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Date Submitted: 06/20/2016 01:55 PM
NATIONAL CENTER FOR CASE STUDY TEACHING IN SCIENCE
Holes in the Matter: A Case of Prion Disease
by
Tonya Laakko Train
Department of Biology
Elon University, Elon, NC
Part I – Symptoms
When university president, George Caspase, hung up the phone, he had a very worried look on his face. Another
alumnus had passed away. It was the second time that the Centers for Disease Control and Prevention (CDC) had
called in the past week. Henry Proton, age 26, Emily Reticulum, age 25, and Frank Spongiform, age 26, had died in
the past year. The loss of these young people was tragic enough, but it was the way they died that had the President’s
mind reeling and the CDC concerned.
Henry was a sportscaster, husband, and father of an infant son. He was outgoing, smart, and seemed to have a bright
future. That spring he began to feel depressed. His wife talked him into seeing a psychologist. He was put on an antidepressant and for awhile it seemed to help, but in a couple of weeks the depression was back and he began hallucinating.
Later that fall, Henry’s movements became more rigid, and it became clear that the problem was more than psychological and that they had better see a neurologist. After months of testing and progressive deterioration of his physical
movements, his doctors still did not have a diagnosis. Henry passed away one week before his son’s first birthday. An
autopsy was ordered; it was discovered that Henry’s brain was filled with holes and a call was made to the CDC.
The stories were similar for Emily and Frank, and it did not take long for Dr. Annie Isomerase, an infectious disease
specialist at the CDC, to determine that these cases were due to a transmissible prion disease. It also did not take long
to determine that these cases had another connection: All of the affected individuals attended the same university at
the same time.
Questions
1. Which prion disease was the most likely to have been contracted by the people in this story?...