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Bio 3021 Infectious Disease & Society > Kuru, Cannibalism, Prions, & Spongiform Encephalopathies > Flashcards

Kuru, Cannibalism, Prions, & Spongiform Encephalopathies Flashcards

1
Q

meaning of Kuru

A

trembling from fear & cold

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2
Q

(5) Symptoms of Kuru

A

prolonged headaches and minor loss of coordination
symptoms rapidly progressed & by 4 months victims were unable to walk
speech disturbed
victims can no longer swallow
death occurred within 1 year

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3
Q

What approaches were tried to treat Kuru?

A 
antibiotics
aspirin
vitamins
drugs to treat parasitic worms
tranquilizers
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4
Q

Who was Kuru dominant in? Absent?

A

women and children

adult men

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5
Q

Kuru was not caused by the following agents?

A

infectious agent
environmental factors
heredity
mass hysteria

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6
Q

Components of Cannibalism in Fore Tribe

A

Practice not unusual to New Guinea that would provide an avenue for passage of infectious agent
Fore tribe ate tissues (especially the brain) of close relatives
A religious ceremony
But no infectious agent could be detected in laboratory studies

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7
Q

Who was sent to diagnose Kuru

A

Gajdusek

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8
Q

Results of the 12 brains observed by Gajdusek?

A

Brain tissue seemed normal (no signs of inflammation or infection)

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9
Q

Results of the 12 brains observed by neuropathologist?

A

brain revealed abnormalities (Loss of neurons

activation of astrocytes)

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10
Q

Loss of neurons?

A

spongiform encephalopathy

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11
Q

(4) What is Creutzfeldt-Jakob Disease?

A

brain disease
rapid progressive global dementia & marked progressive motor dysfunction
normal blood & test results
occurs within 1 year of onset of symptoms

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12
Q

Definitive diagnosis of CJD is based on

A

brain pathology at time of biopsy/autopsy

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13
Q

Why was CJD termed spongiform encephalopathy?

A

Termed spongiform encephalopathy due to gaping holes where neurons had once been

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14
Q

What is it the adult Fore women and children of both sexes are doing, but the adult men are not doing, nor are the people of the adjacent tribes?

A

Eating each other…cannibalism

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15
Q

The Fore tribe believed they were under a sorcerer’s curse due to______, so_____

A

the amount of ppl dying
unmarried men forced to assume task usually done by females
organized meetings to stress friendship & unity (willing to change their traditions)

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16
Q

Social Behavior of the men in the Fore Tribe

A

Adult men and adolescent boys lived largely separate lives living together in huts
Adult men and adolescent boys hunted in the forest, but kept meat for themselves, not sharing with the women

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17
Q

Social Behavior of the women & children in the Fore Tribe

A

The women prepared the body of the deceased for the ceremony, women consumed body tissues
Women used bamboo blades to prepare the tissues and packed the brain in small bamboo tubes to be steamed over an open fire before consumption
Small children playing in the area also given pieces of brain tissue to consume

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18
Q

What was revealed in the rhesus monkeys & chimps after being inoculated with fresh brain tissue taken from victims of Kuru?

A

Two chimpanzees ~20 months after inoculation developed clinical signs and symptoms of Kuru
Histopathologic analysis showed changes consistent with Kuru and Creutzfeldt-Jakob disease
Evidence that a transmissible agent is involved in the pathogenesis of Kuru

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19
Q

What did Kuru have an impact on?

A

medicine, neurology, & infectious disease

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20
Q

How did Kuru have an impact on medicine, neurology & infectious disease?

A

Opened new area of slow virus diseases
Opened new area of transmissible spongiform encephalopathies
Lead to identification of new infectious agent, the prion

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21
Q

In the slow virus disease______

A

Years may separate the time of initial contact with infectious agent and appearance of clinical disease

22
Q

Conventional agents

A

viruses

23
Q

Unconventional agents

A

prions

24
Q

Subacute Sclerosing Panencephalitis

A

Caused by measles virus with defective matrix (M) protein
Incubation period usually 7 - 10 years
Progressive dementia, ataxia, myoclonus
Death usually occurs within 1 - 3 years

25
Q

Transmissible spongiform encephalopathies are rare, but always fatal, slow neurodegenerative diseases caused by an

A

unconventional infectious agents

26
Q

Unconventional agents of the slow virus disease can be found in ____ & _____

A

animals and humans

27
Q

Unconventional agents found in animals?

A

scrapie in sheep
transmissible mink encephalopathy
bovine spongiform encephalopathy

28
Q

Unconventional agents found in humans?

