Transmissible Spongiform Encephalopathies (TSE) D. Heim

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1 Transmissible Spongiform Encephalopathies (TSE) D. Heim

2 What are TSE? Family of diseases Neurological disease accumulation of abnormal prion protein spongiform degeneration of the brain progressive and lethal long incubation period transmissible

3 Which diseases are belonging to TSE? Variant Creutzfeldt-Jakob-Disease Feline Spongiform Encephalopathy Bovine Spongiform Encephalopathy Chronic Wasting Disease Transmissible Mink Encephalopathy Gerstmann-Straeussler- Scheinker-Syndrom Creutzfeldt-Jakob-Disease Kuru Scrapie year

4 Which diseases are belonging to TSE? Variant Creutzfeldt-Jakob-Disease Feline Spongiform Encephalopathy Bovine Spongiform Encephalopathy Chronic Wasting Disease Transmissible Mink Encephalopathy Gerstmann-Straeussler- Scheinker-Syndrom Creutzfeldt-Jakob-Disease Kuru Scrapie year

5 Which diseases are belonging to TSE? Variant Creutzfeldt-Jakob-Disease Feline Spongiform Encephalopathy Bovine Spongiform Encephalopathy Chronic Wasting Disease Transmissible Mink Encephalopathy Gerstmann-Straeussler- Scheinker-Syndrom Creutzfeldt-Jakob-Disease Kuru Scrapie year

6 Which diseases are belonging to TSE? Variant Creutzfeldt-Jakob-Disease Feline Spongiform Encephalopathy Bovine Spongiform Encephalopathy Chronic Wasting Disease Transmissible Mink Encephalopathy Gerstmann-Straeussler- Scheinker-Syndrom Creutzfeldt-Jakob-Disease Kuru Scrapie year

7 Only a few veterinarians in a few regions knew animal TSEs (scrapie) Disease awareness veterinarians low Only a few physicians knew human TSEs Disease awareness physicians low

8 Bovine spongiform Encephalopathy (BSE) in the United Kingdom ( MAD COW DISEASE )

9 Origin of BSE in UK

10 Origin of BSE in UK Insufficient sterilisation of meat and bone meal

11 Origin of BSE in UK Insufficient sterilisation of meat and bone meal Use of meat and bone meal in cattle feed

12 Origin of BSE in UK Insufficient sterilisation of meat and bone meal Use of meat and bone meal in cattle feed

13 Origin of BSE in UK Insufficient sterilisation of meat and bone meal Use of meat and bone meal in cattle feed BAN OF FEEDING MBM TO RUMINANTS

14 First BSE cases in Ireland Portugal Switzerland France

15 Export meat and bone meal from UK

16 BSE and Variant Creutzfeldt-Jakob- Disease (vcjd) - the link

17 Reaction of the public Media hype Public crisis Massive decrease beef consumption

18 Sporadic Human TSE Classical sporadic Creutzfeldt-Jakob-Disease Known since 1920 incidence of sporadic cases worldwide similar (0.3-2 cases per 1 Million inhabitants per year) Mainly older patients Hereditary Familiar CJD Gerstmann-Sträussler-Scheinker-syndrome Fatal familiar insomnia Acquired Kuru Iatrogenic CJD Variant CJD (vcjd)

19 Variant CJD Different from sporadic CJD Younger age Different clinical signs Different pathology Most probable source of infection: consumption of meat products containing CNS

20 Which tissues are infectious in BSE- cattle? Main infectivity in Brain Spinal cord Dorsal root ganglia Distal ileum

21 Crucial measure to minimize risk for consumer Ban of specified risk material (brain,...) in human food United Kingdom 1989 Switzerland 1990 European Union 2000 (some EU-countries with BSE before)

22 Reaction of the countries Countries with BSE: Most implementation of more rigorous measures Contries without BSE Ban of imports from countries with BSE we are BSE-free Countries with surveillance found BSE disadvantages Countries without surveillance did not find BSE advantages

23 How to find out whether there is a risk of having BSE? World Organisation for Animal Health BSE risk status can only be determined after assessing BSE risks

24 GBR I: Highly unlikely Risk assessment EU Argentina, Australia, Iceland, New Caledonia, New Zealand, Panama, Paraguay, Singapore, Uruguay, Vanuatu GBR II: Unlikely but not excluded GBR III: Likely but not confirmed or confirmed at a lower level GBR IV: Confirmed at a higher level Botswana, Brazil, Colombia, Costa Rica, El Salvador, India, Kenya, Mauritius, Namibia, Nicaragua, Nigeria, Norway, Pakistan, Sweden, Swaziland Albania, Andorra, Austria, Belarus, Belgium, Bulgaria, Chile, Croatia, Denmark, Canada, Cyprus, Czech Republic, Estonia, Finland, Former Yugoslav Republic of Macedonia, France, Germany, Greece, Hungary, Ireland, Israel, Italy, Latvia, Lithuania, Luxembourg, Malta, Mexico, Poland, The Netherlands, Romania, San Marino, Slovak Republic, Slovenia, South Africa, Spain, Switzerland, Turkey, USA United Kingdom, Portugal

25 Improvement of surveillance Passive surveillance (notification by owner/veterinarian) Disease awareness Willingness to notify cases Laboratory competence Active surveillance: testing of risk populations Switzerland no. of cases France 2000 Year of diagnosis France EU 2001

26 First cases detected in 2000/2001 Denmark Spain Germany Italy Czech Rep Greece Slovak Rep Slovenia Austria Finland

27 vcjd in the UK PREDICTIONS Less than 870,000 (Ghani et al, 2000) Few hundred-millions (d'aignaux et al 2001)

28 Reaction of the public Media hype Public crisis Massive decrease beef consumption

29 2001: Japan 1.BSE case outside Europe

30 2003: Canada

31 2005: USA

32 Source: FAO

33 BSE-situation Year of diagnosis Number of BSE-cases Number of BSE-cases (UK) United Kingdom (UK) TOTAL EU 15 without UK Japan Year of diagnosis No of cases Canaday Year of diagnosis No of cases

34 30 25 vcjd-situation UK cases (+1) vcjd-cases blood transfusion associated 2 (+1) with clinical signs 1 case without clinical signs Human to human transmission very effective 0

35 vcjd-situation in other countries France 21 Ireland 4 Spain 1 Portugal 1 Netherlands 2 Italy 1 Japan 1 Canada 1 USA 3

36 Conclusion BSE and vcjd-cases decreasing measures taken effective Efficient surveillance needed Control of implemented measures needed Many countries without reported BSE - risk is unknown Risk assessment needed Risk communication important

37 Thank you

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