Prion Diseases

Question 1

Kuru

Answer 1

• prototypical prion disease
• neurodegenerative
• transmitted via ingestion (cannibalism of flesh)

Question 2

Gerstmann-Straussler-Scheinker Syndrome

Answer 2

• rare transmissable spongiform encepalopathy
• dementia common feature
• some rare sporadic cases, usually familial

Question 3

Fatal familial insomnia

Answer 3

• inheirited, rare disorder
• progressive degree of insomnia to sleeplesness
• autonomic instability, motor system degeneration, spongiform changes (like CJD)

Question 4

Creutzfeldt-Jakob Disease

Answer 4

• sporadic, familial, iatrogenic (from pituitary of cadaveric victims)
• neurodegenerative -myoclonus, behavioral changes, dementia
• New Variant (vCJD): associated with mad cow
• genetic susceptibility (met-met at codon 129 of gene PRNP -fatal (no cure)