The Pathological Protein, Chapters 8-9

1. The Pathological Protein, Chapters 8-9 Rhiannon Aguilar HONR299J March 27, 2014

2. Chapter 8: Consuming Fears Introduction • Peter Stent’s cow 133- Dec 1984 • David Bee, veterinarian-- kidney infection? toxin? • Connection to scrapie- Nov 1986- Gerald Wells • Confidential government memo Tracking the Source • John Wilesmith, head of epidemiology at CVL • Joined team doing investigation • Transmissibility confirmed 1988 • Familiar statistics • “All appeared to be index cases”- had MBM in common

3. Chapter 8, Part II Forced Cannibalism • OPEC raised oil prices, encouraged efficiency • Rendering- process around for centuries • Offal, downer cows, pets, roadkill • Solvent extraction vs centrifugation • Scrapie transfer to cows through rendered meat? • Lacey/Dealler- not likely • Taylor- solvent extraction does not destroy agent • Reduced 10x infectivity- threshold amount? • Gabriel Horn 2001 • 7/10 calves die from 1g infected brain

4. Chapter 8, Part III Tackling an Epidemic • Sir Southwood’s low-key “working party” • 1988 ruminant feed ban, 1994 mammalian protein • Lots of skepticism on all sides, little urgency • Mandated slaughter, 50% compensation • Final report, 1989 • “We wanted to point out that there were enormous uncertainties. And that if these uncertainties turned out to be more likely than we had judged there could be catastrophic and very profound consequences” • BSO ban, import restrictions

5. Chapter 8, Part IV Mad Max • 5 y/o siamese cat died 1990 w/ TSE symptoms • Public freak-out! • Wake-up call • 1990 House of Commons investigation • Formed SEAC • Species jump confirmed The Watcher • CJD Surveillance unit, Robert Will • ~½ of investigated cases not CJD • “People forget, prion diseases in animals had never caused any problems in humans as far as we knew, despite extensive exposure” • Different take on Vicky Rimmer

6. Chapter 8, Part V Approaching the Watershed • Back to the Churchills- Narang’s BSE test? • Suddenly many young CJD victims- statistically significant- 10 cases 1994-1996 • 1995- realized 1989 offal ban was ineffective, new ban • “Floral” plaques • March 20, 1996- Stephen Dorrell announcement • Disease naming? • Inquiry, 1998-2000 • Hindsight is 20/20

7. Florid Plaques “The first case of variant Creutzfeldt-Jakob disease in the Netherlands”, Sánchez-Juan, P. et. al., Journal of Neurology 2007. A. florid plaque surrounded by small areas of spongiform change in the occipital cortex (Luxol-PAS stain). B. Multiple small cluster plaques in the occipital cortex (Luxol-PAS stain). C. kuru-type plaque in the cerebellar cortex (Luxol-PAS stain). D. Immunohistochemistry for prion protein (using monoclonal antibody 3F4) shows strong staining of plaques in the occipital cortex, along with amorphous and pericellular deposits

8. Chapter 9: Mad Cow’s Human Toll Introduction • 1992/1993- Epidemic height • BAB and BARB cows concerning Calculating Mortality • No exact proof that BSE causes vCJD • Try to calculate # of cows with BSE • Anderson/Donnelly back-calculation parameters • Study to determine most risky organs -> more bans • Investigating features of vCJD- incubation time, victim characteristics, necessary dose, genetics

9. Chapter 9, Part II Mad Sheep Disease? • Origin- sheep or beef? • Botched study of scrapie and BSE-sensitive mice Spreading the Madness • Hilary Pickles- import/export worries • BSE spread from UK • Japan - > portrayed differently than last book • 92% M/M, means vCJD epidemic would be worse? • Dec 2002, 22 countries with BSE • Banning more risky (?) practices

10. Chapter 9, Part III • Practice of bans is spotty • Lasting economic impacts • Developing countries probably have BSE… • Category rating system • Category I - IV • USA = Cat II

11. http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/en-bse-carte/http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/en-bse-carte/ World Organisation for Animal Health