Wednesday, July 19, 2017

OIE REPORT Bovine spongiform encephalopathy United States of America

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From: oie-info-web
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Sent: Wed, Jul 19, 2017 10:56 am
Subject: USA 19-07-17 OIE Alert - Alerta - Alerte - Bovine spongiform encephalopathy - Encéphalopathie spongiforme bovine - Encefalopatía espongiforme bovina

Bovine spongiform encephalopathy ,United States of America

Information received on 18/07/2017 from Dr John Clifford, Official Delegate, Chief Trade Advisor, Animal and Plant Health Inspection Service, United States Department of Agriculture, Washington, United States of America

Summary

Report type
Immediate notification (Final report)
Date of start of the event
05/07/2017
Date of confirmation of the event
16/07/2017
Report date
18/07/2017
Date submitted to OIE
19/07/2017
Date event resolved
19/07/2017
Reason for notification
Recurrence of a listed disease
Date of previous occurrence
30/06/2012
Manifestation of disease
Clinical disease
Causal agent
Prion (atypical low-type BSE)
Nature of diagnosis
Laboratory (advanced)
This event pertains to
a defined zone within the country

New outbreaks

Summary of outbreaks
Total outbreaks: 1
Outbreak Location
  • ALABAMA ( Perry County, Perry )
Total animals affected
Species
Susceptible
Cases
Deaths
Killed and disposed of
Slaughtered
Cattle
1
1
1
0
0
Outbreak statistics
Species
Apparent morbidity rate
Apparent mortality rate
Apparent case fatality rate
Proportion susceptible animals lost*
Cattle
100.00%
100.00%
100.00%
100.00%

* Removed from the susceptible population through death, destruction and/or slaughter;

Epidemiology

Source of the outbreak(s) or origin of infection
  • Unknown or inconclusive
  • random mutation
Epidemiological comments
As part of the United States’ targeted surveillance program for bovine spongiform encephalopathy (BSE), a case of Atypical BSE was identified in an 11 year old beef type cow. This Atypical BSE was classified as L-type. Native cases of BSE in the United States have all been atypical BSE cases -- this is only the fourth case of atypical BSE identified in over 20 years of surveillance. The last case was in 2012.

The identified animal did not enter any food supply channels and at no time presented a risk to human health. Specified risk material removal and the ruminant-to-ruminant feed bans continue to be effectively applied.

Control measures

Measures applied
  • Screening
  • Traceability
  • Official disposal of carcasses, by-products and waste
  • Vaccination prohibited
  • No treatment of affected animals
Measures to be applied
  • No other measures

Diagnostic test results

Laboratory name and type
National Veterinary Services Laboratories (NVSL) ( National laboratory )
Tests and results
Species
Test
Test date
Result
Cattle
enzyme-linked immunosorbent assay (ELISA)
15/07/2017
Positive
Cattle
western blot
16/07/2017
Positive

Future Reporting

The event is resolved. No more reports will be submitted.

Encéphalopathie spongiforme bovine ,Etats-Unis d'Amérique

Information reçue le 18/07/2017 de Dr John Clifford, Official Delegate, Chief Trade Advisor, Animal and Plant Health Inspection Service, United States Department of Agriculture, Washington, Etats-Unis d'Amérique

Résumé

Type de rapport
Notification immédiate (rapport final)
Date de début de l’événement
05/07/2017
Date de confirmation de l´événement
16/07/2017
Date du rapport
18/07/2017
Date d'envoi à l'OIE
19/07/2017
Date de clôture de l'événement
19/07/2017
Raison de notification
Réapparition d’une maladie listée par l'OIE
Date de la précédente apparition de la maladie
30/06/2012
Manifestation de la maladie
Maladie clinique
Agent causal
Prion (ESB atypique de type L)
Nature du diagnostic
Tests approfondis en laboratoire (i.e. virologie, microscopie électronique, biologie moléculaire, immunologie)
Cet événement se rapporte à
une zone définie à l'intérieur du pays

