Subject: The European Union Summary Report On Surveillance For The Presence Of Transmissible Spongiform Encephalopathies (TSE): The Situation In 2017
234. The European Union Summary Report On Surveillance For The Presence Of Transmissible Spongiform Encephalopathies (TSE): The Situation In 2017
Angel Ortiz Pelaeza, Valentina Rizzia, Giuseppe Rub, Francesco Ingravalleb and Yves Van der Stedea
aUnit on Biological Hazards and Contaminants, Department of Risk Assessment & Scientific Assistance, European Food Safety Authority (EFSA); bBiostatistics, Epidemiology and Risk Analysis (BEAR) Unit. Istituto Zooprofilattico Sperimentale di Piemonte, Liguria e Valle d ’Aosta, Torino (Italia)
CONTACT Angel Ortiz Pelaez angel.ortizpelaez@efsa.europa.eu
ABSTRACT
The European Food Safety Authority publishes a yearly summary of the surveillance activities on transmissible spongiform encephalopathies (TSE) in bovine animals, sheep, goats, cervids, and other species, in the European Union (EU), and in Iceland, Norway and Switzerland. Target groups include: animals clinically suspected of being infected by TSE; animals culled under TSE eradication measures; animals with clinical signs at ante-mortem; emergency slaughtered; fallen stock/not slaughtered for human consumption and healthy slaughtered animals for human consumption, for cervids also hunted and road or predator-injured or killed.
For the first time since bovine spongiform encephalopathy (BSE) had been reported, no cases of classical BSE were reported world-wide in 2017 [1]. Six atypical BSE cases were reported by Spain (1 H /2 L), France (1 H/1 L) and Ireland (1 L), out of the 1,312,714 cattle tested by 28 EU Member States (MS) and 18,526 tested by three non-MS.
In total 431,815 small ruminants were tested in 2017 in the EU. Compared with 2016, there was a 36.2% increase in the number of cases of classical scrapie (CS) in sheep (933), mostly reported by Greece, Spain, Italy and Romania, although over 75% of the cases were sourced in infected flocks. Atypical scrapie (AS) was confirmed in 94 animals. In goats, a decrease of 10% in the number of cases of classical scrapie (567) were reported, 84% in Cyprus. Atypical scrapie was confirmed in nine animals.
Ten-year trend analysis showed a statistically significant decrease in the sheep proportion of CS cases per 10,000 tested animals and an increase in goats. For AS, 10-year data did not detect any statistically significant trend in both species.
After the discovery of chronic wasting disease (CWD) in Norway in 2016, TSE testing in cervids increased in the EU: 10 MS tested 3,585 cervids (75% in Romania, 98.5% from wildlife), all negative. Norway tested 25,736 deer in 2017, leading to the detection of the first case of CWD in a red deer, nine cases in wild reindeer and one in a wild moose. Following EFSA recommendations, the European Commission introduced a 3-year mandatory surveillance programme for six member states starting on 1 January 2018.
By the time of submitting this abstract, CS/AS cases were not yet available, but one new classical BSE case was confirmed in Scotland (2018), one L-type BSE case in Poland (2019) and one case of CWD in a wild moose in Finland in March 2018.
KEYWORDS: BSE; scrapie; CWD; EU; surveillance
134. The transmissible spongiform encephalopathies surveillance in small ruminants in Romania for a period of 10 years
Florica Bărbuceanua, Ioana Neghirlab, Theodora Chesnoiub, Cristina Diaconua, Stefania-Felicia Barbuceanuc and G. Predoid
aInstitute for Diagnosis and Animal Health Bucharest, București, Romania; bNational Sanitary Veterinary and Food Safety Authority, București, Romania; cFaculty of Pharmacy, Carol Davila University of Medicine and Pharmacy, București, Romania; dFaculty of Veterinary Medicine Bucharest, București, Romania
CONTACT Florica Bărbuceanu barbuceanu.florica@idah.ro
ABSTRACT
The scarpie belongs to the TSE group and is a fatal degenerative disease, affecting the central nervous system of ovine and caprine. There are two types of scrapie: classical and atypical. The classical scrapie affects the animals aged from 2 to 5 years and is very contageous, while the atypical scrapie affects the animals older than 5 years and is considered to have a low degree of infectivity. The aim of this study is to present information on the Romanian TSE Surveillance Program, performed in compliance with the EU Commission and OIE requirements, as well as the TSE test results in small ruminants (animals of the category clinical suspicions, dead animals and animals slaughtered normally) performed in the national net for transmissible spongiform encephalopathies, between 2007 and 2017. Between 2007 and 2017 were tested in Romania approximatively 339,643 small ruminants. All the 787 cases of scrapie were confirmed by the TSE NRL (National Reference Laboratory) by immunoblotting and immunohistochemical tests, with the performance of the discriminatory and genotyping testing. All animals diagnosed with scrapie were affected by the classical form. For all suspicion cases, a differential diagnosis was performed against rabies, Aujeszky disease and listeriosis.