A

Creutzfeldt-Jakob disease
Variant Creutzfeldt-Jakob disease (mad cow disease)
(Kuru)

29
Q

(5) Details of transmissible spongiform

A

Dementia and/or ataxia; loss of brain function; death
Prominent astrocytosis and neuronal loss
Lack of inflammation
Possible appearance of amyloid-like plaques or fibrils
Etiologic agent traced to prion,a protease-resistant protein (PrP)

30
Q

The prion is ________

A

a small proteinacious infectious particle which is resistant to inactivation by most procedures that modify nucleic acids

31
Q

Purification of scrapie-infected tissue revealed a 27 - 30 kd protein identified by a procedure that used proteinases and various detergents____

A

known as PrP (proteinase-resistant protein)

32
Q

PrP is smaller in size than _______and have remarkable______

A

conventional viruses; hydrophobic properties

33
Q

PrP is resistant to? (4)

A

Resistant to ionizing radiation & UV light
Resistant to formaldehyde, alcohols, nonionic detergents, proteinase K
Resistant to conventional autoclaving (15 - 30 min at 121 F)
Resistant to nucleases

34
Q

Sequencing of PrP followed by cloning its gene revealed that?

A

PrP is a protein found in normal hosts including humans (explains lack of immune response)

35
Q

PrP mRNA

A

found in highest levels in neurons

36
Q

PrP can be expressed in

A

brains of healthy animals

non-neuronal cells (T cells, B cells, and dendritic cells)

37
Q

What are the 2 isoforms of PrP?

A

PrPC is the cellular isoform of the prion protein

PrPSC (or PrPCJD) is the abnormal pathogenic isoform of the prion protein that causes illness

38
Q

PrPC vs. PrPSC

A

PrPC and PrPSC are encoded by same gene and share amino acid composition (immunological tolerance)
PrPC is sensitive to proteinase K, whereas PrPSC is resistant
PrPC is soluble in nondenaturing detergents, whereas PrPSC insoluble
PrPSC (but not PrPC) aggregates and forms amyloid plaques in brains with TSE
PrPC has an alpha-helix content of 42% and little beta-sheet, whereas PrPSC has an alpha-helix content of ~30% and 45% beta-sheet content

39
Q

After infection with PrPSC isoform, the PrPSC

A

causes the PrPC isoform to be converted into PrPSC

40
Q

PrP is a normal cellular protein

A

PrPc
protease sensitive
alpha helix conformation

41
Q

PrPc is converted to abnormal form

A

PrPsc
protease resistant
beta helix conformation

42
Q

What are the 4 forms of CJD?

A

Sporadic form
Familial form
Iatrogenic form
Variant form of Creutzfeldt-Jakob disease (vCJD)

43
Q

Sporadic Form (4)

A

Accounts for ~85% of all cases of CJD
Bulk of cases occur between ages 50 and 79
Presents as a presenile dementia characterized by rapidly progressive mental deterioration, myoclonic jerking, deterioration of motor functions, ataxia,
Death within one year of onset of symptoms

44
Q

Familial Form (5)

A

Accounts for 5% to 15% of all cases of CJD
A genetic disorder due to point mutations /
Onset takes place at a younger age when compared with sporadic CJD
Disease progression characterized by progressive insomnia, autonomic dysfunction, endocrine changes, decline in motor / cognitive functions,
Death within one year of onset of symptoms

45
Q

Iatrogenic Form (5)

A 
Accounts for ~1% of all cases of CJD
Medical transmission of infectious agent
Median incubation period of 13 years
Disease progression similar to sporadic
Kuru, an epidemic of iatrogenic form of CJD
46
Q

Variant form of Mad Cow Disease was first recognized?

A

1996 Great Britain as a form of CJD that differs from sporadic CJD

47
Q

What age do cases of Variant form of Mad Cow Disease occur?

A

younger age group…mean age 26 years

48
Q

Early Stage? Late? Variant Form Mad Cow Disease

A

early stages illness present w/ psychiatric symptoms

late have sensory symptoms develop that progress to ataxia, dementia, death usually 13 months after onset

49
Q

Origin of Variant Form Mad Cow Disease is unclear, but was identified

A

in pts who received blood transfusions from donors who late died of vCJD

50
Q

Prion Inactivation (5)

A

Incineration
Treatment with mercaptoethanol, SDS
Treatment with sodium hypochlorite (20,000 ppm)
Immersion for 60 min in 2N sodium hydroxide
Autoclaving for 90 min at 132 - 136 F

Bio 3021 Infectious Disease & Society (4 decks)

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