Pathology EMQ template.

Name: Samuel Reiterbund-Olley

Candidate number:

Theme: Prion disease

OPTION LIST

A	Tonsillar biopsy	I	Neurogenic testing
B	Alzheimer's	J	Sporadic CJD
C	Idiopathic CJD	K	Familial dementia
D	Variant CJD	L	Blood test for prion protein
E	Brain biopsy	M	Iatrogenic CJD
F	Vascular dementia	N
G	EEG	O
H	Vasculitis	P

For each scenario below, choose the most appropriate answer from the list above. Each option may be used once, more than once or not at all.

1. A 65 year old man presents with cortical blindness he has had a six month history of non-specific psychiatric symptoms but has very recently developed cerebellar ataxia, involuntary movements and painful sensory disturbances. What is the most likely diagnosis?

2. A 30 year old man presents with psychiatric symptoms of paranoia it is also noted from the history that the man has pain in his hands and has suffered a long history of depression. On examination he has ataxic symptoms. When talking to his mother she has noted that his behaviour has slowly been deteriating over many years.

3. When differentiating between Sporadic CJD and which other condition would a person perform a brain biopsy on a living patient.

4. Which investigation can be used as a test for Variant CJD and also a basis for treatment in a patient whom you suspect possible Variant CJD possibly after exposure to pathological prion protein via contaminated blood.

5. A 35 year old man presents with unsteadiness and in-coordination he has noted that the problems with his "balance have been going on for a long time now". He puts it down to the growth hormone treatment he received when he was a child. He has however no signs or symptoms of dementia... What is the most likely diagnosis?

ANSWERS

1. J

2. D

3. H

4. A

5. M