KEYWORDS: Classical scrapie; Romanian TSE surveillance program; NRL-TSE
154. TSEs in European goats discriminated by Western blotting into five types based on five robust molecular parameters
L Pirisinua, O Andreolettib, I Lantierc, PL Acutisd, C Acine, W Goldmannf, T Sklaviadisg, L Ekateriniadouh, C Fasti, P Papasavva – Stylianouj, S Simonk, J Spiropoulosl, U Agrimia, JG Jacobsm, A Bossersm, LJM van Keulenm, M Mazzad, R Nonnoa and JPM Langeveldm
aIstituto Superiore di Sanità, Department of Food Safety, Nutrition and Veterinary Public Health, Rome, Italy; bUMR INRA ENVT 1225- IHAP, École Nationale Vétérinaire de Toulouse, Toulouse, France; cINRA-Centre Val de Loire, Infectiologie et Santé Publique, Nouzilly, France; dIstituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d’Aosta, Torino, Italy; eCentro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, Universidad de Zaragoza, Zaragoza, Spain; fThe Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, Easter Bush, United Kingdom; gLaboratory of Pharmacology, School of Health Sciences, Department of Pharmacy, Aristotle University of Thessaloniki, Thessaloniki, Greece; hNational Agricultural Research Foundation, Veterinary Research Institute, Thessaloniki, Greece; iInstitute of Novel and Emerging Infectious Diseases, Friedrich-Loeffler-Institute, Greifswald-Isle of Riems, Germany; jVeterinary Services, Nicosia, Cyprus; kService de Pharmacologie et Immunoanalyse (SPI), Laboratoire d’Etudes et de Recherches en Immunoanalyse, CEA, INRA, Université Paris-Saclay, Gif-sur-Yvette, France; lAnimal and Plant Health Agency, New Haw, Addlestone, Surrey, United Kingdom; mWageningen BioVeterinary Research, Lelystad, the Netherlands
CONTACT Laura Pirisinu laura.pirisinu@iss.it
ABSTRACT
Contrasting the knowledge about prion diseases or TSEs in sheep, only a very limited number of strain typing studies are available in goats. Two cases deriving from the zoonotic bovine BSE epidemic were however detected in goats. During 2004–2012, over 70 TSE goat brain samples were collected from seven European countries and evaluated for TSE type/strain variation. A selection of these materials was chosen for in-depth analysis based on various criteria: tissue quality, genotype, broad geographical distribution, potential type variation. Of these, 37 cases were biochemically analysed (present study) and a subset of these subjected to bioassays in seven rodent models (see parallel study of Nonno et al.). Analyses of these isolates showed that in goats different PrPSc types exist, comparable to those found in sheep such as classical scrapie, CH1641-like scrapie and Nor98/atypical scrapie. However, classical scrapie isolates could be further differentiated in two molecular subtypes based on the PK susceptibility of the N-terminus and on the molecular weight of PrPres. Importantly, the two subtypes of classical scrapie showed different geographical distribution, as one subtype was only detected in goats deriving from Italy and France. These analyses also show, that none of the field samples exhibited BSE-like features and offer a comprehensive set of robust molecular PrPres properties to exclude suspicion for the presence of BSE. These are: molecular mass of the triplet bands, presence of N-terminus epitope, glycoprofile markers, a dual population marker and the structural stability of PrP-core.
This work was made possible by national support and by the following European grants: Neuroprion (FP6-FOOD-506,579), GoatBSE (FOOD-CT-2006–36,353) and GOAT-TSE-FREE (EMIDA-ERA NET). We want to memorize Jorg G Jacobs, who died in 2017. Jorg developed and performed the Triplex-WB system as well as characterized antibodies like 94B4, 12B2, 9A2, 6C2, SAF84 and L42. He also was crucial in the distinction of C-, L- and H-type BSE and in 2011 he showed elegantly the allotype composition of prion material in heterozygous sheep by the use of Endo-LysC.
164. Overview of scrapie cases in Iceland 2004–2018
Eva Hauksdottir and Stefania Thorgeirsdottir
Keldur, the Institute for Experimental Pathology, University of Iceland
CONTACT Eva Hauksdottir evahauks@hi.is
ABSTRACT
Introduction: Since 1978 Iceland has actively screened healthy slaughtered (HS) sheep for scrapie, however, in 2004 the screening method changed from histopathological staining to a rapid ELISA test. With this new method scrapie cases in HS were identified for the first time, as before index scrapie cases had only been detected in sheep with clinical symptoms (CS). Use of this sensitive method also led to the detection of the first atypical scrapie case (Nor98) in Iceland in 2004.
Materials and Methods: During 2004–2018 there were 50,515 HS samples tested with ELISA, along with 558 samples from fallen stock and clinical suspects. In this overview, the cases detected during this time, will be analysed further, e.g. origin, age, PrP genotype and number of additional cases from each farm detected after culling of the scrapie flock.
Results: In the years 2004–2018 there were 29 scrapie cases identified, where one case refers to a single affected farm. Two thirds of the classical scrapie cases originate from CS sheep, while Nor98 is generally identified in HS samples rather than CS. The genotype most associated with susceptibility to classical scrapie is frequent in the classical scrapie cases, however a neutral genotype is found in many of those cases as well. After culling, where a scrapie flock (or a portion of it) was tested, additional positive samples of classical scrapie were frequently detected, but only once has an additional Nor98 case been found in a culled flock.
