Imagine, someone you know suddenly starts to change their memory, behavior, and gait very quickly. Have you ever heard of a disease that is difficult for doctors to detect and that progresses very quickly? Today we are going to talk about one of those strange and very rare diseases. We call these prion diseases.
Simply put, what are these prion diseases?
Prion diseases are a group of diseases that affect your brain and nervous system. They can cause
severe dementia or difficulty controlling your body, and they can get worse very quickly. They are very rare, with only about 350 cases reported each year in the United States. Let's look at what causes them.
Prions are tiny proteins that are found in our brains. But for some unknown reason, these proteins misfold, tangle, and become misshapen. The most dangerous thing is that this misfolded prion protein can cause other healthy proteins to become misfolded as well. Just like a bad friend can make others sick. These misfolded proteins accumulate in the brain and start to destroy brain cells (neurons). This is what causes the symptoms.
Many people confuse prion diseases with Alzheimer 's disease . Although both are diseases that cause memory loss, there is a big difference between the two. Alzheimer's disease usually gets worse slowly over many years. In prion disease, the condition worsens very quickly, over a short period of time, usually a few months. And, like Alzheimer's disease, there is still no cure for prion diseases.
What are the main types of prion diseases?
Prion diseases can affect both humans and animals. There are several main types that affect humans. Let's take a look at what they are.
| Disease Name | A short description |
|---|
| Creutzfeldt-Jakob disease (CJD) | This is the most common prion disease in humans. It usually affects people over the age of 60. |
| Variant Creutzfeldt-Jakob disease (Variant CJD - vCJD) | This can also happen to young people. It has been found that eating beef infected with "Mad Cow Disease" can be transmitted. |
| Gerstmann-Sträussler-Scheinker syndrome | This is also a very rare, hereditary genetic disease. |
| Fatal familial insomnia | This is also a genetic condition that is passed down from generation to generation. The main symptom is severe insomnia. |
| Kuru | A disease spread by a custom of eating human flesh among some tribes in Papua New Guinea. This is now almost unheard of. |
What are the symptoms of a prion disease?
Symptoms of a prion disease can appear suddenly. These include sudden changes in your mood, memory, and movements.
| Affected sector | Visible symptoms |
|---|
| Mental and behavioral | Severe anxiety or depression Sudden changes in behavior or personality Rapidly progressive forgetfulness (Dementia) |
| Physical and movement | Balance problems, Unsteady walk, Loss of muscle control, sudden jerking or trembling, Slurred speech, Trouble swallowing |
| Other features | Seizures Vision problems |
Causes and risk factors for developing this disease
Most of the time, diseases like CJD develop without any clear cause, but some risk factors have been identified.
- Family History : About 15% of people develop these diseases due to a genetic defect (a defect in the gene called `PRNP`) that is passed down through generations.
- Infections : This is very rare. Sometimes, this disease can be transmitted when transplanting tissue from someone with the disease, or if the equipment used during surgery is not properly sterilized.
- Eating infected meat: Eating beef infected with " mad cow disease " (Bovine Spongiform Encephalopathy ) can cause a form of the disease called vCJD. When the condition spread in Europe in the 1990s, a small number of people died from the disease. Now, strict regulations have been put in place regarding the feeding of cattle and the donation of blood, so this risk has been greatly reduced.
How is a prion disease diagnosed?
It can be difficult to diagnose this condition because the symptoms can be similar to other conditions. If you have symptoms like forgetfulness, your doctor will first rule out other causes, such as a stroke or a brain tumor. The following tests may be done:
- Spinal Tap / Lumbar Puncture: This involves inserting a thin needle between two of your vertebrae and taking a small sample of the fluid that surrounds your brain and spinal cord (cerebrospinal fluid). The levels of certain proteins in it can be tested to get an idea of the disease.
- MRI scan (Magnetic Resonance Imaging): This uses powerful magnets and radio waves to take detailed pictures of the brain. It can sometimes show specific changes in the brain seen in prion diseases.
- CT scan (Computerized Tomography): A series of X-rays are taken together to create a clear image of the brain.
The most important thing: The only way to be 100% certain that someone has a prion disease is to take a small piece of tissue from the brain (a brain biopsy). But this is a very risky surgery. So this is only done if there is a suspicion that it may be another treatable disease.
Treatment and prevention of the disease
At this time, there is no cure for prion diseases. Current treatments only control symptoms and provide some relief to the patient. This includes painkillers, antidepressants, and anti-anxiety medications. As the condition of a person with prion disease worsens day by day, they will inevitably need help with daily activities. They may need to have a catheter inserted to drain urine, saline to keep the body hydrated, and tube feedings if they are unable to eat.
Can the disease be prevented?
There is no way to prevent hereditary prion diseases, but these steps can help reduce the risk of spreading the disease through infection or food:
- Properly cleaning and disinfecting medical equipment.
- If you have a prion disease or have a family history of it, avoid donating tissue or organs.
- Eating only meat that is certified safe.
The spread of "mad cow disease" has now been very well controlled due to strict regulations imposed by many countries around the world regarding the feed and beef given to cows.
Take-Home Message
- Prion diseases are a group of very rare but serious diseases that damage the brain.
- In these diseases, memory, behavior, and body movements deteriorate very rapidly . This rapidity is the main characteristic that distinguishes it from diseases like Alzheimer's.
- It is difficult to diagnose the disease precisely, and several specific tests are required to do so.
- Although there is currently no specific cure for this, the symptoms can be controlled and the patient can be provided with relief.
- If you or someone you know notices a sudden, unexplained change in their memory, personality, or movements, it is very important to see a doctor immediately for advice .
Prion Diseases, Creutzfeldt-Jakob disease, CJD, brain disease, nervous system, memory loss, dementia, mad cow disease, mad cow disease
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