brain damage that leads to a rapid loss of mental function and motor skills. It originates from a protein called a prion, which, by misfolding, impairs the function of other proteins of its type.
The disease presents in three forms: genetic, sporadic, and infectious. According to Dr. Eduardo López, a neurologist at the Herminda Martín Clinical Hospital, professor at the Catholic University of the Holy Conception, and member of the project, a significant number of CJD cases in Chile are concentrated in the Ñuble region, with the genetic factor being predominant.
“The diagnoses we have here in the region are not related to any type of contagion, but rather to a genetic activation in a group of patients who have this predisposition,” the specialist commented.
According to the previous data considered by the research, in our region there are eight to twelve cases per year, while the average for the world population is only 1 or 2 cases per million inhabitants per year. In this context, given the high prevalence of CJD patients in Ñuble, it is essential to understand the details of this pathology, which currently has no specific treatment.
Dr. López indicated that the research will seek to confirm and verify which genetic variables cause the disease, thus contributing to a possible treatment or solution in the future. In this regard, he stated, “We must study this disease as if there were potential for improvement, even though we don't currently have one, because we have a strong scientific theory that, if proven, will put us on the path to a solution,” the neurologist argued.
The specialist highlighted the facilities that the Establishment has provided to research, since the support and collaboration allow timely access to patients diagnosed with CJD, which helps to delve even deeper into the genetic traits of the population in the territory.
This scientific project also aims to facilitate the understanding of other diseases caused by abnormal protein folding, such as Alzheimer's and Parkinson's.
Chile seeks preventive law for mad cow disease
DEC. 14, 2000
SANTIAGO, Chile, Dec. 14 -- Although Chilean government officials claim there is no threat of the "mad cow disease" in Chile, national legislators Wednesday introduced stringent new legislation to assure the disease would not take hold in the country.
While the mad cow disease -- Bovine Spongiform Encephalopathy, or BSE -- is unknown in Chile, its human variant, Creutzfeld-Jacob Disease, occurs at a higher rate than comparison with other Latin American nations.
Members of the center-left Party For Democracy in conjunction with Chile's Institute of Nutrition and Food Technology said "radical measures" to assure that the disease remains an uncommon ailment.
Hamburgers with non-traceable meat origin are a potential threat to consumers, they said, as is imported meat. They propose the creation of a scientific committee to review a possible prohibition of imports of animal products from high-risk areas.
Chile's Department of Agriculture and Livestock, however, rejected these proposals on the grounds that Chile already imposes stringent standards on imported meat. A SAG representative said that the probability of events which occurred in Britain happening in Chile was "zero" because the same conditions don't exist. There have been no recorded cases of BSE in Chile.
Despite zero incidences of BSE cases, Chile does, however, have a relatively high incidence of CJD cases.
According to PPD's Guido Girardi, the incidence of CJD in Chile is in the order of 2 in a million, a higher figure than in comparable countries.
Girardi would like authorities to investigate the relationship between CJD infections and Chilean consumption of raw sheep blood (niachi).
The EU rates Chile are at the lowest BSE risk rating (Level 1 of 4). Meat can be eaten with this security in only five other countries in the world: Argentina, Australia, Norway, New Zealand and Paraguay.
The EU's fears, however, may have a significant impact on fish-meal exports, one of Chile's primary export products and the primary material for many animal-feed products.
Fearing that cows fed with fish meal-based products may also result in diseased animals, EU legislators are proposing a total ban on the use of animal and fish-based cattle feed.
I’m thinking iatrogenic TSE Prion?
“in our region there are eight to twelve cases per year, while the average for the world population is only 1 or 2 cases per million inhabitants per year.”
BRAZIL BSE START DATE 2023/01/18
BRAZIL BSE CONFIRMATION DATE 2023/02/22
BRAZIL BSE END DATE 2023/03/03
2001 Singeltary on CJD, Journal of American Medical Association
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.