Conclusion: The comparison of classical and atypical scrapie in Iceland shows that they have many different qualities in relation to origin, symptoms, genotype and flock infectivity.
Thursday, May 23, 2019
Prion 2019 Emerging Concepts CWD, BSE, SCRAPIE, CJD, SCIENTIFIC PROGRAM Schedule and Abstracts
https://www.facebook.com/groups/1557515941145821/
WEDNESDAY, MAY 29, 2019
Incomplete inactivation of atypical scrapie following recommended autoclave decontamination procedures USDA HERE'S YOUR SIGN!
WEDNESDAY, JANUARY 18, 2017
Ireland Department of Agriculture confirmed a case of Atypical BSE 18 year old cow
THURSDAY, OCTOBER 18, 2018
Scotland Bovine Spongiform Encephalopathy (BSE) Mad Cow Disease has been confirmed on a farm in Aberdeenshire
SUNDAY, JUNE 25, 2017
PRION 2017 P124 The Present Romanian Epidemiological Context With Regard To The Bovine Spongiform Encephalopathy
>>> It is distinct from atypical BSE, which may develop spontaneously, according to information from the U.S. Centers for Disease Control and Prevention.
THIS IS A MYTH $$$
***atypical spontaneous BSE in France LOL***
FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$
***so 20 cases of atypical BSE in France, compared to the remaining 40 cases in the remaining 12 Countries, divided by the remaining 12 Countries, about 3+ cases per country, besides Frances 20 cases. you cannot explain this away with any spontaneous BSe. ...TSS
Sunday, October 5, 2014
France stops BSE testing for Mad Cow Disease
Thursday, March 24, 2016
FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une vache dans les Ardennes
***atypical spontaneous BSE in France LOL***
FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$
MONDAY, FEBRUARY 04, 2019
POLAND DETECTS BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION
Poland is Proof atypical BSE is NOT an old cow spontaneous disease...tss
we have seen the spontaneous BSE epidemic in France, what about the other HIGH INCIDENCE ATYPICAL BSE COUNTRY OF POLAND, another atypical spontaneous event of high incidence. how can this be blamed on a happenstance of nothing, i.e. old age? goes against all junk science to date on the spontaneous atypical BSE i.e.
> In 2015, the OIE determined that atypical BSE occurred spontaneously at a low rate in all cattle populations and would be excluded for BSE risk. ...
>Atypical BSE occurs in older cattle, usually 8 years of age or greater, and does not appear to be associated with contaminated feed. Like classic or sporadic CJD in humans, it seems to arise rarely and spontaneously.
POLAND ATYPICAL BSE AND SPORADIC CJD
we have seen the spontaneous BSE epidemic in France, what about the other HIGH INCIDENCE ATYPICAL BSE COUNTRY OF POLAND, another atypical spontaneous event of high incidence. how can this be blamed on a happenstance of nothing, i.e. old age? goes against all junk science to date on the spontaneous atypical BSE i.e.
> In 2015, the OIE determined that atypical BSE occurred spontaneously at a low rate in all cattle populations and would be excluded for BSE risk. ...
>Atypical BSE occurs in older cattle, usually 8 years of age or greater, and does not appear to be associated with contaminated feed. Like classic or sporadic CJD in humans, it seems to arise rarely and spontaneously.
POLAND ATYPICAL BSE AND SPORADIC CJD
Atypical status of bovine spongiform encephalopathy in Poland: a molecular typing study
Summary
The aim of this study was to analyze molecular features of protease-resistant prion protein (PrPres) in Western blots of BSE cases diagnosed in Poland with respect to a possible atypical status. Confirmed cases were analyzed by Western blotting with several monoclonal antibodies directed at N-terminal and core epitopes of prion protein (PrP). Most cases showed the classical glycoprofile characterized by the dominance of the di- over the monoglycosylated PrPres band, yielding di-/mono- ratios well above 2 and by reactivity with antibodies having their epitopes in bovine PrP region 110–242 (C-type cases). Surprisingly, seven cases of BSE were atypical. Six were classified as L-type based on a slightly lower molecular mass (Mr) of the non- glycosylated band with respect to C-types and a conspicuously low di-/mono- ratio of glycosylated PrPres bands approaching unity. One case was classified as H-type because of a higher Mr of PrPres bands on the blot when compared with C-type cases. A characteristic epitope of H-type PrPres occurred in the 101–110 region of PrP for which only antibody 12B2 had a sufficient affinity. The occurrence of atypical cases only in animals 9 years of age and older raises questions about the mechanisms of prion diseases and the origin of BSE.
Atypical status of bovine spongiform encephalopathy in Poland: a molecular typing study
M. P. Polak1, J. F. Zmudzinski1, J. G. Jacobs2, J. P. M. Langeveld2
1 National Veterinary Research Institute, Pulawy, Poland
2 Central Institute for Animal Disease Control (CIDC-Lelystad), Lelystad, The Netherlands Received 24 April 2007; Accepted 27 August 2007; Published online 26 September 2007 # Springer-Verlag 2007
snip...
