Have you ever heard of a condition called PML (Progressive Multifocal Leukoencephalopathy)? You may not be familiar with this name. The reason is that PML is a very rare infection that affects our brain. What happens in this is that the cells that produce myelin, a protective covering around the nerve cells in our brain, are gradually destroyed. Myelin is a substance that is like oil. This is what protects the neurons in the brain and spinal cord (our central nervous system). So when this myelin is lost, the white matter in the brain is damaged.
What does the name PML mean?
Now let's see what the letters in the name PML mean. Understanding this will give you a rough idea of the disease.
- P — Progressive: This means that the disease is gradually getting worse . This means that symptoms may increase over time.
- M — Multifocal: This means that the disease is not limited to one area of the brain, but affects multiple areas . It's like there are small lesions in multiple areas of the brain.
- L — Leukoencephalopathy: This word sounds a bit complicated, doesn't it? Simply put, it means that the disease mainly affects the myelin, or white matter, in the brain .
So, PML causes brain damage. This damage can lead to a variety of neurological problems. For example, mental impairment, vision problems, and difficulty walking. Because PML is a progressive disease, it can get worse over time. However, for some people, early treatment can slow the progression of the disease.
What are the early symptoms of PML?
For many people, the symptoms of PML start out very subtle. Also, the symptoms can vary depending on which part of the brain is affected by the infection. Think of it like when a branch of a tree dies, all the other functions on that branch stop working.
The first symptoms of PML may include:
- Clumsiness or lack of coordination
- Difficulty speaking or thinking
- Feeling weak in the body
As the infection progresses, you may also experience symptoms such as:
- Dementia, memory loss
- Speech loss
- Vision loss
- Behavioral and personality changes
Some people may also experience headaches or seizures, but this is rare. Unfortunately, PML usually gets worse over time, andIt can often be fatal. However, outcomes may vary depending on the patient's underlying medical conditions and response to treatment.
What causes PML?
PML is caused by a virus called JC virus (JCV) . The virus is named after the first letter of the name of the person who was first infected with the virus (John Cunningham). This virus is also known as human polyomavirus 2.
Surprisingly, about 85% of adults have the JC virus in their bodies. But experts are still not sure how the virus is transmitted to people. There is some evidence that children can get it through food or water. The virus has also been found in urine tests. So, it is possible that it can be transmitted through urine-oral contamination.
However, although many people have the JC virus in their bodies, it does not become active, it simply lies dormant. Therefore, no symptoms occur. However, in someone with a weakened immune system, this virus can become active in the body and develop into PML. It is like a sleeping enemy that wakes up and attacks us when we are weak.
Who is most at risk of developing this disease?
PML usually only occurs in people with very weak immune systems. You may also be at risk of developing this disease if you have any of the following conditions:
- HIV/AIDS infection
- Cancers of the blood and bone marrow, for example, leukemia
- Cancer of the lymphatic system, for example, lymphoma or Hodgkin disease
- Autoimmune diseases, such as rheumatoid arthritis, multiple sclerosis (MS), or lupus
Also, if you are an organ donor recipient and are taking immunosuppressant medicines to prevent your body from rejecting the organ, you may also be at risk.
How do doctors diagnose PML?
Your doctor will first examine you and listen carefully to your symptoms. Then, they can confirm PML by doing several tests that examine your brain and spinal cord. You may need to have tests like these:
- Brain MRI scan: This can take pictures of lesions in the white matter of the brain. It's like a camera looking inside the brain.
- Spinal tap/lumbar puncture: This involves taking a small sample of your cerebrospinal fluid (CSF), the fluid that surrounds your brain and spinal cord. This can check for the presence of the JC virus.
- Brain biopsy: This is done very rarely. It is only done when it is difficult to confirm the diagnosis.
How is PML treated?
The main focus of treating PML is to strengthen your immune system. Remember, diseases occur when our body's defenses are weakened. So, the most important thing is to strengthen that defense again.
- For example, if you have AIDS, you may be given antiretroviral therapy (ART) . These medications work by reducing the amount of HIV in your body. This treatment can help your immune system function to some extent.
- Some people may also benefit from a treatment called plasma exchange . This treatment removes some of the immunosuppressant drugs from your blood. This treatment can also help your immune system fight the JC virus by boosting its function.
Scientists are currently testing several new drugs to treat PML. The US Food and Drug Administration (FDA) has given special permission for some people to use these drugs, but results have been mixed.
When should I see a doctor?