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
2023
Bovine spongiform encephalopathy in South America: a regional preventive approach
C. van Gelderen '', E.J. Gimeno 2 & A.A. Schudel 13) (1) Inter-American Institute for Agricultural Cooperation (IICA), Bernardo de Irigoyen 88, 5 piso, 1072 Buenos Aires, Argentina (2) Regional Representation for the Americas of the World Organisation for Animal Health (OIE), Cerviño 3101 2, 1425 Buenos Aires, Argentina (3) World Organisation for Animal Health (OIE), 12 rue de Prony, 75017 Paris, France
Summary
Bovine spongiform encephalopathy (BSE) is a neurodegenerative disease of cattle caused by prions that was first described in the United Kingdom (UK) in 1986. The BSE epizootic that commenced in the UK in the 1980s has since spread into other countries in Europe and Asia through exports of contaminated meat-and-bone meal or infected cattle. Over the past few vears, other emerging or reemerging diseases have spread into previously free countries or regions through international trade. This negative effect of globalisation means that to implement successful preventive and strategic programmes to safeguard animal health, such pal praming must asal perrinake a ronial throken abas thinking, successful control of animal diseases In South America, initial preventive actions against BSE were adopted in 1989. Further measures adopted since then and based on new scientific and technical findings, have led to the demonstration that the region is free of BSE. These early preventive actions have reliably protected the region from importing BSE-infected material. An integral part of the project to determine the BSE status of South America was the training of personnel, the incorporation of technology and the provision of updated information through close relationships with international organisations and prominent international researcher workers. Regional activities aimed at harmonising BSE prevention programmes, producing objective and transparent data on the equivalence of regional BSE status and acilitating regional and international trade have recently been launched. Maintaining the BSE-free status of the region must be given high priority by the beef agro-industrial sectors.
Keywords Bovine spongiform encephalopathy - Geographical bovine spongiform encephalopathy risk analysis - Risk assessment - South America.
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Brazil
In 1991, Brazil implemented BSE preventive measures by establishing a ban on the import of cattle from the UK. To date, however, about 6,215 bovines have been imported from countries with occurrences of BSE. The destination of about 115 of these animals is unknown. In 2000, a follow-up programme for imported breeders was implemented to ensure that any potential risk of BSE being introduced was monitored.
Only 346 kg of MBM were imported from the UK and France in the 1990s.
A ban on feeding ruminants with ruminant-derived MBM has been in place in Brazil since 1996. As from 2001, the ban was extended to include a restriction on feeding mammalian MBM to ruminants. Due to the characteristics of the extensive cattle-rearing system, with low yields in the largest livestock-raising areas of the country (north, north-west and south), feed supplements are rarely used and only given to high-yield cattle (dairy cattle).
A BSE surveillance programme based on the existing rabies surveillance programme was initiated in 1992 (16,888 cases were analysed from 1997 to 2002). An active surveillance programme was in place by 1997, in line with OIE requirements. Activities were conducted by professionals trained in BSE awareness with the support of well-equipped laboratories. To date, over 1,700 samples have been tested, using histopathological and immunohistochemical techniques; all results have been negative. In 2001, a second case of scrapie in sheep was diagnosed in Brazil.
Bovine spongiform encephalopathy notification has been mandatory since 1990. In 1995, a training programme for professionals from the food sector was implemented, and an awareness-raising programme for the general public commenced in 1998. An Advisory Scientific Committee was created in 2002.
The GBR for Brazil submitted by the EU in 2000, established that the risk of disease occurrence in the country is negligible. Brazil has been classified by the EU as a Level I country (depending on the certainty of information received from Brazil with regard to the destination of animals imported from countries with occurrences of BSE or high-risk countries).
Chile
Bovine spongiform encephalopathy prevention measures were initiated in 1990 with the implementation of a ban on the import of cattle from the UK. To date, Chile has imported only 65 bovines from a country with occurrences of BSE (Denmark), which, since 1998, have been kept under strict control. During the same period, Chile imported, again from Denmark, 151 tons of MBM for fish-feeding purposes.
In 1991, Chile undertook a risk analysis programme to prove the unlikelihood of BSE being present in the country. In 1996, mandatory notification of BSE was established (including compensation for notification).
