1What does CJD stand for?
Creutzfeldt-Jakob Disease
2What percentage of cases are sporadic?
85-94%
3What percentage of cases are infectious?
6%
4What percentage of cases are hereditary/familar? FCJD
10%
5What is the abbreviation for Creutzfeldt-Jakob Disease?
CJD
6What is CJD classification?
*neurodegenerative prion disease
"sporadic, genetic, infectious"
7What is the causative agent of CJD? be specific :)
*misfolded prion protein isomer known as (PRP sc)
8What is some background info on PRPsc
*Prion Protein (PRP) is coded by PRNP gene
*PRP is a transmembrance protein (alpha helices become beta sheets)
*beta sheets form amyloid fibrils then plaques
*caused by valine and glutamic acid transition to lysine
9Epidemiology of CJD?
Reservoir: Humans, Mammals, Environment
Portal of entry: Digestive Tract, open wound
Mode of transmission: Infectious (contact with infected tissue or environmental piron), Kuru (ingestion of infected meat, this case human), Variant
10Does non-contagious acquistion of CJD exists? If so, explain.
Yes,
Hereditary/familiar (FCJD)
11What percentage of cases are FCJD? Age of onset?
Makes up 10% of cases, onset 50-60 yrs old
12Incubation period of Creutzfeldt Jakob Disease?
variable (weeks-years)
13What is pathogenesis of Crutzfeldt Jakob Disease?
*PRPsc (miss folded prion protein) resists digestion and induces misfolding of normal PRP prion proteins
*PRPsc forms water insoluble aggregates that damage nerve cells (impair brain function)
14What are the symptoms of Creutzfeldt-Jakob Disease?
Dementia
Memory loss
Hallucinations
Paranoia
Obsessive-compulsive
Sleep disturbance
Problems with muscle control
15Complications of CJD? What is the mortality rate (be specific)
*Severe brain damage
*Secondary infections
*Mortality - 85% with in two years of symptom onset. (Median life expectancy 4 months)
16Prevention for CJD?
*don't eat contaminated animals
*avoid blood transfusions or tissue donations from those infected
*follow safety precautions
*incinerate contaminated material
17Treatment fof CJD?
*no cure
*palliative care
18Reservoir of Creutzfeldt Jakob disease?
*humans, mammals, enviornment
19Portal of entry of Creutzfeldt Jakob disease?
digestive tract. open wounds
20Mode of transmission of Creutzfeldt Jakob disease?
*sporadic (hereditary, familiar FCJD)
*infectious (ingestion of infected meat, contact with infected tissue or enviornmental prion)