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Cases Of (Mutated) Atypical
BSE and Atypical Scrapie

From Patricia Doyle, PhD
dr_p_doyle@hotmail.com
2-2-4



Hello, Jeff -
 
It is important to note that both Scarpie and BSE (Mad Cow Disease) have been found to have typical cases in France. Atypical BSE has also been found in Japan and Italy.
 
"This finding suggests either some phenotypic modifications of PrP(res) following infection by the BSE agent or the existence of alternative origins of such diseases in cattle."
 
My guess is that there is the existence of alternative origins.
 
Patricia Doyle
 
 
From ProMED-mail
 
Office International des Epizooties(OIE), Disease Information http://www.oie.int/eng/info/hebdo/a_info.htm
 
Emergency Report
 
Bovine Spongiform Encephalopathy in France - Atypical Cases
 
Translation of information received on 26 Jan 2004 from Dr Isabelle Chmitelin, Deputy Director General, General Directorate for Food (DGAL), Ministry of Agriculture, Food, Fisheries and Rural Affairs, Paris:
 
Date of the report: 16 Jan 2004.
 
Date of initial detection of animal health incident: 11 Jan 2004.
 
Six atypical cases of bovine spongiform encephalopathy (BSE) have been detected within the framework of the routine active surveillance programmes (abattoirs, downers).
 
Affected population: the 6 affected cows were aged from 8 years to 15 years.
 
Location of the cases: departments of Loire-Atlantique (2 cases), Cotes-d'Armor (1 case), Creuse (1 case), Loire (1 case) and Jura (1 case).
 
Control measures: the cohort of each affected cow was destroyed in accordance with Regulation No. 999/2001 of the European Parliament and of the Council of the European Union.
 
Diagnosis:
 
In each case, the electrophoretic profiles observed in the confirmatory tests were different from those observed in classical bovine spongiform encephalopathy cases (apparent molecular weight of PrPSc protein, glycoform distribution, detection using P4 monoclonal antibody.
 
Genetic analysis of several of these atypical cases has ruled out the hypothesis of a polymorphism in the gene coding for the prion protein.
 
Inoculation of samples into laboratory animals is currently under way to determine more accurately the biological characteristics of these isolates.
 
A. Laboratory where confirmatory testing was carried out: NCTA(1)-Virology Unit, French Agency for Food Safety (AFSSA(2)), Lyon.
 
B. Confirmatory test used: western blot (RB1 polyclonal antibody, P4 monoclonal antibody).
 
(1) NCTA: non-conventional transmissible agents
 
(2) AFSSA: Agence francaise de securite sanitaire des aliments
 
-- ProMED-mail <promed@promedmail.org>
 
[Atypical BSE in bovines has been recently recorded in Japan, Italy & France - - see refs. below. - Mod.AS]
 
References ---------- [1] Japan Yamakawa Y et al. 2003. Atypical proteinase K-resistant prion protein (PrPres) observed in an apparently healthy 23-month-old Holstein steer. Jpn. J. Infect. Dis. 56: 221-222.
 
[2] France Biacabe AG, Laplanche JL, Ryder S, Baron T. AFSSA-Lyon, Unite 'Virologie- ATNC', 31 Avenue Tony Garnier, 69364 Lyon Cedex 07, France. Distinct molecular phenotypes in bovine prion diseases. EMBO Rep. 2004 Jan;5(1):110-115
 
Bovine spongiform encephalopathy (BSE) in cattle, the most likely cause of variant Creutzfeldt-Jakob disease in humans, is thought to be caused by a unique infectious agent, with stable features, even when transmitted to other species. Here, we show the existence of an atypical molecular phenotype among cattle diagnosed with BSE in France. Following western blot analysis, three cases showed unusual features of the electrophoretic profiles of the protease-resistant prion protein (PrP(res)) accumulating in the brain. The PrP(res) patterns were similar in these three atypical cases, showing a higher molecular mass of unglycosylated PrP(res) and strong labelling by P4 monoclonal antibody compared to 55 typical BSE cases. This finding suggests either some phenotypic modifications of PrP(res) following infection by the BSE agent or the existence of alternative origins of such diseases in cattle.
 
SCRAPIE, ATYPICAL, SHEEP - FRANCE: OIE ************************************** Date: 31 Jan 2004 From: ProMED-mail <promed@promedmail.org> Source: Office International des Epizooties (OIE), Disease Information
 
http://www.oie.int/eng/info/hebdo/a_info.htm
 
 
Emergency Report
 
Scrapie In France - Atypical Cases
 
Translation of information received on 26 Jan 2004 from Dr Isabelle Chmitelin, Deputy Director General, General Directorate for Food (DGAL), Ministry of Agriculture, Food, Fisheries and Rural Affairs, Paris:
 
Date of the report: 16 Jan 2004.
 
Date of initial detection of animal health incident: 12 Mar 2003 [10 months ago - Mod.JW].
 
3 atypical cases of scrapie have been detected in ewes aged from 5 years to 17 years. Genotyping of the animals for the gene coding for the prion protein has shown that these animals are homozygous for the allele ARR, associated with the highest resistance to the development of clinical signs of scrapie.
 
Location of the cases: departments of Haute-Vienne (2 cases) and Ain (1 case).
 
Diagnosis: the cases were detected within the framework of the routine active surveillance programmes (abattoirs, downers) implemented in accordance with Regulation No. 999/2001 of the European Parliament and of the Council of the European Union.
 
A. Laboratory where confirmatory testing was carried out: NCTA(1) Virology Unit of the French Agency for Food Safety (AFSSA(2)), Lyon.
 
B. Diagnostic tests used: ELISA(3) and immunohistochemical tests. Inoculation of samples into laboratory animals is currently under way to determine more accurately the biological characteristics of these isolates.
 
(1) NCTA: non-conventional transmissible agents (2) AFSSA: Agence francaise de securite sanitaire des aliments (3) ELISA: enzyme-linked immunosorbent assay -- ProMED-mail <promed@promedmail.org>
 
{The following comment is adapted from the 2003 ProMED reference below.] [With new information, it appears there is potentially a new phenotype of 'atypical' BSE/TSE in animals in Japan and France. The article below indicates a new strain of Scrapie from Norway, the 'Nor98', which also has negative IHC and histology. Although this article does not suggest a link to the atypical forms seen in cattle, it does seem more than coincidence that there are also appearing different strains, or perhaps atypical strains of scrapie as well. It has been believed for years that there is a link between BSE and scrapie; perhaps this is another bit of research that should be carefully examined. -
 
Ref: Cases of scrapie with unusual features in Norway and designation of a new type, Nor98. Benestad S.L.; Sarradin P.; Thu B.; Schonheit J.; Tranulis M.A.; Bratberg B. The Veterinary Record, 16 August 2003, vol. 153, no. 7, pp. 202- 208(7) - Mod.TG]
 
Patricia A. Doyle, PhD Please visit my "Emerging Diseases" message board at: http://www.clickitnews.com/ubbthreads/postlist.php?Cat=&Board=emergingdiseases Zhan le Devlesa tai sastimasa Go with God and in Good Health

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