A BSE surveillance programme was also initiated in 1990 and a laboratory approved to perform histopathological diagnosis of BSE. A surveillance programme carried out in accordance with OIE requirements has been in place since then and is updated on a yearly basis. To date, all results have been negative for over 1,450 brain samples examined by histopathological techniques. Immunohistochemistry was introduced in 2000.
A ban on feeding ruminants with ruminant-derived MBM was implemented in 2000 and a training and awareness programme was developed. An Advisory Scientific Committee was created and, in 2001, rules regarding MBM manufacture (obligation to heat MBM to 133°C for two hours) were established. Due to the characteristics of the extensive cattle-rearing system with low yields, feed supplements are rarely used and only given to high-yield dairy cattle.
The GBR for Chile, submitted by the EU in 2000, established that the risk of disease occurrence in the country is negligible. Chile has been classified by the EU as a Level I country.
Colombia
Measures to prevent BSE were initiated in 1997, resulting in the implementation, as of 2001, of a ban on the import of cattle from countries with occurrences of BSE. To date, Colombia has imported 226 bovines from Denmark, France and Spain. The place of destination of most of these animals is unknown.
Recently, Colombia commenced a BSE risk analysis programme. Bovine spongiform encephalopathy notification has been mandatory since 2001.
In 1981, scrapie was diagnosed in sheep in Colombia. In 1998, the BSE surveillance programme was launched, taking advantage of measures already in place for the bovine rabies surveillance programme. The programme is currently being expanded to comply with OIE guidelines and recommendations and is supported by a number of laboratories that conduct histopathological analysis. To date, all samples (over 100 brains per year from 1998 to 2002) have tested negative for BSE.
Given the extensive cattle-rearing system in Colombia and the availability of pastures all year round, little or no grain feed supplements are used. A ban on feeding ruminants with mammalian MBM has been in place since 2001. A training and awareness programme commenced in 2002.
The GBR for Colombia, submitted by the EU in 2000, established that, although never detected, the risk of BSE occurrence in Colombia is possible. Colombia has been classified by the EU as a Level II country.
Paraguay
Bovine spongiform encephalopathy prevention measures were first applied in Paraguay in 1996 by the enforcement of a Ministerial Resolution establishing a ban on imports of cattle, cattle-derived products and by-products from the UK and other countries with occurrences of BSE.
No bovines or MBM have been imported from countries with occurrences of BSE or high-risk countries. The extensive tropical livestock-rearing farming systems practised in Paraguay exclude the use of supplements in cattle feeding as this is economically unviable. In 1996, a resolution establishing an additional preventive measure was issued, banning the use of mammalian meals in animal feed.
Bovine spongiform encephalopathy notification also became mandatory in 1996. An active surveillance programme was initiated, based on the existing bovine rabies surveillance programme. The programme has been effective since 1997 and is supported by a laboratory and personnel with training in BSE awareness. To date, no case of BSE has been detected.
Bovine spongiform encephalopathy training and awareness programmes for professionals and field staff have been implemented, along with a public awareness-raising programme.
The GBR for Paraguay, submitted by the EU in 2000, established that the risk of disease occurrence in the country is negligible. Paraguay has been classified by the EU as a Level I country.
Uruguay
Bovine spongiform encephalopathy prevention measures were first applied in Uruguay in 1988 by the enforcement of a resolution banning imports of cattle, cattle-derived products and by-products from the UK and other countries with occurrences of BSE. Only nine bovine breeding animals in Uruguay were imported from the UK, all of which were slaughtered and buried at the farm of destination. No MBM has been imported from countries with occurrences of BSE or high-risk countries.
The extensive-farming systems typical of livestock-rearing in Uruguay exclude the use of supplements in cattle feeding as this is economically unviable. In 1996, a resolution establishing an additional preventive measure was issued, imposing a ban on the use of mammalian meals in animal feed. This measure has been accompanied by strict controls on animal feedstuffs.
Bovine spongiform encephalopathy notification became mandatory in 1994 and a surveillance programme was implemented and strengthened in 1998, with retrospective analysis to 1978. Uruguay has technical personnel trained in Switzerland in BSE diagnosis and a national laboratory with the capacity to conduct BSE diagnosis using histopathological methods. To date, no BSE case has been detected.