Clarification of whether these atypical cases represent genuine strains of BSE would be accomplished by transmission studies in mice. Such studies have already been performed in France, Germany and Italy [3, 5, 14, 15]. For H-type cases in France, successful transmission was achieved in both wild-type, and transgenic mice expressing bovine and ovine PrPC. In Germany, successful transmission of both an L-type and an H-type case to transgenic mice overexpressing bovine PrPC has been described. PrPres from those mice was identical to the inoculum used in the study, proving the existence of distinct strains of BSE. All atypical features of those isolates were maintained in the inoculated mice, indicating the existence of several prion strains in cattle, or alternatively a possible evolution to a single BSE strain, as suggested from data obtained by Capobianco et al. with wild-type inbred mice [15]. This second hypothesis could fit with data from the United Kingdom, where over 180,000 cases of BSE were diagnosed by passive surveillance. British and European experience based on tissue analysis from clinically affected animals showed consistent characteristics of BSE agent not only on histological sections from cattle brains but also when inoculating mice, pointing to the existence of one uniform strain of BSE. Therefore, it is possible that a sporadic form of BSE present in the cattle population at a very low rate in the past could have spread to naive animals via contaminated meatand-bone meals. Spontaneous BSE, if it occurs, must be a very rare phenomenon. However, data for Poland, where 14% of all cases comprised an atypical form of BSE, seems to be in contradiction to this hypothesis. But when the average age of all positive cases in Poland is taken into account, BSE is generally found in older animals (mean age of 7.7). Analysis of the age structure of cattle in Poland in the period of 2002–2006 shows that 56–60% of all animals were 7 years old and above. A much larger number of cattle should be tested to get better insight into the real prevalence of atypical BSE. However, current tendencies based on economic analysis point to a decrease in the number of tests performed rather than expanding this scheme any further. It would be sensible to maintain a certain level of testing focused on the older age group to distinguish between a stable, thus sporadic-based, situation of BSE, or alternatively a fade-out, thus epidemic-based, situation. Exploring the subject of spontaneous BSE in the cattle population may be ceased for economic reasons, and it may never be known while this answer is in our reach thanks to great financial efforts in recent years.
Poland is Proof atypical BSE is NOT an old cow spontaneous disease...tss
Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide* (excluding the United Kingdom) Country/Year
Poland
89 90 91 92 93 94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09 10 11 12 13 14 15 16
0 0 0 0 0 0 0 0 0 0 0 0 0 4f 5 11 19 10 9 5 4 2 1m 3 1 0 0 0
Source: USDA, APHIS, VS
What is the level of passenger traffic arriving in the United States from Poland?
A total of 188,946 passengers arrived at US airports on direct flights from Poland in fiscal year 2000.
An undetermined number of passengers arrived in the US from Poland via indirect flights.
Under APHIS-PPQ’s agricultural quarantine inspection monitoring, 451 air passengers from Poland were sampled for items of agricultural interest in fiscal year 2000.
Thirteen (13) of these passengers, or 2.9 percent, carried a total of 26.2 kg of meat items that could potentially harbor the pathogen(s) that cause BSE.
None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the US.
Source: US Department of Transportation, and APHIS-PPQ Agricultural Quarantine Inspection data base CEI’s plans for follow up: CEI has no plans to provide additional information on this situation. If you need more information or wish to comment, you may contact Judy Akkina at (970) 490-7852 or Carol Tuszynski at (970) 490-7893.
What measures has USDA-APHIS taken to prevent the introduction of BSE? To prevent BSE from entering the United States, APHIS has restricted the importation of live ruminants and certain ruminant products from countries where BSE is known to exist.
Greetings FDA and public,
if you go to the below site, and search all BSE known countries and check out their air traffic illegal meat they have confiscated, and check out the low number checked, compared to actual passenger traffic, would not take too much for some nut to bring in FMD/TSEs into the USA as a 'suitcase bomb'.
[[Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284 air passengers from Israel were sampled for items of agricultural interest in fiscal year 2001. Seven of these passengers, or 2 percent, carried a total of 11 kg of meat items that could potentially harbor the pathogen that causes BSE. None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the U.S.]]
if they were to have questioned the terrorist that bombed the Twin Towers with jets, if they were to have questioned them at flight school in the USA, i am sure that they would have said they did not intend to visit the Twin Towers as a flying bomb either. what am i thinking, they probably did ask this? stupid me.
[[In 1999 a small amount of non-species specific meat and offal was imported and a small amount of fetal bovine serum (FBS) was also imported. FBS is considered to have a relatively low risk of transmitting BSE.]] more of the USA infamous 'non-species coding system', wonder how many of these species are capable of carrying a TSE?
snip...
A total of 524,401 passengers arrived on direct flights to the U.S. from Israel in fiscal year 2000. This number does not include passengers who arrived in the U.S. from Israel via indirect flights. Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284 air passengers from Israel were sampled for items of agricultural interest in fiscal year 2001. Seven of these passengers, or 2 percent, carried a total of 11 kg of meat items that could potentially harbor the pathogen that causes BSE. None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the U.S.
