Atypical cases

Emergency report

Translation of information received on 26 January 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 January 2004.

Date of initial detection of animal health incident: 12 March 2003.

Three 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 française de sécurité sanitaire des aliments

(3) ELISA: enzyme-linked immunosorbent assay

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BOVINE SPONGIFORM ENCEPHALOPATHY IN FRANCE
Atypical cases

Emergency report

Translation of information received on 26 January 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 January 2004.

Date of initial detection of animal health incident: 11 January 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 six affected cows were aged from 8 years to 15
years.

Location of the cases: departments of Loire-Atlantique (2 cases),
Côtes-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 française de sécurité sanitaire des aliments