Prion diseases
transmissible spongiform encephalopathies
Important characteristics of prions
long incubation time, gradual increase in sx severity, no host immune response, neuro-patho findings
Infectious prion disease etiology
exposure to exogenous PRPsc from eating infected beef, cannibalism, or contaminated surgical instruments
Inherited prion disease etiology
mutation in PrPc gene favors spontaneous misfolded protein structure
Prion dz sxs
dementia, ataxia, myoclonic jerks, death
Specified risk material associated with prion disease
lymphatic and neural tissue