PRION 2018 CONFERENCE
P98 The agent of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism transmits after oronasal challenge
Greenlee JJ (1), Moore SJ (1), and West Greenlee MH (2) (1) United States Department of Agriculture, Agricultural Research Service, National Animal Disease Center, Virus and Prion Research Unit, Ames, IA, United States (2) Department of Biomedical Sciences, Iowa State University College of Veterinary Medicine, Ames, IA, United States.
reading up on this study from Prion 2018 Conference, very important findings ;
***> 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.
PRION 2018 CONFERENCE ABSTRACT
WEDNESDAY, OCTOBER 24, 2018
Experimental Infection of Cattle With a Novel Prion Derived From Atypical H-Type Bovine Spongiform Encephalopathy
MONDAY, JANUARY 09, 2017
Oral Transmission of L-Type Bovine Spongiform Encephalopathy Agent among Cattle
CDC Volume 23, Number 2—February 2017
*** Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
*** Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
FRIDAY, FEBRUARY 22, 2019
Spain Bovine Spongiform Encephalopathy BSE TSE Confirmed
MONDAY, JUNE 19, 2017
PRION 2017 CONFERENCE ABSTRACT P61 vCJD strain properties in a Spanish mother and son replicate as those of a young UK case
FRIDAY, MAY 12, 2017
SPAIN OIE Bovine Spongiform Encephalopathy atypical L-type Camargo, CANTABRIA
FRIDAY, MARCH 10, 2017
OIE Spain Prion (Atypical BSE type L) Bovine Spongiform Encephalopathy Mad Cow Disease
FRIDAY, JANUARY 09, 2009
Mad cow disease detected on Madrid farm
SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma) SEACAgenda 102nd Meeting on Wednesday 4 March 2009 Room 808, Nobel House, 17 Smith Square, Defra, London SW1P 3JR10.05 Approval of draft minutes from SEAC 101
snip...
ITEM 3 - CURRENT ISSUES 8. SEAC was informed about the following issues: . A mother and son in Spain had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. As both the mother and son lived in a region of Spain with a history of BSE, had frequently shared meals of cattle brain, and as no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure. Furthermore, the similar times of onset of disease of the cases did not suggest transmission had occurred from one to the other.
snip...
Thursday, February 26, 2009
SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma)
STATEMENT ON A VARIANT CJD FAMILY CLUSTER
Prof Robert G Will,NCJDSU 25th September 2008r.g.will@ed.a c.uk
Three cases of pathologically confirmed variant CJD have been identified in Spain in recent years, including a man in his early 40s who died earlier this year. The clinical illness in this individual was typical of variant CJD, including the appearances on the MRI brain scan.
A few months ago his mother, who was in her 60s, developed a rapidly progressive neurological illness and died about 5 months from the onset of this illness. An MRI brain scan showed appearances suggestive of variant CJD and preliminary results from post-mortem examination suggest that the suspected diagnosis of variant CJD is correct. Further results, which may confirm this diagnosis, should be available within a few days.
Since 1994 there have been 167 cases of variant CJD in the UK, 23 cases in France and 15 cases in other countries, excluding Spain. The occurrence of variant CJD in more than one member of the same family has not been seen before and it has been the general view that family members of variant CJD cases are not themselves at greater risk of developing this condition. This raises the question as to why two cases of variant CJD have now been found in a family in Spain. There is no evidence of a genetic form of CJD in these Spanish cases and preliminary investigation has not shown any risk of CJD through medical or surgical treatment.
There is no evidence of any risk of transmission of CJD through direct personal contact. The mother and son lived in an area of Spain in which BSE has been found and it is possible that direct consumption of material with high levels of BSE infection may have been the source of the infection. In the UK and other countries it is believed that processed bovine tissues were the most likely source of BSE infection and it is possible that different forms of exposure to BSE infection may explain the occurrence of variant CJD in two family members in Spain and not elsewhere.
This, however, is uncertain and public health policies in relation to variant CJD may have to be reviewed in the light of these two cases in Spain.
Monday, September 01, 2008
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
Spanish woman and son could be first relatives to die of BSE
Submitted by Marina Dimova on Wed, 08/27/2008 - 11:53.
The Spanish health authorities are investigating the death of a woman whose son died earlier of Creutzfeldt-Jakob Disease (CJD), the human variant of bovine spongiform encephalopathy (BSE) or mad cow disease, experts said Wednesday in Madrid.
If it is confirmed that the woman and her son died from the same cause, they would be the first members of the same family in the world to succumb to CJD, neuropathologist Alberto Rabano said.
The woman, who was about 60-years-old, passed away last week in the northern city of Leon. The son died in February. Their identities were not given.
Veterinary experts said the son caught the disease by eating infected meat before 2001, when preventative measures were adopted.
