CREUTZFELDT-JAKOB

DISEASE By Kuah Kia Yue Chloe

Creutzfeldt-Jakob disease (CJD), is a rare and fatal disease that causes rapid deterioration of the
victim’s brain. It’s a type of prion disease, also called Transmissible Spongiform Encephalopathy
(TSE), that is a rare progressive neurodegenerative disorder that affects both humans and animals.

Due to the lack of research, Creutzfeldt-Jakob

disease is believed to be caused by the build-
up of prions. Prions is an abnormal, self-
replicating protein, unlike other pathogens that
uses genetic material to replicate itself, prion is
a deformed version or abnormal folding of a
normal cellular protein, which is also called as
prion proteins. When protein becomes prions,
it can bind with other proteins, thus inducing
abnormal folding of other proteins, and it will
continue to corrupt other normal proteins into
prions. The abnormal folding of prion proteins
causes it to be infectious and resistant to the
commonly used sterilization methods, thus the
amount of diseased prion protein will increase
and destroy the victim’s central nervous
system.

Source : Soton Brain Hub

Creutzfeldt-Jakob disease is usually diagnosed by physical and neurological exams, diagnostic tests
and imaging (such as Brain MRI), and lab tests. There are 4 types of CJD, Sporadic CJD, Familial
CJD, Iatrogenic CJD and Variant CJD. Sporadic CJD is the most common type of CJD cases. The
cause of sporadic CJD is still unknown, but it is generally found in old people, usually around 50
years old and above. The most common symptom of sporadic CJD is dementia, and the infected might
also show signs of clumsiness, ataxia, mental deterioration, myoclonus, akinetic mutism and visual or
cerebellar problems. Majority of the patients diagnosed with sporadic CJD usually die within months.
Familial CJD (also called as Genetic CJD), is caused by a genetic mutation of a prion gene inherited
from one or both parents. It’s usually found around the age range of 30 to 80 years old. The symptoms
of familial CJD are similar to the symptoms of sporadic CJD, the difference between the two is that
familial CJD takes longer for the symptoms to progress, usually around two years, unlike in sporadic
CJD the symptoms rapidly worsen in within months.

Iatrogenic CJD happens when one “acquires” it from various sources, such as medical procedures like
organ or tissue transplants, dura matter and corneal grafts, use of contaminated human growth
hormone or contaminated surgical equipment, usually neurosurgical instruments and stereotactic EEG
depth electrodes which are both used for brain surgery. Although variant CJD and CJD may share
similar names, both of these prion diseases aren’t actually the same, instead variant CJD is related to
Bovine Spongiform Encephalopathy (BSE), which is also known as Mad Cow Disease. Researchers
believe that variant CJD is caused by consuming a product that is contaminated by BSE, such as beef
from a cow that has BSE. The symptoms of variant CJD are dementia, delayed neurologic signs and
prominent psychiatric symptoms. Just like BSE, the symptoms of variant CJD can take years to
appear, but when it does, brain degeneration progresses rapidly in just a few months. Patients that are
diagnosed with variant CJD usually die within a year.

Unfortunately, there is no proven cure for both Creutzfeldt-Jakob disease and variant Creutzfeldt-
Jakob disease. There isn’t a way to slow down the progress of it either. Currently, the only treatment
providable would be supportive to the victims, making sure they are as comfortable as possible and
reducing symptoms with medicines, though usually CJD often progresses too rapidly for treatments to
help before the condition worsens.

References used :
(Image)
https://www.facebook.com/SotonBrainHub/photos/creutzfeldt-jakob-disease-cjd-is-a-very-rare-and-
fatal-degenerative-brain-disord/1425238197659593/
(Videos)
https://www.youtube.com/watch?v=ehsu0pNMJ9E
https://www.youtube.com/watch?v=Pxojz6grwcU
(Articles)
https://www.abc.net.au/news/2022-07-25/end-to-two-decades-long-mad-cow-ban-allows-for-more-
blood-donors/101265116
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https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4978610/
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https://www.cdc.gov/prions/index.html
https://www.cdc.gov/prions/cjd/index.html
https://www.cdc.gov/prions/cjd/about.html
https://www.cdc.gov/prions/cjd/diagnostic-criteria.html
https://www.cdc.gov/prions/cjd/infection-control.html
https://pubmed.ncbi.nlm.nih.gov/22411235/
https://www.reviewofophthalmology.com/article/mad-eye-disease-should-you-worry
https://www.researchgate.net/figure/Various-types-of-CreutzfeldteJakob-disease-50_tbl1_258045114
https://www.researchgate.net/figure/Differences-in-subtypes-of-CJD_tbl1_318772677
https://www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/symptoms/
https://www.cdc.gov/prions/vcjd/about.html