In 1994, a training and BSE awareness programme for professionals and field staff was implemented along with a public awareness-raising programme.
The GBR for Uruguay, submitted by the EU in 1999, established that the risk of disease occurrence in the country is negligible. Uruguay has been classified by the EU as a Level I country.
Bolivia, Ecuador, Guyana, Peru and Venezuela Bovine spongiform encephalopathy prevention measures and specific programmes for BSE surveillance are currently being implemented in Bolivia, Ecuador, Guyana, Peru and Venezuela.
Information on imports of risk material or MBM is scarce. The OIE Regional Representation for the Americas recently conducted the First Regional Training Workshop on TSE diagnosis (10), with the collaboration of prominent world scientists and specialised professionals from all countries in South America, along with other countries of the Americas. The main purpose of the workshop was to provide the region with suitable technical means to safeguard the BSE status of those countries using risk analysis technology and surveillance and diagnosis. Updated information on the conclusions of the workshop will be available shortly.
Discussion
Although prevention programmes were implemented in some countries in the region since the 1980s to avoid the introduction of BSE into South America, not all of countries obtained the same BSE status.
For example, the EU has classified Colombia as a Level II country and Brazil as Level 1, based on capacity to trace-back animals imported trom countries with occurrences of BSE or those at high-risk. Other countries, such as Bolivia, Ecuador, Guyana, Peru and Venezuela, have not yet implemented the necessary measures which would determine their BSE status (Table I).
A strategy should be developed, within the framework of a regional project for the southern hemisphere, to harmonise all activities of the different countries, maintaining the quality of national prevention programmes, to achieve equivalent BSE status for all countries in the region. This would benefit not only the beef market, which currently represents 19% of world cattle population, but also the beef by-products market (e.g. the pharmacological industry). Maintaining the BSE-free status of the region must be given high priority by the beef agro-industrial sector. As a result of activities developed by national prevention projects, Argentina and Uruguay have gained world recognition for their BSE status (based on articles on achieving BSE-free status in the OIE International Animal Health Code and the GBR submitted by the EU). This new regional approach should allow other countries to attain the same BSE status.
Differences in BSE risk and status among countries in South America can be seen clearly after consideration of the main items that comprise a risk assessment performed in accordance with the International Animal Health Code, Chapter 2.3.13., and the internal and external risk factors of the GBR assessment of the EU.
The points to be considered are as follows: with regard to external risks, Brazil and Colombia have recently followed other countries in South America by imposing restrictions on imports of animals and animal byproducts from countries with occurrences of BSE or high-risk countries with regard to internal risks, differences exist in the regulations imposing bans on the use of feeds containing MBM of mammalian origin. In some countries (e.g. Chile), the ban prohibits the feeding of ruminant protein to ruminants, while in others (e.g. Argentina, Brazil, Colombia, Paraguay and Uruguay), the feeding of mammalian proteins to ruminants is prohibited. In most countries, the ban was implemented recently. Argentina is the only country that has eliminated specified risk material (SRM) from the food chain (2002).
- bovine spongiform encephalopathy notification is mandatory in six countries and all of these have implemented surveillance and monitoring systems. However, in some countries, namely:
Bolivia, Ecuador, Peru and Venezuela, implementation is only at the initial stage. Although six countries have diagnostic laboratories and trained personnel, only Argentina possesses a laboratory capable of integral TSE diagnosis (histological, biochemical and molecular diagnosis under biosafety conditions for sample handling).
Conclusions
In South America, initial preventive measures against BSE were adopted by Argentina and Uruguay and then followed by other countries in the region (Brazil, Chile and Paraguay). Further measures adopted since then, and based on new scientific and technical findings, have led to the demonstration that the region is free from BSE. The early preventive actions therefore reliably protected the region from importing BSE-infected material.
A key element of the project to determine the BSE status of the region of South America was the training of personnel, the
incorporation of technology and the provision of updated information through close relationships with international organisations (such as the OIE and the WHO) and prominent international research workers. Regional actions aimed at harmonising BSE prevention and surveillance programmes, at producing objective and transparent data on the equivalence of regional BSE status and at facilitating regional and international trade have recently been launched with the support of the Inter-American Institute for Cooperation on Agriculture (ICA), the Pan American Health Organization (PAHO), the WHO and the OIE Regional Representation for the Americas.
