NS Infection - Prions Flashcards
Whats the main Prion disease?
Creutzfeldt-Jakob Disease (CJD)
What is a prion?
Transmissable Proteinaceous particle
What are the types of CJD?
- Sporadic
- New Variant
- Familial
- Acquired (Cadeveric Growth Hormone treatment, Dura Mater graft, blood transfusion)
What are the clinical features of Sporadic CJD?
- Slow insidious onset usually in the >60s
- Early behavioural abnormalities
- > Rapidly progressing dementia
- > Global Neurological Decline
Can also cause myoclonus, motor abnormalities, cortical blindness and seizures
DDx for CJD?
Alzheimer’s with myoclonus (More prolonged than CJD)
Subacute Sclerosing Panencephalitis (A rare chronic infection with defective measles virus)
CNS vasculitis
Inflammatory Encephalopathies
Prognosis for CJD?
Rapid decline then death (within 6 months mostly)
How does New Variant CJD present differently form Sporadic?
A younger onset (<40)
Early behavioural changes are much more prominent
Longer course (Avg = 13 months)
How does New Variant CJD reach humans?
Eating infected material, mainly cattle.
There’s possibly a genetic susceptibility involved too
How do we investigate CJD?
MRI
EEG
LP (with immunoassay)