If you know that your immune system is weak and you feel like you are gradually developing symptoms that you think may be related to Progressive Multifocal Leukoencephalopathy (PML), it is very important to see a doctor immediately and get checked out.
What questions should I ask my doctor?
When you see your doctor, you can ask questions like these:
- What is my risk of developing PML?
- Is there anything I can do to reduce this risk?
- What symptoms should I look out for in PML?
- What are the risks of taking immunosuppressant medications?
- Are there other treatments for my condition?
- What are the risks and benefits of current treatments?
By asking these questions, you can gain a better understanding of the disease and its treatment.
What is the life expectancy of a PML patient?
This is a really difficult topic to talk about. PML is a progressive disease. This means that symptoms get worse over time. The life expectancy of a person with PML depends on the severity of the infection and the treatment they receive. Between 30% and 50% of people die within the first few months after diagnosis. People who live longer continue to have symptoms, and those symptoms get worse. These symptoms can lead to serious problems, such as loss of speech and vision loss.
However, new studies have shown that a small number of people with HIV-infected PML can stabilize their disease and live longer. Before the advent of antiretroviral therapy (ART) to treat HIV infection, only 10% of people with PML survived more than a year. Now, with ART, the one-year survival rate has increased to 50%. In some cases, ART can help the immune system fight off the JC virus.
Is it possible to completely recover from PML?
People who develop PML while taking immunosuppressant drugs may recover after stopping the drugs. People who have successfully treated with ART can also recover from PML. However, 80% of people who survive PML do not have their symptoms return. This is because new myelin is not produced in the damaged areas. Permanent neurological damage is common. However, some people can live up to 15 years after PML has cleared.
What are the expectations for those undergoing plasma exchange?
If your PML symptoms started because of an immunosuppressant drug, your symptoms may go away once you stop taking that drug. You can also have plasma exchange to remove some of the immunosuppressant drugs from your body. The goal of plasma exchange is to boost your immune system. For some people, plasma exchange may slow the progression of PML and prolong life. However, the benefits of this are not yet fully understood.
Many people may continue to have symptoms of PML after stopping a medication or after receiving plasma exchange. Others may develop neurological problems. There are also other risks of stopping a medication. So, it's important to talk to your doctor when making decisions about your medications.
How do I know if I am at risk of developing Progressive Multifocal Leukoencephalopathy (PML)?
If you have a medical condition that weakens your immune system, ask your doctor about your risk of developing PML.
Do other medical conditions increase my risk of developing PML?
People with diseases such as multiple sclerosis (MS) or Crohn's disease should also have a blood test to check for conditions such as HIV that increase the risk of JC virus infection.
Doctors often prescribe a drug called Natalizumab (Tysabri®) to treat MS or Crohn's disease. This drug may increase the risk of developing PML.
Even if you have inactive JC virus and no symptoms, you may be able to take natalizumab. However, it is important to talk to your doctor about the risks and benefits.
Finally, things to remember (Take-Home Message)
Progressive Multifocal Leukoencephalopathy (PML) is a rare brain infection caused by the reactivation of the JC virus. Most people have the JC virus dormant in their bodies. It does not cause any harm to people who do not have a weakened immune system.
If you have a weakened immune system, it's important to be aware of the symptoms of PML and work with your doctor to do so.
This disease usually gets worse over time. However, there are treatments that can help slow the progression of the disease and prolong your life. So, don't give up hope. The most important thing is to seek medical advice as soon as possible.
👩🏽⚕️ Additional questions (FAQs)
💬 What is PID (Pelvic Inflammatory Disease), a disease that blocks the fallopian tubes?
PID is a serious infection of the entire female reproductive system (uterus, fallopian tubes, and ovaries) caused by 'germs/bacteria' that enter the vagina through the vagina. It is most often caused by untreated sexually transmitted diseases (gonorrhea/chlamydia).
💬 How do you recognize an infection like this inside the body?
The main symptom of this is a constant 'severe lower abdominal pain'. There is also an unusual, yellow/green, foul-smelling discharge from the vagina. There is pain during sex (dyspareunia), and symptoms such as burning during urination and fever/fatigue may also occur.
💬 Will I never be able to have babies because of this?
If you find out in advance and take the prescribed antibiotics (Antibiotics - Ceftriaxone / Doxycycline) continuously for about 2 weeks, most people will recover. However, if you ignore this, the bacteria will go and completely 'scar and block' the fallopian tubes (Scar tissue). Then there is a great risk of permanent infertility and ectopic pregnancy.
` PML, Progressive Multifocal Leukoencephalopathy, JC virus, brain infections, immune system, neurological diseases, myelin
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