Mad Cow Disease Case Study

INSTRUCTIONS: View the Nova Video on Mad Cow disease and read the section on spongiform encephalopathies in the Control of Communicable Diseases handbook (Benenson, 1990). Then answer the following questions.

Acronyms: CJD = Crutzfeldt-Jacob Disease; BSE = Bovine Spongiform Encephalopathy; PrP = Prion Protein; nvCJD = New Variant Crutzfeldt-Jacob Disease

Questions:

(1) Name 3 human spongiform encephalopathies with known prion agents.

(2) Name 3 animal spongiform encephalopathies with known prion agents.

(3) How is Kuru transmitted?

(4) Why did only women and children in the Foray tribe get Kuru (i.e., why were men spared)?

(5) How is BSE transmitted?

(6) Epidemiologists often speak of the person, place, and time variables. What is the typical age of onset for CJD? What is the typical age of onset for nvCJD? Does this imply that a different agent is involved?

(7) What is the portal of entry for the BSE agent?

(8) What is PrP? What types exist, and how do they differ?

(9) What makes prions unique as infectious disease agents?

(10) How are prions able to replicate? Describe the "protein only" theory.

(11) Why are prion so difficult to sterilize via heat, chemicals, and radiation?

(12) Who won the 1997 Nobel Prize for medicine, and what did he win it for?

(13) List 3 clinical differences between CJD and nvCJD.

(14) Why do pathologists who autopsy CJD cases wear metal gloves?

(15) How many British people have died of nvCJD / presumed Mad Cow disease?

(16) How many American people have died of nvCJD?

(17) If nvCJD is indeed Mad Cow Disease in humans, and nearly all British people have been exposed, why have there been so few cases to date?

(18) Would you eat British beef?

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