Dr. Jim Collins

Mad Cow Disease and Mad Scientists

Topics

Kuru, 1920s, and sorcery among the New Guinea Fore people

Carleton Gadjusek M.D. and the discovery of brain lesions

Not a psychiatric disorder

1960’s –“It looks exactly like Scrapie in sheep” William Hadlow

What is Scrapie?

A family of diseases

“Prions” – named by Stanley Prusiner, Nobel Laureate

Incredible properties of prions

Bovine Spongioform Encephalopathy – BSE

“There is no evidence that BSE can be transmitted to humans” – famous words!

Origin questions

TSEs – mink, cats, zoo ungulates

Human TSEs: sporadic, inherited, and transmitted

Chronic Wasting Disease of Deer and Elk

Questions

1. Discuss the three ways that a spongioform encephalopathy can arise in an individual.

2. If you suspected that a laboratory bench was contaminated with prions, how would you disinfect it?

3. Why do you think BSE is still arising in other parts of the world, like Japan, last year?

4. Why might there still be a possible epidemic of vCJD?

Case: Are you prepared to explain why we are not at risk from this case?

First case of vCJD in the US

A 22-year-old British woman living in Florida is believed to have a brain illness linked to mad cow disease, the first known case in the United States, health officials declared recently.

- An official from the Centers for Disease Control and Prevention emphasized there was every reason to suspect that she represented no risk to others in the US.

- Officials with the Florida Department of Health emphasized that there is no reason to suspect cattle in the United States have the cow version, bovine spongiform encephalopathy

- A state Health Department spokesman has reassured the public that there is no evidence to suggest her illness poses a threat to anyone else or the agriculture industry