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MICRO ADYA > Microbiology 9 - Prion disease > Flashcards

Microbiology 9 - Prion disease Flashcards

1
Q

What marker of sCJD may be identified in CSF?

A

14-3-3

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2
Q

What does ‘prion’ stand for?

A

PRotein-only InfectiOus ageNt.

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3
Q

What is the general pathophysiology of sporadic CJD?

A

Rapid neurodegeneration via spongiform vacuolation

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4
Q

Upon which chromosome is prion encoded?

A

C20

predominantly expressed in CNS

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5
Q

Describe the structure and function of healthy prion

A

Alpha helical structure - involved in copper binding

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6
Q

What is the structure of pathological prion?

A

Beta pleated sheet

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7
Q

What is the name given to sporadic prion disease?

A

sporadic Creutzfeld Jacob Disease

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8
Q

What are the acquired prion diseases?

A
  1. Variant - bovine spongiform encephalopathy
  2. iatrogenic CJD
  3. Kuru (Papau New Guinea)
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9
Q

What are the 3 causes of inherited CJD?

A
  1. PRNP mutations (can be inherited)
  2. Gertsmann-Strausser-Sheinker syndrome
  3. Familial fatal insomnia
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10
Q

How does sCJD present?

A

RAPID DEMENTIA with:

  • myoclonus
  • cortical blindness
  • akinetic mutism
  • LMN signs
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11
Q

What is the mean age of onset of sCJD?

A

45-75

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12
Q

How quickly does sCJD cause death?

A

Within 6/12 months

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13
Q

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

A

30 years

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14
Q

What is the mean survival time in variant CJD?

A

14 months

**slightly longer than sporadic

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15
Q

Recall the typical presentation of variant CJD/ BSE

A

Dysphonia
Hallucinations
Anxiety
Paranoia
(Psychological symptoms mostly)

–>later neurological symptoms - peripheral sensory sx, ataxia, myoclonus

**think; younger population presenting w psychotic type symptoms, as opposed to older ppl presenting w dementia type symptoms**

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16
Q

What would an MRI show in variant CJD/ BSE?

A

Positive pulvinar sign

i.e. posterior thalamus highlighted on MRI-T2

17
Q

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

A

Tonsilar biopsy – western blot –> type 4t

18
Q

What is the inheritance pattern of prion proteins?

A

All autosomal dominant

19
Q

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

A

dysarthria progressing to cerebellar ataxia ending in dementia

20
Q

sCJD EEG changes

A

periodic triphasic changes

21
Q
A
22
Q

MRI in sCJD

A

normal/highlighting basal ganglia

23
Q

CSF analysis of sCJD

A

14-3-3 protein positive

24
Q

Genetics of sCJD

A

most cases 129 codon MM

??

25
Q

Western blot result of sCJD

A

Types 1-3

26
Q

Post-mortem of sCJD

A
  1. Spongiform vacuolation 2. PrP amyloid plaques
27
Q

vCJD EEG results

A

non-specific slow waves

28
Q

CSF analysis of vCJD

A

14-3-3 may be normal

29
Q

Genetics of variant CJD

A

ALL cases 129 codon MM

30
Q

Western blot result of vCJD

A

Type 4t

31
Q

post mortem of vCJD

A
  1. PrPSC 4t detedtable in CNS + lymphoreticul ar tissue 2. Florid plaques
32
Q
A
33
Q

How does iatrogenic CJD occur?

how does it present?

A

inoculation with human prions most commonly from surgery

presentation: progressive ataxia–>dementia and myoclonus. speed of progression depends on route of inoculation (CNS inoculation is fastest)

34
Q

ateiology of Kuru

presentation

A

exposure to human prions from cannabalistic feasts

Progressive cerebellar syndrome (death within 2yrs) following 45yr incubation Dementia is late or absent. Epidemic was in the 1950/60s

35
Q

How does fatal familial insomnia present?

A

Insomnia and paranoia progressing to hallucinations and weight loss. Then a mute period. Death 1-18/12 after start of symptoms

36
Q

Tx of CJD

A

Symptomatic: clonazepam – mycolonus: (Valproate, Levetiracetam, Piracetam)
• Delaying prion ‘conversion’: Quinacrine, Pentosan, Tetracycline

MICRO ADYA (22 decks)

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