Maintaining the BSE-free status of South America must be given high priority by the beef agro-industrial sector. Communication has led to a consensus between the animal production and industrial sectors on the advantageous situation in the region with regard to BSE and the important implications of this position on the trade of beef, beef products and by-products, whether as food commodities or inputs for specialised industries (pharmaceutical, cosmetic, food, etc.).
However, a lack of knowledge about the origin and nature of BSE, as well as the particular physico-chemical characteristics (resistance, structure) and biological characteristics (species barrier, transmissibility, long incubation period, lack of diagnostic methods on live animals) of the agent pose serious scientific questions. Research and developments in BSE diagnosis and control must be closely followed by countries in South America to strengthen, modify or adopt harmonised and suitable preventive measures to maintain the BSE status acquired by the region.
Acknowledgements
The country information was kindly provided by Dr M. Luz Gallego Alarcón and D. Barrera León from Colombia, Drs D. Gaymonat and E. Perfumo from Uruguay and Dr L. Mascitelli from Argentina.
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Brazil MAPA OMSA Confirms BSE TSE Prion atypical H-type
OFFICIAL NOTE - Map confirms that case of Bovine Spongiform Encephalopathy is atypical type H
Ministry is immediately adopting measures so that exports of Brazilian beef are reestablished as soon as possible
Published on 03/02/2023 22:18 Updated on 03/03/2023 00:08
According to the indications of the technical staff of the Ministry of Agriculture and Livestock (MAPA), the analysis of the reference laboratory of the World Organization for Animal Health (OMSA) confirmed, on Thursday night (02), that the isolated case of Spongiform Encephalopathy Bovine (BEE) detected in the municipality of Marabá (PA) is atypical type H.
The Minister of Agriculture and Livestock, Carlos Fávaro, immediately communicated the result of the sample to President Luiz Inácio Lula da Silva and immediately began inserting said information into the system for official communication to the WHOA and the Chinese authorities.
Once the process is completed, a virtual meeting will be scheduled with the Chinese government to deal with the release of beef exports to the country.
"I emphasize that speed, efficiency and the transparency requested by President Lula was fundamental. I thank our team and that of the governor of Pará, Helder Barbalho, who allowed us to act quickly from the identification of the case", commented Fávaro.
As this is an atypical case, that is, it occurred due to natural causes in a single 9-year-old animal and with all health measures promptly adopted, the Ministry of Agriculture and Livestock is immediately adopting measures, in accordance with health protocols , so that Brazilian beef exports are reestablished as soon as possible.
Press Releases press@agro.gov.br
Brazil Confirms BSE Mad Cow Case, Strain not known yet
OFFICIAL NOTE
Mapa adopts measures on BSE case in Brazil
Meat for market consumption is not affected by confirmation
Published on 02/22/2023 8:15 pm Updated on 02/22/2023 9:51 pm
Faced with the confirmation of a case of Bovine Spongiform Encephalopathy ('mad cow disease') in a 9-year-old male animal on a small property in the municipality of Marabá (PA), the Ministry of Agriculture and Livestock (Mapa) has been adopting all the necessary government measures for the Brazilian meat market.
The World Organization for Animal Health (WHOA) was notified and the samples were sent to the institution's reference laboratory in Alberta, Canada, which will be able to confirm whether the case is atypical.
The animal, raised on pasture, without feed, was slaughtered and its carcass incinerated on site. The official Brazilian veterinary service is carrying out the epidemiological investigation which may be continued or terminated depending on the result.
“All measures are being adopted immediately at each stage of the investigation and the matter is being treated with total transparency to guarantee Brazilian and global consumers the recognized quality of our meat”, highlighted Minister Carlos Fávaro.
Following the official health protocol, exports to China will be temporarily suspended from this Thursday (23). However, the dialogue with the authorities is being intensified to demonstrate all the information and the prompt reestablishment of the Brazilian beef trade.
