Prion Diseases Flashcards

You may prefer our related Brainscape-certified flashcards:
1
Q

are prions highly resistant to chemicals?

CHARACTERISTICS OF PRIONS

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Consist entirely of protein with ____

CHARACTERISTICS OF PRIONS

A

no nucleic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

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

CHARACTERISTICS OF PRIONS

A

do not present virus morphology

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

can prions be integrated into nucleic acid

CHARACTERISTICS OF PRIONS

A

Not integrated into nucleic acid of infected host cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

do prions elicit inflammatory reactions?

CHARACTERISTICS OF PRIONS

A

Do not elicit inflammatory reaction or cytopathic effects in host

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

do prions elicit antibody formation in host?

CHARACTERISTICS OF PRIONS

A

Do not elicit antibody formation in the host

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what are prions responsible for?

CHARACTERISTICS OF PRIONS

A

Responsible for vacuoles and abnormal fibers forming in brain of host

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

tramitted how?

CHARACTERISTICS OF PRIONS

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

WHAT IS A PRION?

WHAT IS A PRION?

A

Proteinaceous infectious particles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

An abnormal form of a normally harmless protein that may induce

WHAT IS A PRION?

A

disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Causes what?

WHAT IS A PRION?

A

slow neurodegenerative diseases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

CJD usually affects persons older than

WHO IS AT RISK?

A

50 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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?

A

prions

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
Q

clinical feature of prion disease

A

myoclonus

in the book

26
Q

Why are prions so resistant to disinfection?

PP

A

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
Q

prions are resistant to ?

PP

A

Resistant to a wide range of chemical and physical treatments, such as formaldehyde, ultraviolet radiation, and heat up to 80° C

28
Q

How can prion disease be treated?

PP

A

There is no treatment available

29
Q

How can prion disease be prevented? From spreading

PP

A

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

For GSS syndrome and CJD this is treatment process for neurosurgical tools and electrodes

30
Q

Cessation of ritual cannibalism in the Fore tribe in New Guinea has led to the disappearance of

PP

A

kuru

31
Q

Elimination of animal products from livestock feed to prevent ______ ?

PP

A

vCJD development and transmission

32
Q

sporadic CJD (sCJD), MOT?

Mechanism of Transmission

CLINICAL SYNDROMES OF PRION DISEASE

A

not known; possibly by alteration of normal prior protein (PrP) to rogue form due to somatic mutuation

MOT

33
Q

Variant CJD (vCJD), MOT?

Mechanism of Transmission

CLINICAL SYNDROMES OF PRION DISEASE

A

eating contaminated cattle products and by secondary bloodborne transmission

34
Q

Familial CJD (fCJD), MOT?

Mechanism of Transmission

CLINICAL SYNDROMES OF PRION DISEASE

A

Mutation in germline PrP gene

35
Q

Iatrogenic CJD (iCJD) MOT?

Mechanism of Transmission

CLINICAL SYNDROMES OF PRION DISEASE

A

contaminated neurosurgical instruments, corneal graft, gonadotrophic hormone, and, secondairly, by blood transfusion

36
Q

GSS ( Gerstmann- Straussler-Scheinker disease), MOT?

Mechanism of Transmission

CLINICAL SYNDROMES OF PRION DISEASE

A

mutuation in germline PrP gene

37
Q

Fatal familial insomnia (FFI), mot?

Mechanism of Transmission

CLINICAL SYNDROMES OF PRION DISEASE

A

mutuation in germline PrP gene

38
Q

After long incubation periods, these agents cause damage to the central nervous system, leading to a ______

Book notes

A

subacute spongiform encephalopathy

39
Q

Consists of an alternate conformation of a normal cell surface glycoprotein termed

A

cellular prion protein (PrP^C)

40
Q

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

A

PrP^Sc