Crutzfelt-Jakobs Disease Flashcards
What is a prion?
A prion is an abnormal protein that can trigger normal proteins in the brain to fold abnormally.
How do prions affect normal proteins in the brain?
Prions cause normal proteins to misfold, leading to damage in brain tissue.
Are prions infectious agents like bacteria or viruses? Why or why not?
No, prions are not like bacteria or viruses. They are just proteins and do not contain any infectious material like bacteria or viruses.
What are the main types of Creutzfeldt-Jakob Disease (CJD)?
The main types of CJD are:
Iatrogenic CJD (iCJD)
Sporadic CJD (sCJD)
Familial CJD (fCJD)
Variant CJD (vCJD)
What is iatrogenic CJD (iCJD), and how is it transmitted?
Iatrogenic CJD (iCJD) is transmitted accidentally through medical procedures, such as contaminated surgical instruments, corneal transplants, exposure to infected brain tissue, or blood transfusion
What is the typical cause of sporadic CJD (sCJD)?
Sporadic CJD occurs without any known trigger, often due to a spontaneous prion mutation, typically around age 60.
How is familial CJD (fCJD) inherited?
Familial CJD is inherited through a family history of prion disease, caused by a genetic mutation.
What is variant CJD (vCJD), and how is it contracted?
Variant CJD is caused by consuming meat from cows infected with Bovine Spongiform Encephalopathy (BSE), also known as Mad Cow Disease.
Why is there no treatment for CJD?
There is no treatment for CJD because it is caused by prions, which are resistant to most conventional medical treatments.
Name two common symptoms of CJD.
Common symptoms include rapid onset of dementia and difficulty walking.
What are the three main symptom sets observed in CJD patients?
Set 1: Difficulty sleeping, fatigue, and decreased appetite.
Set 2: Neurological issues, memory loss, behavioral changes, and confusion.
Set 3: Focal signs, cerebral ataxia, language disorders, visual disturbances, and motor weakness.
How can cerebrospinal fluid (CSF) analysis help in diagnosing CJD?
CSF analysis can show elevated protein levels, which may indicate prion disease.
When is a brain biopsy typically performed in relation to CJD?
A brain biopsy is typically done post-mortem (after death) for a definitive diagnosis.
How can prions be transmitted to humans?
Prions can be transmitted through exposure to contaminated brain or spinal cord tissue via wounds, injections, or consumption of contaminated meat.
What is the average survival time for a patient with classic CJD?
The average survival time for a patient with classic CJD is 4–5 months.
What is the average survival time for a patient with variant CJD?
The average survival time for a patient with variant CJD is 13–14 months.
Why are prions resistant to standard sterilization techniques?
Prions are resistant to standard sterilization because they do not contain genetic material like bacteria or viruses, and they are incredibly stable and tough to break down.
What should be done with instruments that may have been contaminated by prions?
Instruments contaminated with prions should not be reprocessed and must be disposed of to prevent further contamination.
What are the major risk factors for developing CJD?
Major risk factors include genetics (family history of prion disease), age (sporadic CJD typically develops around age 60), and exposure to contaminated tissue (iatrogenic CJD and variant CJD from consuming infected meat).