Terry S. Singeltary Sr. Doctor Antonio Ruiz Villaespesa, pathologist and CJD researcher deceased because of Creutzfeldt-Jakob Disease SPAIN. 21 Apr 2009. [Accessed 11 Apr 2012]. In: Monitoring the occurrence of emerging forms of CJD [blog]. Available from:
snip...see full text ;
MONDAY, SEPTEMBER 01, 2008
Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
SUNDAY, MARCH 28, 2010
SPAIN BSE, Nor-98 atypical scrapie, SPORADIC CJD HIGH INCIDENT RATE >2 PER MILLION
FRIDAY, JANUARY 09, 2009
Mad cow disease detected on Madrid farm Friday, January 9, 2009
Eurosurveillance, Volume 10, Issue 31, 04 August 2005
Articles
Citation style for this article: Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(31):pii=2764. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2764
First case of vCJD reported in Spain
Centro Nacional de Epidemiología, Instituto de Salud Carlos III, Madrid, Spain (http://cne.isciii.es/)
The Spanish Ministry of Health has reported the detection of a case of variant Creutzfeldt-Jakob disease (vCJD) in Spain [1].
The patient was a 26 year old woman in Madrid who died on 10 July 2005. She had no specific risk factors for vCJD (no prior blood transfusions or visits to the United Kingdom).
The patient’s symptoms began in November 2004, with rapid progression to dementia, normal MRI and EEG, and 14-3-3 protein found in cerebral spinal fluid. Other neurological manifestations including ataxia, dysarthria, apraxia and myoclonus appeared in early 2005. The first abnormal MRI was observed in April 2005. The patient was methionine homozygous at codon 129 of the prion protein gene (PRPN). There were no identified PRPN mutations or family history of CJD.
The case was notified to the Spanish CJD state registry in May 2005 and initially fitted the criteria for both probable sporadic CJD (sCJD) and possible vCJD; it was registered as probable sCJD. Brain neuropathology results of tests conducted at Fundación Hospital de Alcorcón at Madrid subsequently confirmed vCJD. Materials have been sent to the European reference centre at the United Kingdom’s National CJD Surveillance Unit in Edinburgh for further study, and results are expected later in August.
References:
Ministerio de Sanidad y Consumo. La Comunidad de Madrid comunica al Ministerio de Sanidad la probable detección del primer caso de la variante de la enfermedad de Creutzfeldt-Jakob en España. Press release 29 July 2005. (http://www.msc.es/gabinetePrensa/notaPrensa/desarrolloNotaPrensa.jsp?id=385)
164. Overview of scrapie cases in Iceland 2004–2018
Eva Hauksdottir and Stefania Thorgeirsdottir
Keldur, the Institute for Experimental Pathology, University of Iceland
CONTACT Eva Hauksdottir evahauks@hi.is
ABSTRACT
Introduction: Since 1978 Iceland has actively screened healthy slaughtered (HS) sheep for scrapie, however, in 2004 the screening method changed from histopathological staining to a rapid ELISA test. With this new method scrapie cases in HS were identified for the first time, as before index scrapie cases had only been detected in sheep with clinical symptoms (CS). Use of this sensitive method also led to the detection of the first atypical scrapie case (Nor98) in Iceland in 2004.
Materials and Methods: During 2004–2018 there were 50,515 HS samples tested with ELISA, along with 558 samples from fallen stock and clinical suspects. In this overview, the cases detected during this time, will be analysed further, e.g. origin, age, PrP genotype and number of additional cases from each farm detected after culling of the scrapie flock.
Results: In the years 2004–2018 there were 29 scrapie cases identified, where one case refers to a single affected farm. Two thirds of the classical scrapie cases originate from CS sheep, while Nor98 is generally identified in HS samples rather than CS. The genotype most associated with susceptibility to classical scrapie is frequent in the classical scrapie cases, however a neutral genotype is found in many of those cases as well. After culling, where a scrapie flock (or a portion of it) was tested, additional positive samples of classical scrapie were frequently detected, but only once has an additional Nor98 case been found in a culled flock.
Conclusion: The comparison of classical and atypical scrapie in Iceland shows that they have many different qualities in relation to origin, symptoms, genotype and flock infectivity.
Thursday, May 23, 2019
Prion 2019 Emerging Concepts CWD, BSE, SCRAPIE, CJD, SCIENTIFIC PROGRAM Schedule and Abstracts
MONDAY, JANUARY 28, 2019
Scrapie (Rida) Iceland Information received on 28/01/2019 from Dr Sigurborg Daðadóttir, Chief Veterinary Officer
FRIDAY, NOVEMBER 2, 2018
OIE Final Report Iceland Scrapie TSE Prion 02/11/2018 Start of Event 12/09/2018
MONDAY, DECEMBER 18, 2017
Scrapie Detected on North Iceland Sheep Farm
FRIDAY, SEPTEMBER 23, 2016
North Iceland reporting more cases of Scrapie (Rida)
FINLAND MOOSE FOUND DEAD IN FOREST WITH CHRONIC WASTING DISEASE 8.3.2018 12:56
The chronic wasting disease (CWD) has been found in a moose or European elk (Alces alces) for the first time ever in Finland. The disease was diagnosed in Kuhmo in a 15-year old moose that had died naturally. The results of the analyses carried out by Finnish Food Safety Authority Evira have been verified by a EU reference laboratory. Species of the deer family, known as “cervids”, can suffer from the chronic wasting disease, and it is always fatal. The disease is not known to have been contracted by people.
