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By Carmen Ferreiro

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The existence of strains could be the reason why scrapie has received different names in different countries and at different times in history and why it took so long for scientists to recognize the different pathologies caused by CJD in humans as a single disease. q 58 3/10/08 11:11 AM Page 58 MAD COW DISEASE (BOVINE SPONGIFORM ENCEPHALOPATHY) The possibility that the scrapie agent had different strains was suggested in the 1950s by Iain Pattison and Geoffrey C. 3 Pattison and Millson inoculated goats with brain tissue from scrapieinfected sheep.

Q 64 3/10/08 11:12 AM Page 64 MAD COW DISEASE (BOVINE SPONGIFORM ENCEPHALOPATHY) The TSE agent was not destroyed by freezing or by boiling (100° C [212° F] for 30 minutes) the samples that contained it, nor by dry-heating the samples at 600° C (1,112° F). The same team exposed scrapie-infected brain tissue in water to ultraviolet (UV) light (254 nanometers). UV light of this wavelength causes nucleic acids to break apart. Yet it had no effect on the infectivity of the TSE samples. More impressive still: Blasting scrapie-infected tissue with high-energy beams of electrons (an elemental particle with a negative electrical charge) didn’t destroy its infectivity either.

The people affected also developed crossed eyes and emotional instability, and although they seemed to be mentally aware until the end, they were unable to speak. Eventually, they couldn’t even swallow and wasted away until they died, usually of pneumonia or other opportunistic infections. There was no cure for kuru. After the symptoms appeared, adults died in about 12 to 18 months; children in about 3 to 12 months. Until as late as the 1950s, the Fore still lived in the Stone Age. They had not yet discovered the wheel, and, unaware of metals, they made their tools and weapons from bone or stone.

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