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NEURO 411 > Prion clinical > Flashcards

Prion clinical Flashcards

1
Q

What is the earliest known prion disease?

A

Scrapie

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2
Q

What is unique about prion disease?

A
  • They can develop spontaneously
  • They can be inherited
  • They can be acquired
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3
Q

What does a prion mean?

A

“A small proteinaceous infectious particle”

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4
Q

Three steps for prion pathology?

A

Prion conversion (PrPc to PrPsc) (alpha to beta structure) –> accumulation –> spread

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5
Q

Prion pathology end stage?

A

Spongiform change, neuronal loss, astrocytosis (inflammation of astrocytes); accumulaiton fo PrPres(resistant to protease)

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6
Q

What type of plaques are formed with sCJD, vCJD, and GSS?

A
  • SCJD –> spontaneous spongiform
  • vCJD –> variant, concentraed plaques, spongiform around
  • GSS –> Genetic, multicentred plaques
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7
Q

Define prion strain

A

A specific form of misfolded PrPSc that reproduciley causes a specific phenotype of disease

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8
Q

Both ___ and ___ of prion aggregates influence disease phenottype

A

Size and shape

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9
Q

How is prion disease layed out?

A
  • 1-2million / year incidence
  • 90% sporadic
  • 10% genetic (familial CJD; PrP amyloidosis; Thalanmic degeneratioN)
  • 1% Acquired (kuru, vCJD, Iatrogenic)
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10
Q

Describe sporadic CJD

A
  • ALWAYS FATAL
  • Peak incidnece at 7th decade
    *Ataxia, visual symptoms, dementia, myoclonus
  • Akinetic mutism (cannot move)
  • Average time of death 4-6months after onset
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11
Q

How is sporadic CJD diagnosed?

A

Definite: Autopsy only; codon 129 status can give us clues (via glycoform ratio)

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12
Q

True or false: V127 E219K may protect against CJD?

A

true

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13
Q

What are Gerstmann Straussler Scheinker (GSS) and Peripheral amyloidosis?

A

Types of PrP amyloidoses (genetic prion disease)

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14
Q

Describe GSS

A
  • Early 50s, early ataxia, late cog. decline, motor/parkinsons feautres
  • Longer duration (6years)
  • Multicentred amyloid plaques
  • Majority mutations P102L
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15
Q

What are the clinical phenotypes of GSS?

A
  • Typical
  • GSS w/ areflexia and paresthesia
  • Pure dementia
  • CJD-like GSS

Variability determiend by ratio of misfolded Prp and WT Prp protein

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16
Q

What type of genetic prion disease is fatal familial insomnia (FFF) under?

A

Thalamic degeneration

17
Q

Describe FFF

A
  • Progressive sleep disturbance, autnoic changes, tremor, dysarthia, ataxia, myoclonus
  • Dementia
  • 1 year duration
  • NO SPONGIFORM or AMYLOIDOSIS and thalamus atrophy
  • Transmissable to mice
18
Q

Genetics of FFF?

A
  • Mutation in prion protein
  • Caused by D178N, however has to be on same allele as M129
  • If it is in cis with V129 –> familial CJD phenotype
  • MM129 makes faster disease, V129 slower

Must test for PRNP mutation for diagnosis

19
Q

Why are women and children most affected w/ kuru?

A

Portions of person consumed was varied: women and children got brain, men got mm.

20
Q

Does the presence of PrpSc (scrapie) equal infectivity?

A

No, it is dose dependent

21
Q

Has Prpsc been detected in dura mater?

A

No, but probably contamintation has led to transmission in dura transplants

growth hormone graft can also spread PrPsc

22
Q

Is CJD transmitted sexually or vertically?

A

No, also no increase incidence in healthcare workers

two researchers did die in lab due to mad cows disesase

23
Q

Is PrpSc transmissable via CSF?

A

No

can be detected in csf using RT-QuIC

24
Q

What is mad cow disease a part of?

A

Variant CJD (from acquired cjd) - probably acquired through scrapie which is reprocessed in the feed of animals -

not many cases ~ 232 world wide

25
Q

How do proteins cross species barrier?

A
  • Overlap of various protein types
  • Adaptation –> overlap w/ other hosts
26
Q

Define strains

A

As a prion isolates taht consistenyl induce a specific disease phenotype, pathology and incubation period in a given house – many shapes can be present in single strains

conformational drug resistance = orions adapting to diff. shapes

Strain selectivity –> have to target ALL strains

CWD prions adapy faster than BSE

27
Q
A

NEURO 411 (12 decks)

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