Norway was before this case the only European country where CWD has been diagnosed. The monitoring of the occurrence of the disease was intensified from the beginning of 2018 in Finland and five other EU Member States.
In Finland, the occurrence of the disease has been studied already since 2003. None of the ca. 2 500 samples analysed so far had tested positive for the disease. The monitoring of the disease will now be further intensified in the Kuhmo and Kainuu region. Hunters are going to be provided with more instructions before the start of the next hunting season, if appropriate.
The chronic wasting disease is not known to have been contracted by people. Moose meat is safe to eat and no restrictions are imposed on the sales and exportation of meat of animals of the deer family. As a precautionary measure the export of live animals of the deer family to other countries will be discontinued for now.
CWD is a slowly progressing disease of deer, elk, reindeer, and moose which always leads to death. The chronic wasting disease is a prion disease and related to the BSE (bovine spongiform encephalopathy) and other TSE diseases (transmissible spongiform encephalopathy). The disease is common in North America. The moose found in Kuhmo did not suffer from the North American, highly contagious form of the chronic wasting disease. The disease seems to resemble most the form of cervid TSE diagnosed in Norway, which appears to be found incidentally in individual animals of the deer family.
For more information, please contact:
Leena Räsänen, Director, tel. +358 50 388 6518 (Food Safety)
Terhi Laaksonen, Head of Unit, tel. +358 40 159 5812 (Control of Animal Diseases)
Sirkka-Liisa Korpenfelt, Senior Resarcher, tel. + 358 50 351 0308 (Laboratory Analyses)
Antti Oksanen, Research Professor, tel. +358 44 561 6491 (Wild Animal Diseases)
Kajsa Hakulin, Ministerial Advisor, Ministry of Agriculture and Forestry, tel. +358 295 162361 (National and EU Legislation)
https://www.evira.fi/en/animals/current_issues/2018/moose-found-dead-in-forest-with-chronic-wasting-disease/
SATURDAY, MARCH 10, 2018
FINLAND REPORTS FIRST CASE OF CHRONIC WASTING DISEASE CWD TSE PRION IN A moose or European elk (Alces alces)
FRIDAY, APRIL 12, 2019
Sweden Wasting Disease (CWD) discovered on moose in Norrbotten County
FRIDAY, MARCH 29, 2019
First Detection of Chronic Wasting Disease in a Wild Red Deer (Cervus elaphus) in Europe
TUESDAY, MAY 14, 2019
First case of atypical scrapie in a goat in Japan
TUESDAY, JULY 03, 2018
Chronic Wasting Disease CWD TSE Prion Global Report Update, USA, CANADA, KOREA, NORWAY, FINLAND, Game Farms and Fake news
FRIDAY, MARCH 15, 2019
Saskatchewan Chronic Wasting Disease TSE Prion 349 Cases Positive for 2018
THURSDAY, MARCH 14, 2019
USDA APHIS CDC Cervids: Chronic Wasting Disease Specifics Updated 2019
SATURDAY, MARCH 16, 2019
Chronic Wasting Disease CWD TSE Prion United States of America Update March 16, 2019
TUESDAY, MARCH 26, 2019
USDA ARS 2018 USAHA RESOLUTIONS Investigation of the Role of the Prion Protein Gene in CWD Resistance and Transmission of Disease
WEDNESDAY, APRIL 03, 2019
Estimating the amount of Chronic Wasting Disease infectivity passing through abattoirs and field slaughter
WEDNESDAY, MARCH 06, 2019
Norway The Madness Continues in Nordfjella Chronic Wasting Disease CWD TSE Prion
hay, straw, grains...and cwd tse prion
***> NORWAY CWD UPDATE December 2018 Report from the Norwegian Scientific Committee for Food and Environment (VKM) 2018: 16
Factors that can contribute to spread of CWD – an update on the situation in Nordfjella, Norway
Opinion of Panel on biological hazards of the Norwegian Scientific Committee for Food and Environment 13.12.2018 ISBN: 978-82-8259-316-8 ISSN: 2535-4019 Norwegian Scientific Committee for Food and Environment (VKM) Po 222 Skøyen 0213 Oslo Norway FRIDAY, DECEMBER 14, 2018 Norway, Nordfjella VKM 2018 16
Factors that can contribute to spread of CWD TSE Prion UPDATE December 14, 2018
THURSDAY, OCTOBER 25, 2018
***> Norway New additional requirements for imports of hay and straw for animal feed from countries outside the EEA due to CWD TSE Prion
TUESDAY, AUGUST 28, 2018
USDA finds BSE infection in Florida cow 08/28/18 6:43 PM
http://animalhealthreportpriontse..blogspot.com/2018/08/usda-finds-bse-infection-in-florida-cow.html
WEDNESDAY, AUGUST 29, 2018
USDA Announces Atypical Bovine Spongiform Encephalopathy Detection USDA 08/29/2018 10:00 AM EDT
WEDNESDAY, AUGUST 29, 2018
Transmissible Spongiform Encephalopathy TSE Prion Atypical BSE Confirmed Florida Update USA August 28, 2018
(Le français suit)
The shipments of imported dried beef jerky from Brazil, repackaged in US establishment for export to Canada, must have an additional declaration.