Press Releases
press@agro.gov.br
Brazil Suspected case of bovine spongiform encephalopathy
COMUNICADO
Caso suspeito de Encefalopatia Espongiforme Bovina
para Copiar para área de transferência Publicado em 20/02/2023 15h36
Atualizado em 20/02/2023 15h39 O
Ministério da Agricultura e Pecuária (Mapa) informa que, acerca do caso suspeito de Encefalopatia Espongiforme Bovina (Mal da "vaca louca"), todas as medidas estão sendo adotadas pelos governos.
A suspeita já foi submetida a análise laboratorial para a confirmação ou não e, a partir do resultado, serão aplicadas imediatamente as ações cabíveis.
Informações à Imprensa
imprensa@agro.gov.br
=====
COMMUNICATE
Suspected case of bovine spongiform encephalopathy
to Copy to clipboard Posted on 2/20/2023 3:36 PM
Updated on 02/20/2023 3:39 pm The
Ministry of Agriculture and Livestock (MAPA) informs that, regarding the suspected case of Bovine Spongiform Encephalopathy ("Mad Cow Disease"), all measures are being adopted by governments.
The suspicion has already been submitted to laboratory analysis for confirmation or not and, based on the result, the appropriate actions will be applied immediately.
2023/01/18
EVENT 4918
Brazil - Bovine spongiform encephalopathy
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Causal agent
Prion (awaiting typing)
Genotype/ Serotype/ Subtype
-
Animal type
TERRESTRIAL
Disease category
OIE-listed
Reason for notification
Recurrence of an eradicated disease
Event occurs in
The country/territory
Event start date
2023/01/18
Event confirmation date
2023/02/22
Event end date
-
Date of last occurrence
2021/09/03
HISTORICAL REPORTS
CONTROL MEASURES
QUANTITATIVE DATA
EPIDEMIOLOGICAL COMMENTS
OUTBREAKS
DIAGNOSTIC TESTS Report number Report ID New/Updated outbreaks On-going outbreaks Total outbreaks Report date IN 159469 1outbound 0 1 2023/02/23
WAHIS wahis.woah.org wahis.woah.org
FRIDAY, MARCH 3, 2023
Brazil MAPA OMSA Confirms BSE TSE Prion atypical H-type
https://animalhealthreportpriontse.blogspot.com/2023/03/brazil-mapa-omsa-confirms-bse-tse-prion.html
THURSDAY, FEBRUARY 23, 2023
Brazil Confirms BSE Mad Cow Case, Strain not known yet
https://animalhealthreportpriontse.blogspot.com/2023/02/brazil-confirms-bse-mad-cow-case-strain.html
TUESDAY, FEBRUARY 21, 2023
Brazil Suspected case of Bovine Spongiform Encephalopathy
*** 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; 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 ***
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see;
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.
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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.
see;
''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.
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
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REFERENCES
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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.
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.
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***thus questioning the origin of human sporadic cases***
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***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.
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PRION 2015 CONFERENCE
***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.
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Taylor & Francis
Prion 2016 Animal Prion Disease Workshop Abstracts
WS-01: Prion diseases in animals and zoonotic potential
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.
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.
*** please see files of U.K. exports of M.B.M. AND OTHER MEAT PRODUCTS (metric tonnes), and to the countries (including Germany), they went to;
UK EXPORTS OF MBM TO WORLD
OTHERS
BEEF AND VEAL
LIVE CATTLE
FATS
EMBRYOS
GELATIN ETC
SEMEN
MEAT
Camel Prion Disease Update 2026
It is clear that the designing scientists must also have shared Mr Bradley's surprise at the results because all the dose levels right down to 1 gram triggered infection.
6. It also appears to me that Mr Bradley's answer (that it would take less than say 100 grams) was probably given with the benefit of hindsight; particularly if one considers that later in the same answer Mr Bradley expresses his surprise that it could take as little of 1 gram of brain to cause BSE by the oral route within the same species. This information did not become available until the "attack rate" experiment had been completed in 1995/96. This was a titration experiment designed to ascertain the infective dose. A range of dosages was used to ensure that the actual result was within both a lower and an upper limit within the study and the designing scientists would not have expected all the dose levels to trigger infection. The dose ranges chosen by the most informed scientists at that time ranged from 1 gram to three times one hundred grams. It is clear that the designing scientists must have also shared Mr Bradley's surprise at the results because all the dose levels right down to 1 gram triggered infection.
cwd to cattle 2023
Prion Conference 2023
Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure
Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.
