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Creutzfeldt-Jakob Disease (CJD)
Transcript of Creutzfeldt-Jakob Disease (CJD)
Creutzfeldt-Jakob disease (CJD) is a rare, progressive, and fatal brain disorder caused by an abnormal
Prions are misfolded proteins that damage neurons.
After a long incubation time (replication), PrP causes the brain tissue to rapidly degenerate with the formation of plaques (build up of prion proteins) and vacuoles (empty spaces).
sCJD (~90 %) spontaneous
transformation of normal proteins into abnormal PrP.
Signs and symptoms of CJD include memory loss, worsening dementia, personality changes, deterioration in motor skills, changes in gait, confusion, disorientation, hallucinations, lack of coordination, muscle stiffness, muscle twitching, sleepiness, trouble speaking, sudden jerky movements, and seizures.
As the disease progresses, the individual can acquire urinary
because voluntary control is lost.
A nervous system and mental examination will show memory problems and intellectual declines ("neuro tests").
A motor system examination (an exam to test muscle reflexes, strength, coordination, and other physical functions) will show abnormalities.
An eye examination shows areas of blindness that the person may not notice.
CT, MRIs, EEGs, spinal taps, and blood tests will be used to help diagnose CJD.
Urinary tract infections (UTIs) can also occur.
Proper antibiotics will be needed to treat the UTI.
CJD is characterized as an transmissible
Spongiform refers to the appearance of the infected brain which resembles a sponge.
The human form of mad cow disease (bovine spongiform encephalopathy or BSE) is believed to be the cause of
Creutzfeldt–Jakob (vCJD) disease in humans with the transmission of infected beef.
Myoclonus (jerky movements)
Ataxia (balance and coordination dysfunction)
Three Body Systems
What is the etiology of Creutzfeldt-Jakob Disease (CJD)?
d. Cow's meat
What is the initial clinical manifestation of CJD?
What is the main course of treatment for CJD?
a. There is no treatment
b. Palliative treatment
The protein takes a different folded shape than the normal protein. Once they appear, abnormal prion proteins aggregate, or clump together. Investigators think these protein aggregates may lead to the neuron loss and other brain damage seen in CJD.
CJD is characterized by rapidly progressive dementia. Initially, individuals experience personality changes, including impaired memory, judgment, and thinking.
b. Palliative treatment
There is no CURE but there are treatments to help manage the rapidly progressing symptoms.
Thanks for your attention.
Any additional questions?
- classic CJD has been recognized since the early 1920s. The risk of sCJD increases with age (>50 years of age). There were 222 reported cases of sCJD in 2014 in the US.
- roughly one case per 1 million population per year, worldwide.
-sCJD can afflict anyone. The disease affects both men and women of diverse ethnic backgrounds usually between the ages of 50 to 75 years.
- about 90% of individuals die within 1 year. CJD is ultimately fatal. Average lifespan is 6-12 months.
- CJD cannot be transmitted through the air or through touching or most other forms of casual contact. Exposure to brain tissue and spinal cord fluid from infected individuals should be avoided.
There is no treatment that can cure or control CJD.
Current treatment is aimed at alleviating symptoms and making the patient as comfortable as possible (
Opiate analgesics can help relieve pain.
Benzodiazepines and anti-epileptics may help relieve involuntary muscle jerks.
Ages of sCJD and vCJD
An infected prion invades the brain where it replicates and produces more abnormal prions. The prion also causes normal proteins to fold abnormally.
After this replication time, tissue destruction, plaque formation, and the formation of vacuoles develop and disrupts the nervous system.
This compares a normal prion to the
abnormally folded prion in CJD.
As the disease progresses, complications to the urinary system also occurs. This requires more support and basic care for the individual.
The most important factor regarding CJD is that it causes a complete shut down of the muscular system with resulting paralysis.
Problems with muscular coordination:
Myoclonus (Involuntary muscle jerks)
Eventually lose the ability to move and speak
The degeneration of the neurons in CJD causes the brain to become unable to initiate and control muscle movement.