Nouveaux foyers

Récapitulatif des foyers
Nombre total de foyers : 1
Localisation du foyer
  • ALABAMA ( Perry County, Perry )
Nombre total d'animaux atteints
Espèce(s)
Sensibles
Cas
Morts
Mis à mort et éliminés
Abattus
Bovins
1
1
1
0
0
Statistiques sur le foyer
Espèce(s)
Taux de morbidité apparent
Taux de mortalité apparent
Taux de létalité apparent
Proportion d'animaux sensibles perdus*
Bovins
100.00%
100.00%
100.00%
100.00%

* Soustraits de la population sensible suite à la mort, à l´abattage et/ou à la destruction;

Epidémiologie

Source du/des foyer(s) ou origine de l´infection
  • Inconnue ou incertaine
  • Mutation aléatoire
Autres renseignements épidémiologiques / Commentaires
Dans le cadre du programme de surveillance ciblée des États-Unis pour l’encéphalopathie spongiforme bovine (ESB), un cas d’ESB atypique a été identifié chez une vache de boucherie de 11 ans. Cette ESB atypique a été classifiée comme type L. Les cas indigènes d’ESB aux États-Unis ont tous été des cas d’ESB atypique ; il s’agit du quatrième cas seulement d’ESB atypique aux États-Unis identifié en plus de 20 ans de surveillance. Le dernier cas remonte à 2012.
L’animal identifié n’a pas été introduit dans la chaîne alimentaire et, à aucun moment il n’a présenté un risque pour la santé humaine. La mise en œuvre efficace de la destruction spécifique des matières à risque ainsi que de l’interdiction de nourrir des ruminants avec des produits de ruminants se poursuit.

Mesures de lutte

Mesures de lutte appliquées
  • Dépistage
  • Traçabilité
  • Destruction officielle des carcasses, des sous-produits et des déchets
  • Vaccination interdite
  • Aucun traitement des animaux atteints
Mesures à appliquer
  • Aucune autre mesure

Résultats des tests de diagnostics

Nom du laboratoire et type
Laboratoires des services vétérinaires nationaux (NVSL) ( Laboratoire national )
Tests et résultats
Espèce(s)
Test
Date du test
Résultat
Bovins
méthode de dosage immuno-enzymatique (ELISA)
15/07/2017
Positif
Bovins
western blot
16/07/2017
Positif

Rapports futurs

L’événement est terminé. Aucun autre rapport ne sera envoyé.

Encefalopatía espongiforme bovina ,Estados Unidos de América

Información recibida el 18/07/2017 desde Dr John Clifford, Official Delegate, Chief Trade Advisor, Animal and Plant Health Inspection Service, United States Department of Agriculture, Washington, Estados Unidos de América

Resumen

Tipo de informe
Notificación inmediata(Informe final)
Fecha del inicio del evento
05/07/2017
Fecha de confirmación del evento
16/07/2017
Fecha del informe
18/07/2017
Fecha de envio del informe a la OIE
19/07/2017
Fecha del cierre del evento
19/07/2017
Motivo de la notificación
Recurrencia de una enfermedad de la Lista de la OIE
Fecha de la anterior aparición de la enfermedad
30/06/2012
Manifestación de la enfermedad
Enfermedad clínica
Agente causal
Prion (EEB atípica de tipo L)
Naturaleza del diagnóstico
Pruebas de diagnóstico de laboratorio avanzadas (ej. virología, microscopía electrónica, biología molecular e inmunología)
Este evento concierne
una zona definida dentro del país

Nuevos focos

Resumen de los focos
Número total de focos: 1
Localización del foco
  • ALABAMA ( Perry County, Perry )
Número total de animales afectados
Especies
Susceptibles
Casos
Muertos
Matados y eliminados
Sacrificados
Bovinos
1
1
1
0
0
Estadística del foco
Especies
Tasa de morbilidad aparente
Tasa de mortalidad aparente
Tasa de letalidad aparente
Proporción de animales susceptibles perdidos*
Bovinos
100.00%
100.00%
100.00%
100.00%

* Descontados de la población susceptible a raíz de su muerte, destrucción o sacrificio;

Epidemiología

Fuente del o de los focos u origen de la infección
  • Desconocida o no concluyente
  • mutación aleatoria
Otros detalles epidemiológicos / comentarios
Dentro del programa de vigilancia específica de los Estados Unidos para la encefalopatía espongiforme bovina (EEB), se ha identificado un caso de EEB atípico en una vaca de engorde de 11 años. Esta EEB atípica fue clasificada como tipo L. Todos los casos autóctonos de EEB en los Estados Unidos fueron casos de EEB atípica; solo se trata del cuarto caso de EEB atípica en los Estados Unidos identificado en más de 20 años de vigilancia. El último caso fue en el 2012.
El animal identificado no entró en la cadena alimentaria y en ningún momento presentó un riesgo para la salud humana. La implementación eficaz de la destrucción especifica de las materias de riesgo así como la interdicción de alimentar a los rumiantes con productos de rumiantes continua.

