Question

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 antide- pressant 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 psychologi- cal 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? Justify your choice.

2. Propose a mode of infection.

3. Are the family members of the affected individuals at risk? Why or why not?

4. If you were the university president, who would you inform and what would you tell them?

5. Would early intervention help any others who might be infected? If so, what possible treatments could be

implemented?

Answer 1

1. Which prion disease was the most likely to have been contracted by the people in this story? justify your choice?

Ans. Creutzfeldt-Jakob disease(CJD), one of the types of prion disease was contracted by the people in this story. Autopsy that was done after Henry's death declared that there were holes in his brain and this observed in CJD. Creutzfeldt-Jakob disease is a rare neurodegenerative disease that destroys brain cells and causes holes in it.

2. Propose a mode of infection?

Prion disease is cause by prion protein which belong to the class of amyloid proteins. It is a membrane associated protein that is present in most of the eukaryotes. Prion disease occurs when the prion proteins are folded in wrong way resulting in a prion which causes prion disease. prions can also trigger the misfolding of normal proteins as well. The prions that were formed silently spread in the brain affecting the brain cells. Most of the times symptoms are not observed in any individual. Prion disease occurs in both animals and humans. Creutzfeldt-Jakob disease ( CJD) is the most common forms of prion disease affecting humans.

CJD is found to be transmitted from one person to another but not through casual contact or sexual contact with the infected person. It is found to be transmitted when the person is exposed to infected brain during medical procedure. It can also be transmitted when a person eats meat which is from an animal that already has the disease. CJD form of disease in animals is called mad cow disease.

3. Are the family members of the affected individual are at risk? why or why not?

Ans. Yes the family members of the affected individuals are at risk because about fifteen out out of hundred cases were found to be inherited with CJD . Prion disease is caused by a faulty gene which can be passed on from parent to the child and run in the family.

4. If you were the university president, who would you inform and what would you tell them?

If i were the university president i would share this news with existing students. I would tell them that we had a great loss through the death of three brilliant ex-students who were affected with CJD. I would give a suggestion to the students to check if any one in their family was affected with CJD, as it is heriditary.

5. would early intervention help any others who might be infected? if so what possible treatments could be implemeted

A person likely to be experiencing symptoms of CJD is subjected to diagnosis which includes few brain examinations. A brain biopsy is carried out and the sample of brain tissue collected is studied by a neuropathologist. There is no proper cure for CJD but yet we can use opiate drugs to relieve pain and ease the symptoms. Myoclonus symptoms can be treated with sodium valproate and clonazepam.