Pathology EMQ template.

Name: Jen Richardson

Candidate number:

Theme: Prion disease

OPTION LIST

A	Alzheimer’s disease	I	Multi-infarct dementia
B	Binswanger’s Disease	J	Pick’s disease
C	Bovine spongiform encephalopathy	K	Steele-Richardson-Osliewski Syndrome
D	Fatal Familial Insomnia	L	Variant Creuzfeldt-Jakob disease
E	Gerstmann-Straussler-Scheinker disease	M
F	Huntingdon’s disease	N
G	Inherited Creuzfeldt-Jakob disease	O
H	Kuru	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 27 year old man presents with a 6 week history of deteriorating co-ordination, increasing muscle twitching and incoherent speech. As a child, he was treated for hypopituitarism but is otherwise well.

2. A 35 year old woman visits her GP after 2 months of waking in the night and falling asleep in the day. She has also noticed increased sweating.

3. A 68 year old man is seen in Neurology out-patients after being referred by his GP with a 4 month history of confusion, abnormal gait and poor co-ordination. His father died from a neurological disease.

4. A 55 year old man with the following genotype: M/M polymorphism at codon 129 of the prion protein, HLA DQ7. His wife is worried that he has exhibited signs of personality changes.

5. A 68 year old man referred to the Neurology department with a 3 year history of progressive confusion and personality change.

ANSWERS

1. L

2. D

3. G

4. G

5. A