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CJD Overview Bob Will, National CJD Surveillance Unit, Edinburgh, UK

CJD Overview Bob Will, National CJD Surveillance Unit, Edinburgh, UK Associazione Italiana Encefalopatie da Prion Milano 3 Ottobre 2009. Science 1968. Questions. What is the origin of infection in CJD? Is there a link to scrapie in sheep?

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CJD Overview Bob Will, National CJD Surveillance Unit, Edinburgh, UK

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  1. CJD Overview Bob Will, National CJD Surveillance Unit, Edinburgh, UK Associazione Italiana Encefalopatie da Prion Milano 3 Ottobre 2009

  2. Science 1968

  3. Questions • What is the origin of infection in CJD? • Is there a link to scrapie in sheep? • What are the clinical and pathological characteristics of CJD? • What are the epidemiological characteristics of CJD?

  4. SYSTEMATIC STUDIES OF CJD WORLDWIDE

  5. DISTRIBUTION OF SPORADIC CJD IN THE UK: 1990-2002

  6. SIGNIFICANT RISK FACTORS IN CONTROLLED STUDIES

  7. HUMAN TSEs (Prion diseases

  8. 60-80% SENSITIVITY (TESTING POLICY) ? SPECIFICITY (? 74%) (CONTEXT DEPENDENT) ‘SUBJECTIVITY’ OF REPORTING NO EEG CRITERIA PROSPECTIVELY VALIDATED IN LARGE NUMBERS OF CASES SPORADIC CJD : EEGPERIODIC TRIPHASIC DISCHARGES

  9. 14-3-3 Western Blot sCJD AD vCJD SpCJD CSF Analysis Dr Alison Green, The National CJD Surveillance Unit ECDC funded meeting, 10th March 2009

  10. MRI brain scan in sporadic CJD

  11. MRI brain scan in variant CJD

  12. IATROGENIC CREUTZFELDT-JAKOB DISEASE WORLDWIDE

  13. DURA MATER CASES WORLDWIDE SHOWN BY YEAR OF OPERATION AND YEAR OF ONSET OF SYMPTOMS FOR CJD Mean incubation period from operation to onset of symptoms: 6.8 years (range 1-16)

  14. THE HUMAN PRION PROTEIN GENE Mutations and polymorphisms • Clinical diagnosis • Screening of family members • Pre-natal testing • Influence on phenotype

  15. The UK BSE epidemic ‘BSE posed the greatest political and economic challenge to the EU since its foundation’ The specified bovine offal ban UK Dec 1989/ Jan1990

  16. DIFFERENCES BETWEEN SPORADICAND VARIANT CJD

  17. AGE AT DEATH FOR SPORADIC CJD CASES AND vCJD CASES BY 5-YEAR AGE GROUP Number of cases Age Group

  18. ResultsTemporal distribution of vCJD cases in France and UK According to the year of onset, the number of vCJD cases in France reached a peak of incidence in 2004, five years after the peak observed in the UK in 1999

  19. YEAR OF ONSET OF ILLNESS OF vCJD WORLDWIDE

  20. CHARACTERISTICS OF TSEs • Prolonged incubation periods. • Uniformly fatal neurological diseases. • Causal agents (prions) resistant to sterilisation. • No serological test for infection. • Infection may be present in tissues (LRS) during the incubation period.

  21. vCJD case (Case 1) Donor onset Donor death Donation (RBC) Transfusion to recipient Recipient onset Recipient death vCJD case (Case 3) Donor onset Donor death Donation 1 (RBC) Transfusion to recipient Recipient onset Recipient death vCJD case (Case 4) Donor onset Donor death Donation 2 (RBC) Transfusion to recipient Recipient onset Recipient death Pre-clinical infection (Case 2) Donor onset Donor death Donation (RBC) Transfusion to recipient Recipient death Years shown by quarter RBC=red blood cells

  22. There is no evidence of transmission of any form of CJD through: • Social contact • Treating minor injuries • Occupational contact • Maternal transmission • Sexual transmission • General surgery

  23. EUROCJD & NEUROCJD Joint Meeting Lake Garda, Italy May 2003

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