Creutzfeldt-Jakob disease (CJD)

Neurodegenerative Prion Diesease

Miss folded Prion Protien (PRPsc)

coded by PRNP gene;

Humans, mammals, enviroments

Digestive tract or open wound

Ingestion of infected meat, conact with infected tissue/enviromental protien; sporadic (85-94% of cases), hereditary (10% of cases)- onset age 50-60 yrs

Variable- weeks to years

Mis-folded prion protiens resist digestion and induce misfolding of normal prion protiens. PRPsc forms water insoluble aggregates that damage nerve cells and impair brain function

Dementia, memory loss, hallucinations, paranoia, obessive-complusive, sleep distrubance, problems w/ muscle control

severe brain damage, secondary infections, mortality- 85% w/in two years of symptoms onset (median life expectancy 4 months)

dont eat contaminated animals, avoid blood transfusions or tissue donations from at risk individuals, follow strict safety precautions, incinerate contaminated material

No cure, pallative care