Prion Disease#Source : Murray

Rizka A. Maghfirah

1. Prion is the acronym for...

Proteinaceous infectious particle
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2. Which statement is untrue about prion pathogenesis?

Elicit no immune response
Resistant to inactivation by heat
Cause vacuolation of neurons
Induce inflammation in ganglia
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3. The normal protein that is protease sensitive and held in cell membrane is ...
PrPC (cellular prion protein)
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4. Protein conformation of PrPc should be...

Alfa-helical
Beta-helical
Alfa-sheet
Beta-sheet
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5. The cell-free protein that is protease resistant and aggregates into amyloid
rods termed as...
PrPSc (scrapie-like prion protein)
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6. Name the theory that explain how abberant protein can be causing a disease.
template-mediated protein refolding
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7. Protein conformation of PrPSc is...

Alfa-helical
Beta-helical
Alfa-sheet
Beta-sheet
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8. PrPSc bind to an anionic structure on the cell surface and PrPC and then causing
PrPC refolds. What is the anionic structure?
Phospholipid
Cholesterol
Acetylglucosamine
Glycosaminoglycan
Glucosamine
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9. What is the chromosome that encode PrPC in human?

chromosome 20
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10. The mutation of PrPC will produce different strain of PrPSc. Based on this
etiology, what type of disease occur?
Acquired disease
Sporadic disease
Genetic disease
Infectious disease
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11. Self-perpetuating alternative folding patterns of the PrPC will produce

different strain of PrPSc. Based on this etiology, what type of disease occur?
Acquired disease
Sporadic disease

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12. Severity of CJD is determined by the specific mutation of what codon?

codon 129
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13. These are diseases that occur because of aggregation of other proteins into
prions or prion-like structures, except
Alzheimer disease
Addison disease
Huntington disease
Parkinson disease
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14. Acquired-prion disease can occur through 3 pathways. Mention all of them.
Ingestion
Penetration through cut in the skin
Direct infection through the infected tissue
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15. In patient with symptomatic spongioform encephalitis, some protein markers

would be detected in CSF. Mention 2 names of this protein markers.
(tau protein or 14-3-3 brain protein
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16. Kuru is endemic to what country?

New guinea
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17. Who are the people in high risk to get kuru?

Women and children in Fore tribe of New Guinea
(JAWABANNYA HARUS INI)
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18. What is the source of vCJD outbreak in younger people (<45 years) in 1996 in
the UK?
Beef
Eek meat
Cattle’s viscera
Sheep’s brain
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19. What is the disease that can be acquired/sporadic/genetic prion disease?

Creutzfeldt-Jakob disease (CJD
FFI
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20. Mention all acquired-prion disease

kuru, Creutzfeldt-Jakob disease (CJD), and variant CJD (vCJD
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21. The most often occurrences of fatal familial #insomnia is....

Acquired
Genetic
Sporadic
Infection
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22. Mention 4 prion diseases in animal.

Scrapie (sheep and goats)
Transmissible mink encephalopathy
Bovine spongiform encephalopathy (BSE [mad cow disease])
Chronic wasting disease (mule, deer, and elk)

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23. Chronic wasting disease occur in these animals, except

Elk
Moose
Mule
Deer
Antelope
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24. Scrapie occur in what animals?

Sheep and goat
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25. In Western blot test for the prion disease diagnosis confirmation, commonly use
the antibody of PrP in what tissue biopsy?
Brain
Heart
Tonsils
Peyer Patches
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26. A 73-year-old man complained of weakness, forgetfulness, difficulty speaking,

and involuntary movements of his right arm. After 3 months, myoclonus (muscle
twitching) and other neurologic signs were noted and he was hospitalized. Protein
14-3-3 was detected in cerebrospinal fluid, but there was no evidence of an
infection. The patient’s condition continued to deteriorate, he slipped into a
coma, and he died 4 months after the onset of symptoms
What is the diagnostic clue of to confirm the dx of this scenario?
Protein 14-3-3 was detected in cerebrospinal fluid
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27. A 30 year-old woman come to family doctor with tremors of her all limbs. The
doctor just gave a supportive treatment. After 3 months, she complained the
difficulty of remembering. The doctor identify her CSF and found the elevation of
tau protein. Within 10 months of the onset, she passed away. This woman live in
cattle farm and working as buthcer in her slaughterhouse. ###What is the dx?
Variant Creutzfeldt-Jakob disease
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28. A 30 year-old woman come to family doctor with tremors of her all limbs. The
doctor just gave a supportive treatment. After 3 months, she complained the
difficulty of remembering. The doctor identify her CSF and found the elevation of
tau protein. Within 10 months of the onset, she passed away.#This woman live in
cattle farm and working as buthcer in her slaughterhouse. ##Based on her history,
what type of occurnes of the disease?
Acquired
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29. a 66-year-old woman who was referred to a memory clinic for further evaluation
of a 5-month history of rapidly progressive dementia. She had problems with short-
term memory and functional abilities, including getting dressed, using the toilet,
and getting lost in her house. A non-contrast MRI of her brain was significant for
global parenchymal loss. He returned one week later to discuss her test results, by
which time she had developed cogwheel rigidity. Her symptoms progressively worsened
and she died 7 months after the onset. The postmortem brain autopsy demonstrated
the presence of abnormal protease-resistant prion protein by Western Blot analysis.
What is the dx?
Creutzfeldt-Jakob disease
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30. a 66-year-old woman who was referred to a memory clinic for further evaluation
of a 5-month history of rapidly progressive dementia. She had problems with short-
term memory and functional abilities, including getting dressed, using the toilet,
and getting lost in her house. A non-contrast MRI of her brain was significant for
global parenchymal loss. He returned one week later to discuss her test results, by
which time she had developed cogwheel rigidity. Her symptoms progressively worsened
and she died 7 months after the onset. The postmortem brain autopsy demonstrated
the presence of abnormal protease-resistant prion protein by Western Blot analysis.
What is treatment for the patient if she still alive?
No treatment
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