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.
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
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.
cwd to pigs
WEDNESDAY, JANUARY 28, 2026
Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak
cwd to sheep
Chronic Wasting Disease CWD vs Scrapie TSE Prion
Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, 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.
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Research
Title: Differentiation of scrapie from chronic wasting disease in white-tailed deer
Accomplishments
1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure. It has long been suggested that prion disease in deer (chronic wasting disease (CWD)) was caused by the prion agent from sheep. The prion disease that affects sheep, scrapie, has been recognized for hundreds of years. However, chronic wasting disease, a similar disease found in WTD, has only been recognized since the 1960s. ARS researchers in Ames, Iowa, showed that white-tailed deer sick with scrapie from sheep can infect other deer under conditions mimicking natural exposure. Furthermore, this work shows that CWD is difficult to differentiate from WTD infected with scrapie. WTD scrapie prions accumulate in the lymphoreticular system in a manner similar to CWD, meaning that environmental contamination may occur through feces, saliva, and other body fluids of scrapie affected WTD as has been shown for CWD. The presence of WTD infected with scrapie could confound mitigation efforts for chronic wasting disease. This information informs regulatory officials, the farmed cervid industry, and officials tasked with protecting animal health such as state Departments of Agriculture, Natural Resources, or Parks and Wildlife with regard to a disease similar to CWD but arising from sheep scrapie that could be present in WTD that have contact with scrapie affected sheep and/or goats.
Saturday, April 25, 2026
USDA Statement BSE Surveillance Information Center Update 2026
USA Report, Scrapie, CWD, BSE, TSE, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026
April 2026
Possible alignment of the EU BSE surveillance with the new WOAH provisions
I’m thinking iatrogenic TSE Prion
Servicios Personalizados Revista
Revista médica de Chile versión impresa ISSN 0034-9887
Resumen
LEGUA-KOC, Sergio et al. Creutzfeldt-Jakob disease, experience in 17 patients. Rev. méd. Chile [online]. 2021, vol.149, n.9, pp.1285-1291. ISSN 0034-9887. http://dx.doi.org/10.4067/S0034-98872021000901285.
Background:
Creutzfeldt-Jakob disease (CJD) is a prion affection that typically produces a rapidly progressive dementia with different neurologic and extra-neurologic manifestations.
Aim:
To characterize clinical, imaging and electroencephalography findings in patients with a probable CJD.
Patients and Methods:
A case series study of patients admitted in the Neurology department at a public hospital, between 2014 and 2019. Demographic, clinical, imaging, and electroencephalographic data of patients with probable CJD were analyzed.
Results:
Seventeen patients aged 63 ± 11 years (53% women) with a probable CJD were gathered. The incidence was 4.7 cases/year per million inhabitants. Twenty four percent of patients had a family history of CJD. The median time between the onset of symptoms and the hospital admission was three months with a survival of four months. The most common clinical manifestations were an amnesic syndrome in 88%, myoclonus in 76%, frontal syndrome and ataxia in 71%. Brain MRI was abnormal in all patients. The preponderant finding was the involvement of the caudate nucleus in 82% of cases. In the EEG, 94% of patients had abnormalities. All had a theta-delta slowing as a base rhythm. The pseudo-periodic pattern was observed in the 29% and status epilepticus in 18%.
Conclusions:
In this group of patients we observed the heterogeneity of the clinical manifestations of the disease, the frequent imaging and electroencephalographic alterations and the short evolution time leading to death.
Palabras clave : Dementia; Creutzfeldt-Jakob Syndrome; Prion Diseases; Status Epilepticus.
18 patients exposed cjd
State of the art in biosafety at the European National Reference Laboratories for Transmissible Spongiform Encephalopathies
terry
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