NS Infection - Prions

Question 1

Whats the main Prion disease?

Answer 1

Creutzfeldt-Jakob Disease (CJD)

Question 2

What is a prion?

Answer 2

Transmissable Proteinaceous particle

Question 3

What are the types of CJD?

Answer 3

• Sporadic
• New Variant
• Familial
• Acquired (Cadeveric Growth Hormone treatment, Dura Mater graft, blood transfusion)

Question 4

What are the clinical features of Sporadic CJD?

Answer 4

• 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

Question 5

DDx for CJD?

Answer 5

Alzheimer’s with myoclonus (More prolonged than CJD)

Subacute Sclerosing Panencephalitis (A rare chronic infection with defective measles virus)

CNS vasculitis

Inflammatory Encephalopathies

Question 6

Prognosis for CJD?

Answer 6

Rapid decline then death (within 6 months mostly)

Question 7

How does New Variant CJD present differently form Sporadic?

Answer 7

A younger onset (<40)

Early behavioural changes are much more prominent

Longer course (Avg = 13 months)

Question 8

How does New Variant CJD reach humans?

Answer 8

Eating infected material, mainly cattle.

There’s possibly a genetic susceptibility involved too

Question 9

How do we investigate CJD?

Answer 9

MRI
EEG
LP (with immunoassay)