Pathology EMQ template.

Name:      

Candidate number:

Theme: Prion Disease

 

OPTION LIST

 

A	Gerstmann-Straussler-Scheinker Syndrome	I
B	Familial Fatal Insomnia	J
C	Iatrogenic CJD	K
D	Kuru	L
E	Sporadic CJD	M
F	Variant CJD	N
G		O
H		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. Mr. S is a 55-year old man who lives alone and has no family.  He is brought in by his neighbour who from time to time visits him; the neighbour says she found Mr. S completely unresponsive and mute and, becoming worried by this behaviour, brought him to the GP.  The neighbour gives a haphazard history of noticing changes in Mr. S over the previous 18 months, including apparent phobias at the outset followed by noticeable panic attacks and frank paranoia.  At times, Mr. S seemed to hallucinate.  This is in stark contrast to how Mr. S was previously, where he was soft-spoken, polite and calm.  He has also lost a lot of weight in the last year.

 

 

2. Brain MRI shows high signalling within the posterior thalamus, otherwise known as a positive pulvinar sign.

 

 

3. At presentation, patients have psychiatric signs followed by neurological signs.  Their median survival time is 14 months.

 

 

4. A 65-year old gentleman with known dementia and a family history of what he says was Multiple Sclerosis presents with slight ataxia and diminished reflexes upon neurological examination. He is found to have the PRNP P102L mutation and his prognosis for survival is anywhere between 2 and 10 years.

 

 

5. A 65-year old gentleman is brought in by his worried daughter.  She explains that over the last 5 weeks he has become so forgetful and absent-minded that she has had to hire a carer for him.  On neurological examination the striking findings are myoclonus and LMN signs.  An EEG shows periodic, triphasic complexes.

 

 

 

ANSWERS

1. B

2. F

3. F

4. A

5. E