4. What is Creuzfelt Jacob
Disease ?
▹ CJD is a rapidly progressive,
invariably fatal neurodegenrative
disorder.
▹ It has 100% fatality rate within a
year.
▹ The annual incidence rate is
around one per million in the
world.
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5. What is CJD’s history ?
▹ The disease was first described
by German neurologist Hans
Gerhard Creutzfeldt in 1920.
▹ And shortly afterward by Alfons
Maria Jakob, giving it the name
Creutzfeldt–Jakob.
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6. What it looks like
?
Let’s get a visual reference of the disease.
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7.
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9. What Causes it ?
Let’s see what makes this disease kick.
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10. PRIONS
▹ Prions are proteins that can self–replicate. But they do
not multiply in the host organism.
▹ They are normally found in the neurons of the Central
Nervous System.
▹ An abnormal folding in Prion enables them to convert
themselves into diseased state causing CJD and other
Prion related diseases.
.
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15. Bad Prions increases
The number of Bad Prions will increase
exponentially and the process leads to a large
quantity of insoluble protein in affected cells.
This mass of misfolded proteins disrupts
neuronal cell function and causes cell death.
Mutations in the gene for the prion protein
can cause a misfolding of the dominantly
alpha helical regions into beta pleated sheets.
This change in conformation disables the
ability of the protein to undergo digestion.
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▹ Sporiadic (sCJD)
▹ Variant (vCJD)
▹ Familia (fCJD)
sCJD is a rare form in which the prion simply
mutates from the normal prion.
vCJD can be acquired from using contaminated
HGH or consuming meat (human or bovine)
fCJD can occur due to a mutated gene for Prion
Protein around 5-10% cases are such.
18. Do You have it ?
Let’s look at signs and symptoms.
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