The shipments will initially be exported in bulk to the U.S. The bulk shipment will then be packaged and labelled in the U.S. for re-export to Canada without undergoing any further processing in the U.S. All establishments involved in the chain of distribution have to be approved by the CFIA.
Shipments meeting the following requirements are allowed entry into Canada:
- The product is exported from the U.S. under an FSIS-issued Official Meat Inspection Certificate (OMIC).
- The U.S. OMIC is accompanied by an additional declaration negotiated between CFIA and Brazil.
- A copy of the OMIC issued by Brazil with which the product was initially exported to the U.S. must accompany the OMIC issued by FSIS for re-exporting product to Canada.
- The U.S. OMIC has the following text in the comments section or as an additional annex to the U.S. OMIC:"Beef jerky covered by this certificate was imported from Brazil or Argentina (delete as appropriate), under the Official Meat Inspection Certificate Number__________, a copy of which is attached" and complies with the conditions specified in the CFIA3 Animal Health Import Permit No_________
- The shipment is accompanied by an Animal Health Import Permit issued by the CFIA.
For more information, see Conditions for importation of meat products from the USA.
end
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
WEDNESDAY, MAY 15, 2019
COOL, NAIS, Brazil, Canada, USA, BSE, TSE, Prion
WEDNESDAY, APRIL 24, 2019
USDA Announces Atypical Bovine Spongiform Encephalopathy Detection Aug 29, 2018 A Review of Science 2019
TUESDAY, APRIL 30, 2019
Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies 2018 Annual Report
THURSDAY, MARCH 14, 2019
USDA APHIS CDC FDA BSE TSE PRION UPDATE 2019
MONDAY, JANUARY 21, 2019
Bovine Spongiform Encephalopathy BSE TSE Prion Surveillance FDA USDA APHIS FSIS UPDATE 2019
SUNDAY, APRIL 14, 2019
Estimation of prion infectivity in tissues of cattle infected with atypical BSE by real time-quaking induced conversion assay
WEDNESDAY, APRIL 17, 2019
Estimating the impact on food and edible materials of changing scrapie control measures: The scrapie control model
TUESDAY, MARCH 26, 2019
USDA ARS 2018 USAHA RESOLUTIONS TWO PRONGED APPROACH NEEDED FOR ADVANCING CATTLE TRACEABILITY
***> Wednesday, January 23, 2019
***> CFIA SFCR Guidance on Specified risk material (SRM) came into force on January 15, 2019 <***
SATURDAY, MARCH 2, 2019
MAD COW TSE PRION DISEASE AND THE PEER REVIEW PROCESS OF BSe Science $$$
WEDNESDAY, MARCH 13, 2019
CWD, TSE, PRION, MATERNAL mother to offspring, testes, epididymis, seminal fluid, and blood
USDA APHIS FDA CWD BSE SCRAPIE CJD UPDATE MARCH 2019
SATURDAY, MARCH 16, 2019
Chronic Wasting Disease CWD TSE Prion United States of America Update March 16, 2019
THURSDAY, MARCH 14, 2019
USDA APHIS CDC Cervids: Chronic Wasting Disease Specifics Updated 2019
FRIDAY, MARCH 15, 2019
Saskatchewan Chronic Wasting Disease TSE Prion 349 Cases Positive for 2018
THURSDAY, MARCH 14, 2019
USDA APHIS CDC FDA BSE TSE PRION UPDATE 2019
FRIDAY, MARCH 15, 2019
USDA APHIS SCRAPIE TSE PRION Sheep and Goat Health Update 2019
TSE PRION POKER, ARE YOU ALL IN $$$
SATURDAY, MARCH 16, 2019
Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) Guidance for Industry and Food and Drug Administration Staff Document issued on March 15, 2019 Singeltary Submission
MONDAY, FEBRUARY 25, 2019
MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019
THURSDAY, OCTOBER 04, 2018
Cervid to human prion transmission 5R01NS088604-04 Update
MONDAY, APRIL 01, 2019
PUBLIC HEALTH U of M launches Chronic Wasting Disease Program to address potential health crisis
Scientific Advisors and Consultants Staff 2001 Advisory Committee TSE PRION Singeltary Submission Freas Monday, January 08,2001 3:03 PM FDA Singeltary submission 2001
Greetings again Dr. Freas and Committee Members,
I wish to submit the following information to the Scientific Advisors and Consultants Staff 2001 Advisory Committee (short version). I understand the reason of having to shorten my submission, but only hope that you add it to a copy of the long version, for members to take and read at their pleasure, (if cost is problem, bill me, address below). So when they realize some time in the near future of the 'real' risks i speak of from human/animal TSEs and blood/surgical products. I cannot explain the 'real' risk of this in 5 or 10 minutes at some meeting, or on 2 or 3 pages, but will attempt here:
fda link is dead in the water;
snip...see full text
Terry S. Singeltary Sr.
