Historical Contexts
This prion disease common in animals was first recognized in Colorado in the late 1960s.
What is CWD?
This prion disease is characterized by rapidly progressive dementia and is commonly found in older adults.
What is Creutzfeldt-Jakob disease?
This is what defines a prion as a prion.
What is a misfolded protein?
This prion disease is the most common form, accounting for around 85-90% of cases worldwide.
What is sporadic Creutzfeldt-Jakob Disease (sCJD)?
This antibiotic, along with tetracycline, has been tested for its effects on prion-infected mice, but clinical trials showed no survival benefit in humans.
What is doxycycline?
BSEs were first discovered in what decade?
What is the 1980s?
This hereditary prion disease causes severe insomnia, autonomic dysregulation, and ultimately leads to death within a few months to a few years after the onset of symptoms.
What is Fatal Familial Insomnia (FFI)?
This term refers to prion diseases that are inherited through mutations in the prion protein gene (PRNP)
What are familial prion diseases?
Inherited prion diseases, such as familial CJD and fatal familial insomnia, account for approximately this percentage of all prion disease cases globally.
What is 10-15%?
This antimalarial drug was tested in both the UK and the USA for treating prion diseases but showed no survival benefit in clinical trials.
What is quinacrine?
The first recognized case of Creutzfeldt-Jakob disease (CJD) was documented in this year, highlighting its impact on human health.
What is 1920?
This prion disease primarily affects sheep and is characterized by behavioral changes, loss of wool, and eventual death.
What is scrapie?
This symptom refers to the gradual decline in cognitive function and memory often seen in prion diseases.
What is dementia?
This many cases of vCJD were reported in the United States as of 2020, all of which are believed to have been acquired from exposure in other countries.
What is 4?
This type of care is provided to manage symptoms and improve quality of life.
What is palliative care?
In 1996, this variant of Creutzfeldt-Jakob disease was linked to the consumption of infected beef, leading to widespread public health concerns.
What is variant Creutzfeldt-Jakob disease (vCJD)?
This rare prion disease is characterized by dementia, ataxia, and is associated with mutations in the prion protein gene.
What is Gerstmann-Sträussler-Scheinker syndrome?
This term describes the way prion diseases can be spread through contaminated tissue or surgical instruments.
What is horizontal transmission?
The average duration of illness for sporadic CJD from the onset of symptoms to death is this many months.
What is 4-6 months?
This human antibody, derived from a mouse version, binds tightly to prion proteins and has shown the potential to cure prion-infected mice in experimental models.
What is PRN100?
Who published the first document detailing the concept of prions?
Who is Stanley Pruisner? (1982)
This terminology describes prion diseases and is known for its long incubation period which can lead to spongiform changes in the brain tissue upon autopsy.
What is a transmissible spongiform encephalopathy (TSE)?
These insoluble protein aggregates are characterized by a beta-sheet structure and are associated with several neurodegenerative diseases, including Alzheimer’s and Parkinson’s.
What are amyloid fibers?
Fatal familial insomnia (FFI) has an average life expectancy of this many months after symptoms first appear.
What is 12-18 months?
Although not proven to stop the progression of prion diseases, this semi-synthetic drug, originally derived from beech wood, has been given to patients in the UK via direct brain infusion.
What is pentosan polysulphate (PPS)?