Creutzfeldt-Jakob Disease (CJD) is a neurodegenerative disorder caused due to the abnormal folding of proteins. It is known as prion disease. This diseases progresses rapidly and is always fatal. The patient dies within one year of the onset of the disease.
The prions accumulate in the brain at very high levels and destroy brain cells and develop holes in the brain. It is a rare but serious disorder. One million cases of Creutzfeldt-Jakob Disease are diagnosed every year worldwide.
Types of Creutzfeldt-Jakob Disease (CJD)
- Sporadic CJD
- Variant CJD
- Familial or Inherited CJD
- Iatrogenic CJD
This is the most common and rare type of CJD. It is believed to have been caused due to the abnormal folding of the proteins. It mostly affects adults between 45 to 75 years of age. It develops between the ages of 60 to 65 years.
Variant CJD (vCJD)
vCJD is spread through the meat of the cow suffering from Bovine Spongiform Encephalopathy. The prion can also be transmitted through blood transfusion. It is controlled by preventing the infected meat from entering the food chain.
Familial or Inherited CJD
It is caused when one of the genes inherited from the parents is mutated in the offspring which causes prions to form in the adult. The symptoms are observed in the people in their early 50s.
In this, the infection is spread from an infected person to the healthy one through medical or surgical treatments. It can be caused if the surgical instruments used during the surgery of a diseased person are not cleaned properly before using it on the other patient. In the past, human pituitary growth hormones were isolated from dead patients and were used to treat the growth hormone problems. Most of the times the isolated hormones were from the people who were infected with CJD. This is how CJD was transferred to uninfected patients.
Also read: Prions
Symptoms of Creutzfeldt-Jakob Disease (CJD)
Following are the symptoms of CJD:
- Reduced memory and intelligence
- Personality changes
- Loss of body balance and reduction in coordination
- Slurred speech
- Low vision
- Gradual loss of brain function and movement
- Numbness in different body parts
At the final stages of the disease, the patient becomes bedridden. They need to be taken care of round the clock. They are not even aware of their surroundings.
Diagnosis of Creutzfeldt-Jakob Disease (CJD)
CJD can be diagnosed by carrying out the following tests.
MRI Scan- Radio waves and strong magnetic waves produce a detailed image of the brain that shows up abnormalities in the brain related to CJD.
EEG- The brain activity is recorded and abnormal electrical patterns are visible.
Lumbar Puncture- A sample of cerebrospinal fluid is drawn out from the spine with the help of a needle. It is then tested for a specific protein that indicates CJD.
Genetic Test- A simple blood test is done to detect any mutation in the genes which might result in the abnormal folding of proteins.
Tonsil Biopsy- A small tissue from the tonsils is taken and checked for any abnormal prions which are present only in vCJD.
Treatment of Creutzfeldt-Jakob Disease (CJD)
CJD has no specific treatment. Medications are provided to relieve the patients of the symptoms and make them as comfortable as possible. Some painkillers to relieve the pain and antidepressants to help with depression and anxiety are prescribed by the doctors.
In the advanced stages, the patients also need help in daily chores like washing, moving, sitting, and even peeing. Many people cannot even swallow and are therefore fed with the help of feeding tubes. They can be treated at homes depending upon the progression and severity of the disease.
Also read: Virus
Key Points Of Creutzfeldt-Jakob Disease
- It is a rare, degenerative neural disorder that results in dementia and eventually death.
- It starts around the age of 60 and is characterized by memory problems, vision problems, behavioural changes, and poor muscle coordination.
- There are three main types of CJD – sporadic CJD, hereditary CJD, and acquired CJD.
- A related disease is seen in cattle known as Bovine Spongiform Encephalopathy or mad cow disease. Therefore, the disease can also be transmitted from the beef of the infected animal.
- There is no specific treatment for the disease. Opiate drugs can be given to relieve pain if required. The patient should be made comfortable to ease the symptoms.
For more details on the Creutzfeldt-Jakob Disease or CJD and related topics, visit BYJU’S Biology website or download BYJU’S app.
Frequently Asked Questions
What is sporadic CJD? How can it be prevented?
The CJD that develops for no specific reason is known as sporadic CJD. This is the most common type of Creutzfeldt-Jakob disease. The sporadic CJD cannot be prevented. If you have a family history with a neurological disorder, genetic counselling might help in reducing the risk associated with the disease.
Is mad cow disease and CJD the same?
No, mad cow disease and CJD are not the same. CJD occurs only in humans, whereas mad cow disease occurs only in cattle. However, both are a type of Transmissible Spongiform Encephalopathy.
Is it possible to kill the prions by heating the beef?
Unlike several other pathogens, prions cannot be killed by heating. They are present in the nervous system and are proteins that trigger the misfolding of other proteins.
How can the prions be destroyed?
The prions should be denatured to such an extent that they no longer cause the misfolding of proteins. Subjecting the prions to a temperature above 900°F can destroy a prion.
Name a few other prion diseases.
Kuru in humans who practice cannibalism, scrapie in sheep and goats, fatal familial insomnia- an inherited human disease are some of the prion diseases other than Creutzfeldt-Jakob disease.
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