Brittany Garcia HSCI 451 Case Study #1 1.

Which prion disease was the most likely to have been contracted by the people in this story? Justify your choice. Creutzfeldt-Jakob disease because the symptoms match: hallucinations, rigid posture, holes in brain tissue where nerve cells have died. 2. Propose a mode of infection. Ingestion; possible consumption of contaminated beef. 3. Are the family members of the affected individuals at risk? Why or why not? No, CJD can be transmitted through surgical procedures or consumption of contaminated beef but oftentimes, to get CJD, the individual must have three factors to become infected. These factors include being exposed to the prion; have the specific genetics (10-15% of cases are inherited); and being exposed to organophosphate which is found in pesticides. As long as the family members did not consume the same contaminated beef as the infected individual and does not have all of the above factors, they should not be at any risk. 4. If you were the university president, who would you inform and what would you tell them? I would try to get the histories of all the individuals that were infected and try to find something that connected them all. From there, I would inform other peers that were with them during the time of infection. I would tell them to be aware that they may have CDJ as well if they consumed the same contaminated beef. I would also locate the place that distributed the contaminated meat and notify and report them. 5. Would early intervention help any others who might be infected? If so, what possible treatments could be implemented? There are no treatments for CJD. There are several studies that are looking at possible ways to slow down the prions progression, but no definitive results have been found. 6. What was a likely mode of transmission of this prion disease? Ingestion; ate meat contaminated with BSE. 7. Is it possible that other members of the study abroad trip could be infected? Why or why not? Yes, if they ate the same foods as those who got infected, they may also be infected. 8. Disease-causing prions are a unique infectious agent. What makes this type of infection unique? The prion protein converts normally shaped proteins to the disease-causing protein. This process is fatal and no successful treatments exist to slow or stop the lethal prion proteins.

Kuru Location of occurrences New GuineaFore tribe

CJD Worldwide; sporadic, acquired or hereditary

# of cases

1957-2004 total of 2,700 cases 2003-2008 2 deaths 1996-2004 11 cases 55 years old Range: 5-60 years

1-2 cases per million people 279-352 cases/year

Average age of onset

65 years Range: 20-70 years

Symptoms

Headache, joint pains, difficulty walking, tremor, difficulty swallowing/eating 5-50 years

Dementia, hallucinations, confusion, lack of coordination, muscle twitching & stiffness, speech impairment 15 mo.-50 years

Incubation period (time from acquisition to symptom onset) Duration of illness (symptom onset to death)

Can be up to 30 years or longer, death usually occurs 3 mo.-2 yrs. within the symptom onset New Guinea; cannibalistic ingestion of infected human brain

4.5 months

Origin of disease

United Kingdom; cows were fed remains of cows in their feed