Masika M.M.

Characteristics
Transmission
Pathogenesis
Clinical Manifestations
Diagnosis
Treatment & Prevention

Human Prionoses

Animal Prionoses

Creutzfeldt-Jacob Disease
(CJD)

Bovine Spongiform
Encephalopathy (BSE) - cows

Fatal Familial Insomnia (FFI)

Gerstmann-StrausslerScheinker Syndrome (GSS)
Kuru

vCJD

Scrapie -Sheep
Chronic Wasting Disease
(CWD) Mules, deers

 Familial/Hereditary

Sporadic
Infectious

Small PROteinaceous INfectious particles that are

resistant to inactivation by procedures that usually

modify proteins and nucleic acids

 Made of protein
Resistant to inactivation
Reproduce by converting normal protein to abnormal protein
Do not induce the immune system
Cause vacuolation of neurons >> spongiform appearance

Alpha-Helix

Beta chains

Cellular (PrPc)

Characteristic

Predominantly
Alpha-helical

Structure

Predominantly betasheets

Soluble

Solubility

Insoluble

Non-infectious

Infectivity

Infectious

Resistance to
Proteinase K

Resistant

Susceptible

Scrapie (PrPsc)

 Blood transfusion
Organ transplant
Hereditary
Ingestion
Beef
Cannibalism

 Heralded by accumulation of abnormal prion protein PrPsc

Normal cellular protein (PrPc) is found on the surface of brain cells & other cells
Infection or Mutation of PRNP gene on c.20 can cause PrPc to misfold into PrPsc

PrPsc dissociates from the cell membrane

PrPsc causes PrPc to misfold and dissociate from the cell surface

More PrPc is synthesized >> PrPsc induces misfolding & release of these proteins
Accumulation of PrPsc forms proteinaceous plaques between the brain cells
Aggregated PrPsc is then internalized into cells >> spongiform appearance

 Long incubation period, slow, progressive & fatal disease

Cognitive impairment
Ataxia
Myoclonic jerks
Mutism
FFI: Insomnia, dysautonomia, motor paralysis

 Clinical presentation
Exclude DDx
EEG
Brain biopsy
Western Blot
Gel Electrophoresis
MRI

 No treatment
Prion diseases are invariably fatal (within months to years)
Supportive treatment (Palliative care)

 Sterilization of surgical equipment

Screening
Do not feed cows to cows
Screening of blood & organs