Prions Flashcards
1
Q
Prion
A
- misfolded proteins that aggregate in CNS and cause neurodegeneration
- exists in normal isoforms that may transform to pathogenic
2
Q
Kuru
A
- discovered in Fore tribe in remote highlands of Papa New Guinea
- transmitted via cannibalism
3
Q
Creutzfeldt-Jakob disease
A
- rapidly progressive
- sporadic, familial, or iatrogenic
- characterized by myoclonus, behavior changes, dementia
- rarely results from tissue transplants
4
Q
New Variant CJD
A
- “mad cow”
- associated with uncommon genetic homozygosity: Met-Met at codon 129 of PRNP
- occurs in young adults, presents with psychiatric symptoms
5
Q
Fatal familial insomnia
A
- inherited with progressive course of insomnia
- autonomic instability with increased sympathetic tone
- signs of motor system degeneration and later spongiform changes
6
Q
Gerstmann-Straussler-Scheinker syndrome
A
- spongiform encephalopathy
- usually familial
- cerebellar degeneration and dementia
