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Prion Diseases Flashcards

1
Q

are prions highly resistant to chemicals?

CHARACTERISTICS OF PRIONS

A

Highly resistant to chemicals, radiation, and heat (can withstand autoclaving)

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2
Q

Consist entirely of protein with ____

CHARACTERISTICS OF PRIONS

A

no nucleic acid

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3
Q

Do/do not _____ present virus morphology in electron microscopy of infected brain tissue

CHARACTERISTICS OF PRIONS

A

do not present virus morphology

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4
Q

can prions be integrated into nucleic acid

CHARACTERISTICS OF PRIONS

A

Not integrated into nucleic acid of infected host cells

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5
Q

do prions elicit inflammatory reactions?

CHARACTERISTICS OF PRIONS

A

Do not elicit inflammatory reaction or cytopathic effects in host

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6
Q

do prions elicit antibody formation in host?

CHARACTERISTICS OF PRIONS

A

Do not elicit antibody formation in the host

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7
Q

what are prions responsible for?

CHARACTERISTICS OF PRIONS

A

Responsible for vacuoles and abnormal fibers forming in brain of host

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8
Q

tramitted how?

CHARACTERISTICS OF PRIONS

A

Transmitted only by close, direct contact with infected tissues and secretions

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9
Q

WHAT IS A PRION?

WHAT IS A PRION?

A

Proteinaceous infectious particles

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10
Q

An abnormal form of a normally harmless protein that may induce

WHAT IS A PRION?

A

disease

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11
Q

Causes what?

WHAT IS A PRION?

A

slow neurodegenerative diseases

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12
Q

what is kuru?

WHO IS AT RISK?

A

Members (especially women and children) of the Fore tribe in New Guinea were at risk for kuru because of ritual cannibalism.

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13
Q

CJD usually affects persons older than

WHO IS AT RISK?

A

50 years

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14
Q

how are Transplant and brain surgery patients at risk?

WHO IS AT RISK?

A

There are well-established cases of iatrogenic transmission, notably by corneal transplantation, deep implantation of electrodes in the brain, and administration of contaminated preparations of naturally derived human growth hormone.

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15
Q

symptoms of prion disease

CLINICAL PRESENTATION OF PRION DISEASE

A
  • Severe headaches
  • Depression, anxiety, apathy
  • Insomnia
  • Ataxia, slurred speech
  • Tremors in hands
  • Memory loss & moments of confusion; rapidly progressing dementia
  • Hallucinations
  • Coma-like state
  • Myoclonus (muscle twitching) on sudden stimulation
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16
Q

What key features of the postmortem findings are characteristic of the diseases caused by prions?

CLINICAL PRESENTATION OF PRION DISEASE

A

Lack of inflammation and the vacuolation of the brain are strong indicators of prion diseases

important

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17
Q

Progressive multifocal leukoencephalopathy (PML) caused by

WHAT IS A DIFFERENTIAL DIAGNOSIS OF PRION DISEASE?

A

JC polyomavirus

18
Q

list the diseases?

WHAT IS A DIFFERENTIAL DIAGNOSIS OF PRION DISEASE?

A
  • Dementia
  • Alzheimer disease
  • Stroke
  • Viral encephalitis
  • Autoimmune diseases
  • Neoplastic disease
19
Q

what diseases are associated with misfolded ⍺- synuclein3 ?

WHAT IS A DIFFERENTIAL DIAGNOSIS OF PRION DISEASE?

A

Multiple System Atrophy (MSA), Amyotrophic Lateral Sclerosis (ALS), Parkinson’s Disease

20
Q

Other proteins including amyloid beta (Aβ), tau, and huntingtin can all become

WHAT IS A DIFFERENTIAL DIAGNOSIS OF PRION DISEASE?

21
Q

Elevated protein markers (tau protein or 14-3-3 protein) can be detected in the

WHAT LABORATORY DIAGNOSTIC TESTS ARE USED TO DIFFERENTIATE AND DIAGNOSE PRION DISEASE?

A

cerebrospinal fluid of symptomatic persons

22
Q

Proteinase K–resistant form of PrP in a ____?

WHAT LABORATORY DIAGNOSTIC TESTS ARE USED TO DIFFERENTIATE AND DIAGNOSE PRION DISEASE?

A

Western blot using antibody to PrP

23
Q

No methods for directly detecting prions in tissue, Initial diagnosis must be made on _____ ?

WHAT LABORATORY DIAGNOSTIC TESTS ARE USED TO DIFFERENTIATE AND DIAGNOSE PRION DISEASE?

A

clinical grounds

24
Q

At autopsy: characteristic amyloid plaques, spongiform vacuoles, & immunohistologically detected ___?

WHAT LABORATORY DIAGNOSTIC TESTS ARE USED TO DIFFERENTIATE AND DIAGNOSE PRION DISEASE?

A

PrP can be observed

25
clinical feature of prion disease
myoclonus | in the book
26
Why are prions so resistant to disinfection? | PP
Prions are an alternate conformation of a normal mammalian protein that forms multimers. There is no genetic information to be inactivated, and the protein is already denatured from its normal functional form.
27
prions are resistant to ? | PP
Resistant to a wide range of chemical and physical treatments, such as formaldehyde, ultraviolet radiation, and heat up to 80° C
28
How can prion disease be treated? | PP
There is no treatment available
29
How can prion disease be prevented? From spreading | PP
Autoclaving at 15 psi for 1 hour (instead of 20 minutes) or treatment with 5% hypochlorite solution or 1.0 M sodium hydroxide can be used for decontamination ## Footnote For GSS syndrome and CJD this is treatment process for neurosurgical tools and electrodes
30
Cessation of ritual cannibalism in the Fore tribe in New Guinea has led to the disappearance of | PP
kuru
31
Elimination of animal products from livestock feed to prevent ______ ? | PP
vCJD development and transmission
32
sporadic CJD (sCJD), MOT? | Mechanism of Transmission ## Footnote CLINICAL SYNDROMES OF PRION DISEASE
not known; possibly by alteration of normal prior protein (PrP) to rogue form due to somatic mutuation | MOT
33
Variant CJD (vCJD), MOT? | Mechanism of Transmission ## Footnote CLINICAL SYNDROMES OF PRION DISEASE
eating contaminated cattle products and by secondary bloodborne transmission
34
Familial CJD (fCJD), MOT? | Mechanism of Transmission ## Footnote CLINICAL SYNDROMES OF PRION DISEASE
Mutation in germline PrP gene
35
Iatrogenic CJD (iCJD) MOT? | Mechanism of Transmission ## Footnote CLINICAL SYNDROMES OF PRION DISEASE
contaminated neurosurgical instruments, corneal graft, gonadotrophic hormone, and, secondairly, by blood transfusion
36
GSS ( Gerstmann- Straussler-Scheinker disease), MOT? | Mechanism of Transmission ## Footnote CLINICAL SYNDROMES OF PRION DISEASE
mutuation in germline PrP gene
37
Fatal familial insomnia (FFI), mot? | Mechanism of Transmission ## Footnote CLINICAL SYNDROMES OF PRION DISEASE
mutuation in germline PrP gene
38
After long incubation periods, these agents cause damage to the central nervous system, leading to a ______ | Book notes
subacute spongiform encephalopathy
39
Consists of an alternate conformation of a normal cell surface glycoprotein termed
cellular prion protein (PrP^C)
40
Binding to PrPSc changes the conformation of the PRPC protein which is rich in alpha-helical configuration to a beta-sheet enriched form to produce the aberrant protein termed
PrP^Sc

Microbiology (21 decks)

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