Pathology EMQ template.

Name: Anna Roche

Candidate number:

Theme: Prion Disease

OPTION LIST

A	Alzheimer’s disease	I	Kuru
B	Atypical CJD	J	Paraneoplastic syndrome
C	Cerebral vasculitis	K	Parkinson’s disease
D	Familial CJD	L	Spinocerebella ataxia
E	Familial fatal insomnia	M	Sporadic CJD
F	Gerstmann-Straussler-Sheinker syndrome	N	Underlying neoplasm
G	Huntington’s disease	O	Variant CJD
H	Iatrogenic CJD	P	Vascular dementia

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 disease presenting with a rapid dementia with a serial EEG demonstrating pseudoperiodic complexes.

2. A disease caused by exposure to human prions through a cannibalistic feast.

3. A disease presenting with psychiatric syndromes e.g. anxiety, hallucinations, followed by neurological symptoms such as ataxia. A tonsillar biopsy is always diagnostic.

4. The result of a germline PRNP mutation, most commonly P102L, resulting in a slowly progressive ataxia, diminished reflexes and dementia with a median survival of 2-10 years.

5. A disease that has been seen to occur several years after procedures such as blood transfusions, appendicectomy and corneal transplants.

ANSWERS

1. M

2. I

3. O

4. F

5. H