Pathology EMQ template.

Name: Arunashis Sau

Theme: Prion Disease

OPTION LIST

A	Sporadic CJD	I	Dysphoria
B	Kuru	J	Ataxia
C	Variant CJD	K	Dementia
D	Gerstmann-Straussler-Sheinker syndrome	L	Positive Pulvinar Sign
E	Iatrogenic CJD	M	Diminished reflexes
F	Myoclonus	N	Dysautonomia
G	Hallucinations	O	Methionine-Methionine
H	Periodic complexes on EEG

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 30-year-old male is being investigated by the neurology team. Tonsillar biopsy yields a conclusive diagnosis. What is the diagnosis?

2. A 25-year-old female with Variant CJD undergoes brain MRI scanning, what is seen?

3. A 70-year-old male is suffering from iatrogenic CJD. What would have been the initial feature?

4. A 30-year-old female undergoes genetic testing and is found to have the PRNP P102L mutation. What condition does she suffer from?

5. A 70-year-old male has elevated 14-3-3 protein in the CSF. What finding would you expect in him but NOT in a patient with variant CJD?

ANSWERS

1. C

2. L

3. J

4. D

5. H