Creutzfedt-Jakob Disease

Question 1

What is Creutzfedt-Jakob Disease?

Answer 1

Group of neuro-degenerative diseases caused by prions

Question 2

What is Creutzfedt-Jakob Disease caused by?

Answer 2

Prion proteins

Question 3

What can it result in?

Answer 3

A rare, rapidly progressive and fatal neurological condition caused by prion proteins

Question 4

How can occur?

Answer 4

Sporadic (90% of cases)
Genetic (10-of cases),
Acquired/’variant’(<1% of cases).

Question 5

What do the prions cause?

Answer 5

Induce the formation of amyloid folds resulting in tightly packed beta-pleated sheets resistant to proteases.

Question 6

What is the main presenting symptom of Creutzfedt-Jakob Disease?

Answer 6

• rapidly progressive dementia over weeks or months
• eventually leads to immobility and mutism

Question 7

What are the other presenting symptoms of Creutzfedt-Jakob Disease?

Answer 7

Myoclonus
Ataxia
Visual disturbance

Question 8

How is Creutzfedt-Jakob Disease usually diagnosed?

Answer 8

Tissue biopsy

Question 9

Is there a cure for
Creutzfedt-Jakob Disease?

Answer 9

No

Question 10

What is the prognosis of
Creutzfedt-Jakob Disease?

Answer 10

Progresses rapidly and is fatal
Average of 6-12 months between symptoms onset and death