CJD And VCJD

Question 1

Give two macroscopic features of spongiform encephalopathies.

Answer 1

Cysts forming - spongy appearance

Plaques forming as prions aggregate on the membranes of neurones

Question 2

Name the four types of CJD.

Answer 2

Iatrogenic
Variant
Familial
Sporadic

Question 3

What is the cause of vCJD in humans?

Answer 3

Eating cows with mad cow disease or sheep with scrapie

Question 4

What is CJD spread by cannibalism of infected flesh?

Answer 4

Kuru

Question 5

What is the symptom onset like in CJD?

Answer 5

May not show up for decades but then begin to progress rapidly leading to death within a year

Question 6

What are some early and late signs of CJD?

Answer 6

Ataxia
Poor memory
Behavioural changes
Visual disturbances

Muscle weakness
Myoclonus
Dementia (RP) 
Blindness 
Weakness
Coma

Question 7

What protein is elevated in lumbar puncture of people with CJD?

Answer 7

14-3-3 protein

Question 8

What is death usually caused by?

Answer 8

Pneumonia due to impaired coughing reflex

Question 9

Classic CJD vs vCJD

Mean age of death?
Duration of illness?
Which one has psychiatric signs first with delayed neurological signs?
Sharpwaves of EEG?
Florid plaques?

Answer 9

vCJD die sooner (28 years vs 68)
vJCD illness lasts for longer (13-14m vs 4-5)

vCJD

Classic
VCJD