Medidas de Control

Medidas implementadas
  • Tamizaje
  • Trazabilidad
  • Eliminación oficial de canales, subproductos y desechos de origen animal
  • Vacunación prohibida
  • Ningún tratamiento de los animales afectados
Medidas para implementar
  • Ninguna otra medida

Resultados de las pruebas diagnósticas

Nombre y tipo de laboratorio
Laboratorios de los servicios veterinarios nacionales (NVSL) ( Laboratorio nacional )
Pruebas y resultados
Especies
Prueba
Fecha de la prueba
Resultados
Bovinos
prueba inmunoenzimática (ELISA)
15/07/2017
Positivo
Bovinos
western blot
16/07/2017
Positivo

Informes futuros

El episodio ha sido resuelto. Ningún otro informe será enviado


TUESDAY, JULY 18, 2017 

USDA announces Alabama case of Bovine Spongiform Encephalopathy Alabama


TUESDAY, JULY 18, 2017 

MINK FARMING USA TRANSMISSIBLE MINK ENCEPHALOPATHY TSE PRION DISEASE SURVEILLANCE AND TESTING


MONDAY, NOVEMBER 30, 2009 

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE


MONDAY, JULY 17, 2017 

National Scrapie Eradication Program May 2017 Monthly Report Fiscal Year 2017


THURSDAY, JUNE 22, 2017 

World Organisation for Animal Health (OIE) to establish liaison office in College Station, Texas


THURSDAY, JULY 13, 2017 

EFSA BSE Sixty cases of mad cow disease since 2001 breached feed ban likely the cause 

Scientists investigate origin of isolated BSE cases

http://bovineprp.blogspot.com/2017/07/efsa-bse-sixty-cases-of-mad-cow-disease.html

THURSDAY, JULY 20, 2017 

USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589.200


First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress 

Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1 

University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen 

This is a progress report of a project which started in 2009. 21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves. 

Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice. 

At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation. 

PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS 

 Subject: PRION 2017 CONFERENCE DECIPHERING NEURODEGENERATIVE DISORDERS VIDEO

PRION 2017 CONFERENCE DECIPHERING NEURODEGENERATIVE DISORDERS

PRION 2017 CONFERENCE VIDEO



Chronic Wasting Disease CWD TSE Prion to Humans, who makes that final call, when, or, has it already happened?

TUESDAY, JUNE 13, 2017

PRION 2017 CONFERENCE ABSTRACT First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress


TUESDAY, JUNE 13, 2017

PRION 2017 CONFERENCE ABSTRACT Chronic Wasting Disease in European moose is associated with PrPSc features different from North American CWD


TUESDAY, JULY 04, 2017

*** PRION 2017 CONFERENCE ABSTRACTS ON CHRONIC WASTING DISEASE CWD TSE PRION ***


Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

Location: Virus and Prion Research

Title: Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease

Author item Moore, Sarah item Kunkle, Robert item Kondru, Naveen item Manne, Sireesha item Smith, Jodi item Kanthasamy, Anumantha item West Greenlee, M item Greenlee, Justin

Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 3/15/2017 Publication Date: N/A Citation: N/A Interpretive Summary:

Technical Abstract: Aims: Chronic wasting disease (CWD) is a naturally-occurring, fatal neurodegenerative disease of cervids. We previously demonstrated that disease-associated prion protein (PrPSc) can be detected in the brain and retina from pigs challenged intracranially or orally with the CWD agent. In that study, neurological signs consistent with prion disease were observed only in one pig: an intracranially challenged pig that was euthanized at 64 months post-challenge. The purpose of this study was to use an antigen-capture immunoassay (EIA) and real-time quaking-induced conversion (QuIC) to determine whether PrPSc is present in lymphoid tissues from pigs challenged with the CWD agent.

