Name: Krishan Patel

Theme: Prion Disease

 

OPTION LIST

 

A

Alzheimer’s Disease

I

Parkinson’s Disease

B

Cerebral Vasculitis

J

Sporadic CJD

C

Familial Fatal Insomnia

K

Variant CJD

D

Gerstmann-Straussler-Sheinker syndrome

L

Vascular Dementia

E

Huntington’s Disease

M

 

F

Iatrogenic CJD

N

 

G

Kuru

O

 

H

Paraneoplastic syndrome

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 70 year old male being treated for mixed dementia died 6 months after he first presented with brief involuntary twitches. He was reported to become gradually more passive with decreasing speech and movements. He developed cortical blindness in the right eye. On autopsy, a brain biopsy showed he had spongiform vacuolation and PrP amyloid plaques. What disease did he suffer from?

 

2. A 35 year old female presents to her GP with a one year history of abnormal movements that have been getting progressively worse. Her spouse has noticed some increasingly impulsive behaviour and irritability. She reported that her father had similar symptoms, with a slow decline in cognitive function over a period of 15 years and subsequent death. What disease is she most likely to be suffering from?

 

3. A 30 year old male presented with paranoia and hallucinations to his GP. He later developed involuntary twitches and ataxia. After referral to a neurologist, an MRI scan of the brain was done and showed a positive pulvinar sign. A subsequent tonsillar biopsy was also positive for PrPSc. What disease is he suffering from?

 

 

4. An 80 year old male was reported to be showing signs of progressive memory loss and global cognitive impairment. He has been getting increasingly irritable and has been found disorientated and wondering the hallways of his care home on numerous occasions. What is the most likely diagnosis?

 

 

5. Following a corneal transplant, a 50 year old female developed mild ataxia which then got progressively worse. She also developed some random jerking movements of her arms and memory loss. What is the most likely cause of her symptoms?

 

 

 

ANSWERS

1. J

2. E

3. K

4. A

5. F