Microbiology 9 - Prion disease Flashcards Preview

Year 5 Pathology ICSM > Microbiology 9 - Prion disease > Flashcards

Flashcards in Microbiology 9 - Prion disease Deck (21)
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1

Describe some possible symptoms of prion disease

Ataxia
UMN weaknesses
Nystagmus
Limb spasticity
Hyperreflexia
Paraesthesia
Tremor
(all neuro symptoms!)

2

What marker of Creutzfeld Jacob Disease may be identified in CSF?

14-3-3

3

What is the recognised abnormailty on MRI that is indicative of CJD?

Increased signal in right parietal lobe

4

What does 'prion' stand for?

PRotein-only InfectiOus ageNt.

5

What is the general pathophysiology of prion diseases?

Rapid neurodegeneration via spongiform vacuolation

6

Upon which chromosome is prion encoded?

C20

7

Describe the structure and function of healthy prion

Alpha helical structure - involved in copper binding

8

What is the structure of pathological prion?

Beta pleated sheet

9

What is the name given to sporadic prion disease?

Creutzfeld Jacob Disease

10

What are the 2 types of acquired prion disease currently known about?

1. Variant/ iatrogenic CJD (included mad cow BSE)
2. Kuru (Papau New Guinea)

11

Recall 3 genetic causes of prion disease

1. PRNP mutations
2. Gertsmann-Strausser-Sheinker syndrome
3. Familial fatal insomnia

12

How does CJD present?

RAPID DEMENTIA with:
- myoclonus
- cortical blindness
- akinetic mutism
- LMN signs

13

What is the mean age of onset of CJD?

65

14

How quickly does CJD cause death?

Within 6/12

15

What is the mean age of presentation of variant CJD/ BSE?

26

16

What is the mean survival time in variant CJD?

14 months

17

Recall the typical presentation of variant CJD/ BSE

Dysphonia
Hallucinations
Anxiety
Paranoia
(Psychological symptoms mostly)

18

What would an MRI show in variant CJD/ BSE?

Positive pulvinar sign

19

What test is 100% sensitive and specific for variant CJD/ BSE, but useless in sporadic CJD?

Tonsilar biopsy

20

What is the inheritance pattern of prion proteins?

All autosomal dominant

21

What are the symptoms of Gertsmann-Strausser-Scheinher syndrome?

Progressive ataxia

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