Imagine that someone you know well and who was previously healthy suddenly starts acting a little strange. They lose their memory, have difficulty walking , and slur their words when they speak. If these things happen at an unimaginable rate over a few months, it could be due to a very rare but very serious brain condition. Today we are talking about one such disease, Creutzfeldt-Jakob Disease, or CJD as we all call it. Although this is a somewhat scary topic, it is very important to be well informed about it.
Are CJD and "mad cow disease" the same thing?
Many people confuse CJD with "Mad Cow Disease." However, the two are not the same.
Simply put, Bovine Spongiform Encephalopathy (BSE) is a brain disease that only affects cows. When it occurs, the cows' nervous systems are damaged, causing them to become immobile and behave strangely.
However, there is a type of CJD that can be contracted by humans by eating the meat of a cow infected with mad cow disease . It is called Variant CJD (vCJD) . But this is very, very rare . There have been very few cases reported worldwide. So this is not how the CJD disease we usually talk about occurs.
Why does this CJD disease occur?
The main cause of CJD is an abnormal change in a protein in our body. This abnormal, mutated protein is called a prion .
Think of a healthy protein in our brain as a beautifully folded piece of paper. This mutant protein called a prion comes along and folds this good piece of paper incorrectly. Then that misfolded piece goes on to fold the other good pieces of paper incorrectly. And so, the brain cells are gradually destroyed. If you look at it under a microscope, this damaged brain tissue looks like a sponge.
There are three types of CJD depending on how the prion is formed.
| Type of CJD | Reason and explanation |
|---|---|
| Sporadic CJD | This is the most common type. For no apparent reason, a healthy protein suddenly mutates and becomes a prion. Most CJD patients have this type. |
| Familial CJD | This is caused by a genetic defect. If one of the parents has a mutated gene that causes this disease, it can be passed on to the children. But this is also very rare. Only about 10%-15% of CJD patients fall into this category. |
| Acquired CJD | This is the rarest type. A person can contract CJD through contaminated medical equipment (e.g. during surgery), an organ transplant, or contaminated growth hormone injections. This risk has been greatly reduced by the strict safety measures in hospitals today. |
What are the symptoms of CJD?
The most dangerous thing about CJD is that the symptoms start very quickly and get worse very quickly. Often, these symptoms are similar to those of dementia.
The main visible features are:
- Memory loss and confusion
- Difficulty walking and loss of balance
- Sudden muscle twitches (jerky movements)
- Personality changes (suddenly becoming angry, agitated)
- Difficulty thinking and making decisions
- Vision problems
- Insomnia and depression
Difference between CJD and Alzheimer's disease
These symptoms may remind you of Alzheimer's disease. But the main difference is the speed . In Alzheimer's disease, things like memory loss occur slowly over years. But in CJD, the patient's condition worsens very quickly, within weeks or months of the onset of these symptoms.
How to diagnose this disease exactly?
Unfortunately, there is no single test that can confirm CJD. Doctors make the diagnosis based on the patient's symptoms and how quickly they develop. However, if the disease is suspected, several tests are performed to confirm it.
- MRI (Magnetic Resonance Imaging) scan: This can take detailed pictures of the brain. It can look for specific changes in the brain that occur in CJD.
- EEG (Electroencephalogram) test: This test measures the electrical activity of the brain using small sensors attached to the scalp. This test can show patterns that are characteristic of CJD patients.
- Lumbar Puncture: Using a very fine needle, a small amount of cerebrospinal fluid is taken from inside the spinal cord and tested for specific proteins seen in CJD.
The only way to confirm this disease with 100% certainty is to take a brain tissue sample (biopsy). However, taking such a sample from a living person's brain is very risky for both the patient and the doctor, so it is usually not done. Often, the disease is definitively confirmed after the patient's death.
How the disease worsens over time
CJD progresses through several stages over time, making it a very painful experience for both the patient and their family.
| Stage of the disease | Expected features |
|---|---|
| Early stage | Slurring of words while speaking, dizziness, numbness in parts of the body, seeing things that are not there (hallucinations), getting angry for no reason, withdrawing from society, and insomnia. |
| Later stage | Inability to control bowel and bladder control, severe difficulty swallowing and speaking, severe memory loss, paranoia, bed rest, coma. |
About 70% of people with CJD die within a year of diagnosis. Hearing this news can make you feel scared, sad, and angry. That's normal. Don't suffer alone at a time like this. It's important to talk to your doctor and get the support you need.
Is there a treatment for CJD?
Unfortunately, there is still no cure or treatment for CJD. Researchers have tried several drugs, but none have been successful.
The only thing that can be done right now is to control the symptoms . That means:
- Giving painkillers for pain.
- Giving medication for muscle stiffness and spasms.
- Providing the necessary facilities to make the patient as comfortable as possible.
Once the disease becomes severe, the patient will need full-time care. Research is ongoing to find treatments for this disease in the future. If you are interested, you can ask your doctor about clinical trials that are testing new treatments.
Take-Home Message
- Creutzfeldt-Jakob Disease (CJD) is a very rare and rapidly spreading fatal brain disease.
- This is caused by a mutated protein called a prion that destroys brain cells.
- CJD and "Mad Cow Disease" are two different diseases. However, there is a very rare form of CJD (vCJD) that can be contracted from eating infected beef.
- There is no cure for this disease yet. All that can be done is to control the symptoms and make the patient comfortable.
- If someone you know experiences significant changes in memory, behavior, and physical function in a short period of time, see a doctor immediately . A prompt diagnosis is essential for proper planning and care.
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