Methods: At two months of age, crossbred pigs were challenged by the intracranial route (n=20), oral route (n=19), or were left unchallenged (n=9). At approximately 6 months of age, the time at which commercial pigs reach market weight, half of the pigs in each group were culled (<6 challenge="" groups="" month="" pigs="" remaining="" the="">6 month challenge groups) were allowed to incubate for up to 73 months post challenge (mpc). The retropharyngeal lymph node (RPLN) was screened for the presence of PrPSc by EIA and immunohistochemistry (IHC). The RPLN, palatine tonsil, and mesenteric lymph node (MLN) from 6-7 pigs per challenge group were also tested using EIA and QuIC.

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

This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge.

CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease.

Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.


CONFIDENTIAL

EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY

While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...


we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.


 Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....


 snip...see much more here ;

WEDNESDAY, APRIL 05, 2017

Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease


TUESDAY, APRIL 18, 2017 

*** EXTREME USA FDA PART 589 TSE PRION FEED LOOP HOLE STILL EXIST, AND PRICE OF POKER GOES UP ***


TUESDAY, MARCH 28, 2017 

*** Passage of scrapie to deer results in a new phenotype upon return passage to sheep ***


SUNDAY, JULY 16, 2017

*** Temporal patterns of chronic wasting disease prion excretion in three cervid species ***


National Prion Center could lose all funding just as concern about CWD jumping to humans rises

SATURDAY, JULY 15, 2017 

National Prion Center could lose all funding just as concern about CWD jumping to humans rises


Some unofficial information from a source on the inside looking out -

Confidential!!!!

As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!

----------

You can take that with however many grains of salt you wish, and we can debate these issues all day long, but the bottom line, this is not rocket-science, all one has to do is some experiments and case studies. But for the life of me, I don't know what they are waiting on?

Kind regards,

Terry S. Singeltary Sr. Bacliff, Texas USA

More here:



http://collections.europarchive.org/tna/20080102173630/http://www.bseinquiry.gov.uk/files/ws/s018.pdf




more from deep throat;

DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)

The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"

again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.) 

SATURDAY, JULY 15, 2017 

National Prion Center could lose all funding just as concern about CWD jumping to humans rises




Date: February 4, 2004 at 10:53 am PST

Alabama man dies of Creutzfeldt-Jakob disease, Alabama does not have to report CJD

The Associated Press

A DeKalb County resident who died last year was diagnosed with a form of a rare illness sometimes linked to mad cow disease, but it was unclear how he got the infection.

Doctors determined that a man who died in November while under hospice care suffered from Creutzfeldt-Jakob disease, Coroner Tom Wilson said Tuesday. The disease was listed as the cause of death on the death certificate, he said.

Health officials said the illness kills a few people each year in Alabama, but there has never been any sign of a link with mad cow disease, which has drawn wide attention since the Dec. 23 announcement that a cow in Washington state had tested positive for it.

Wilson declined further comment and referred questions to New Beacon Hospice, which cared for the victim in DeKalb County. Mary Colley of New Beacon refused comment, citing patient confidentiality laws.

Officials with the DeKalb County Public Health Department and the area health office covering northeast Alabama said they were unaware of the case. Doctors are not required to report cases of Creutzfeldt-Jakob to the state.

WHNT-TV of Huntsville reported that the man was diagnosed with sporadic Creutzfeldt-Jakob disease, a designation given cases where the source of the infection was unknown.

Researchers believe there is a connection between mad cow disease, or bovine spongiform encephalopathy, and a variation of Creutzfeldt-Jakob disease, a fatal disorder that causes rapid dementia and loss of muscle control.

Sharon Thompson, a nurse with the epidemiology office of the Alabama Department of Public Health in Montgomery, said four to five people die each year in Alabama of Creutzfeldt-Jakob disease.

None of those deaths have been from the variant of the disease sometimes linked to mad cow disease, she said. "There are cases of it that occur naturally," said Thompson.

About one person in 1 million died of Creutzfeldt-Jakob annually in the United States from 1979 through 1994, according to statistics from the Centers for Disease Control and Prevention in Atlanta.


ALABAMA CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE REPORTING 

cjd tse prion disease is not reportable apparently...




TSS