23 prion diseases Flashcards by amilah yaqoob

Describe Prions Diseases and their characteristics

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NAME THE 2 PPL THAT first described PDs

Creutzfeldt and Jakob in 1920

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What are prions (3 points)

infectious proteins ; w no nucleic acid and resistant to killing w radiation/ UV etc.

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Characteristics of prion disease

fatal dementia
transmissable spongiform encephalopathies (TSE) (SPONGY)
long incubation periods
progressive & fatal on CNS
spongiform degenration of neurons

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what is the one cause of prion diseases

prions

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Clinical presentation of PD

EARLY - DEMENTIA
recurrent seizures - later
dead few mnths after symptoms show

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wht is a key diagnostic tool for prion diseases

neuropathology - confirm diagnosis

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What is CJD

Creutzfeldt-Jakob disease

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What is BSE

Bovine spongiform encephalopathy
mad cow

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what is CJDnv/CJDv

Creutzfeldt-Jakob disease – New variant

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CJD has approx what number oof cases yearly in mid aged & elders (rare)

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How has BSE affected CJD^nv rates

higher rates in young ppl since BSE

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other terms

FFI
Fatal Familial Insomnia
GSSD
Gerstmann-Sträussler-Scheinker Disease
PrP
Proteinaceous Infectious particle
PrPc
Normal Cellular PrP
PrPSc
Scrapie Type PrP
PrPRES, PrPCJD
Synonymous with PrP
TSE
Transmissible spongiform encephalopathy

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what year was BSE in cattle described (outbreak yr)

1986 - infection w prions of unkown origin (transmssion)

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After 1986 which animals derived infections possible due to being held captive ?

Abbreviation
Meaning
BSE
Bovine spongiform encephalopathy
CJD
Creutzfeldt-Jakob disease
CJDnv/CJDv
Creutzfeldt-Jakob disease – New variant
FFI
Fatal Familial Insomnia
GSSD
Gerstmann-Sträussler-Scheinker Disease
PrP
Proteinaceous Infectious particle
PrPc
Normal Cellular PrP
PrPSc
Scrapie Type PrP
PrPRES, PrPCJD
Synonymous with PrP
TSE
Transmissible spongiform encephalopathy

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Name some PDs of animals

What animal does scrapie occur in

sheeps & goats

name 3 symptoms of scrapie (sheeps&goats)

loss of coordination
irritability
intense itch (rip wool off)
histology img (purple spongy like)

BSE peaked in what year

1992

what did BSE do to cows

infection: ingestion
behav.chng - freezing, nose licks
motor abnorms - funny walk
death within month after symtpoms

How were cows cleaned up during BSE major outbreak

incinerated
even if one cow whole flock killed

What and when was the first described prion disease in HUMANS

kuru - 1900s - transmitted by cannabilism

(PDs of human table)
Name the disease in 1986 that was infection w prions of BSE origin?

Disease
Transmission
Year of description
Kuru
Ritualistic Cannibalism or “Transumption”
1900s
Sporadic Creutzfeldt-Jakob disease (sCJD)
Spontaneous PrPC→PrPScconversion or somatic mutation
1920
Familial CJD- (fCJD)
Mutations inPRNP
1924
Gerstmann-Sträussler-Scheinker(GSS)
Inherited Mutations inPRNP

1936
Iatrogenic CJD (iCJD)
Infection with Prions of human origin by transplants, Growth hormone or dura mater, inadequately sterilised instruments
1974
Fatal Familial Insomnia (FFI)
PRNPhaplotype 178N-129M

1986
Variant-CJD(vCJD)
Infection with Prions of BSE origin

1996
Sporadic Familial Insomnia (sFI)
Spontaneous PrPC→PrPScconversion or somatic mutation

1999
Variably protease-sensitive prionopathy (VPSPr)
Spontaneous PrPC→PrPScconversion?
2008

Who did kuru affect and what were the symtposm and transmission

ppl of Papau New Guinea (laughing death)

symptoms - ataxia, dementia, laughing (shake)

transmision: direct contact NGESTION - canabals ; women ate ppl mostly no men

origin: 1900ish individ. dvpd CJD

How long is the incubation period of Kuru

45-20yrs

What PD is assoc. w ppl eating sheep brains

kuru PNGuinea

hen was the last death of kuru

2005/2009

Classic Creutzfeldt Jakob Disease (CJD) worldwide epidemiology ?

1/2 /million ppl

Can CJD be caused by meat intake

Whats the average age of onset of CJD

65 yrs

What is classic Creutzfeldt Jakob Disease (CJD)

sporadic encephalopathy affecting humans that occurs worldwide at a rate of 1-2 cases per million people. It can occur spontaneously, genetically, or iatrogenically.

classic cjd description ?

sporadic encephalopathy affecting humans that occurs worldwide at a rate of 1-2 cases per million people. It can occur spontaneously, genetically, or iatrogenically.

Describe pathogenesis of CJD

after being ingested appears in blood multiplies in spleen and other organs of reticuloendothelial system spread to CNS migrating up peripheral nerves to spinal cord astrocytosis amyloid plaques Scrapie-associated fibrils (SAF)

What doe the holes on a hsitology img of spongiform CJD show?

BRAIN - holes are characteristics of spongiform encelopathies

Who first put forward prions. ?

Stanley Prusiner

prion description of variants

PrPc: these are small glycoproteins (33-55KDa) found in normal cells. (c=cellular) No apparent function PrPsc: a variant form found only in scrapie infected brain (sc=scrapie) perhaps mediated by a point mutation in the PrP gene (ch 20) codon 129 polymorphism (between Met and Val) Influences the susceptibility towards and the clinical features of human prion diseases

mutation PrPC PrPSc PrPC: α-structure PrPSc: β-structure accumulation in lysosomes

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