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Prion Diseases

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by

Sarah DiPasquale

on 17 December 2013

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Transcript of Prion Diseases

Why is it dangerous?
prions are always present, even if you aren't affected
100% mortality rate
no cure
in the US
incubation period: up to 50 years
Fatal Familial Insomnia (FFI)
caused by a mutation in protein PrP^c
death from lack of sleep
often presents itself between ages 18 to 60
average age of patients: 50
can develop spontaneously
mostly inherited
Why are prions so dangerous?
resistant to denaturation
no cure
always fatal
can have long incubation periods
Why is it dangerous?
100% mortality rate
no cure
average survival span: 18 months after symptoms appear
offspring have 50% chance of inheriting and developing FFI if only one parent carries the mutated gene
sleeping pills, barbiturates, and therapy usually have little to no effect
sleep is very important
Beginning Symptoms
depression
fever
epilepsy
excessive sweating
raise in blood pressure and heart rate
neck stiffness
constipation
anxiety
sudden change in character and behavior
Prion Diseases
Beginning Symptoms of CJD
rapidly progressing dementia
changes in eyesight and personality
problems with muscle coordination
impaired memory, judgement, and thinking
insomnia
depression
unusual sensations
As it progresses...
damage becomes horrible
involuntary muscle jerks
severe mental impairment
can go blind
lose ability to move and speak
die within 4 months of infection
An overview of prions
a prion is an infectious agent composed of incorrectly folded proteins that cause other proteins to be incorrectly folded
not living, reproduce using host cell
attack central nervous system
ex:scrapie in sheep, Creutzfeldt-Jakob Disease in humans, kuru in humans, FFI in humans
spongiform
Creutzfeldt-Jakob Disease
most common prion disease in humans
like mad cow disease, but for humans
can be genetic, acquired, or sporadic
multiplies rapidly after incubation period
typical age of people affected is 55-95
Diseases of the Central Nervous System
Kuru
mainly attacks cerebellum
transmitted mostly through cannibalism
originated in New Guinea
believed to have started from eating meat infected with Mad Cow Disease or Scrapie
so common in the area that victims can diagnose themselves
Beginning
Symptoms
tremors
slurred speech
arm and leg pain
headache
Why is it
dangerous?
As it
progresses...
no treatment
no cure
incubation period: years to decades
destroys brain
always fatal
extreme memory loss
severe coordination problems
uncontrollable laughing or crying
trouble swallowing
inability to eat
not able to sit or stand independent of help
death within 1-2 years after infection
As it progresses...
panic attacks
paranoia
phobias
hallucinations
loss of weight
dementia (i.e.Alzheimer's)
difficulty walking or speaking
become "half-asleep," similar to sleepwalkers
By Sarah DiPasquale, Heather Gendelman, Mallory Hudson, & Simone Barbee
Sources Used in This Presentation
http://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease

http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm

http://medicalxpress.com/news/2013-12-blood-transmit-creutzfeldt-jakob-disease.html

http://www.cjdfoundation.org/

http://www.cdc.gov/ncidod/dvrd/cjd/

http://www.who.int/mediacentre/factsheets/fs180/en/

http://www.cnn.com/2013/09/20/health/creutzfeldt-jakob-brain-disease/

http://www.ninds.nih.gov/disorders/kuru/kuru.htm

http://www.macalester.edu/psychology/whathap/UBNRP/tse10/Kuru.html

http://www.nlm.nih.gov/medlineplus/ency/article/001379.htm

http://en.wikipedia.org/wiki/Prion

http://www.nbcnews.com/id/6822468

http://en.wikipedia.org/wiki/Fatal_familial_insomnia
Full transcript