Do you sometimes feel like your legs and arms are slowly going numb, or your strength is decreasing? Do you feel like you're not as strong as before when you walk, and maybe you even lose your balance? Although these are symptoms that you may not pay much attention to, sometimes there can be a more serious reason behind them. That's what Primary Lateral Sclerosis is, or PLS for short.
Let's simply see what this PLS means?
Simply put, PLS is a progressive neuromuscular disease. It causes your muscles to gradually weaken and/or become stiff. Symptoms often start in your legs. Over time, this weakness and stiffness can spread to other muscles in your body.
Think of it this way: our muscles work like an electrical device. They need to receive the 'current', the message from the brain, exactly as it should. In PLS, the nerve cells called Upper Motor Neurons (UMN), which are like 'wires' that carry these messages, are damaged.
Unfortunately, there is currently no cure for PLS. So treatment is mainly focused on controlling your symptoms and helping you to do everyday tasks more easily. For example, helping you use a cane or walker.
How common is PLS?
PLS is actually a very rare condition. There are no statistics yet on exactly how many people develop it.
What is the difference between PLS and ALS? Can PLS be ALS?
You've probably heard of ALS, or Amyotrophic Lateral Sclerosis. Both PLS and ALS are diseases of the nerves and muscles. But there are important differences between the two.
The main difference is that PLS only affects your upper motor neurons (UMNs) . These are the nerves that carry messages from the brain to the spinal cord. In ALS, these UMNs are also affected, as are the lower motor neurons (LMNs) , which carry messages from the spinal cord to the muscles.
Now you may be wondering, 'So, can someone with PLS later develop ALS?' Yes, there is a chance that it can. Sometimes the early symptoms of ALS can be similar to PLS. If you have symptoms that only affect the UMN, you may initially be diagnosed with PLS. But if you later develop ALS symptoms that affect both the UMN and LMN, your doctor may change your diagnosis to ALS.
In fact, many cases that look like PLS can be the first signs of ALS that affects the UMN (`UMN-predominant ALS`). That's why doctors say that to be definitively diagnosed with PLS, you need to have symptoms for at least three to four years .
What are the symptoms of PLS?
The symptoms of PLS come on very slowly. You won't notice a big difference all at once.
Symptoms that may be seen in the early stages:
At first you may feel things like this:
- Stiffness in the legs. It may feel like your legs are stuck.
- The muscles in your legs become weak. You may feel like you're not as strong as you used to be.
- It becomes difficult to walk, or to keep your balance. You may stumble or fall.
- Muscle spasms or painful cramps occur.
Symptoms that may appear as the disease progresses:
As the disease progresses, additional symptoms such as these may appear:
- The muscles in the fingers, hands, and arms also become stiff and weak. It can be difficult to grasp small objects or write.
- Difficulty controlling urination. This can lead to urinary urgency and incontinence.
- Pain occurs in the lower back and neck.
Rare symptoms affecting the muscles of the tongue:
This is very rare, but some people can also have their tongue muscles affected. If this happens:
- Slurred speech (dysarthria) occurs. It becomes difficult to pronounce words clearly.
- Difficulty swallowing (dysphagia). You may feel like you are choking when you swallow food or drink.
What causes PLS?
In most cases, we still don't know the exact cause of PLS. That's the truth.
However, very rarely, there is a type of PLS that affects young children and young adults (`juvenile primary lateral sclerosis`). This is caused by a change in their DNA at the time of conception.
What happens to our bodies from PLS?
PLS is a neuromuscular condition. It mainly affects the motor neurons in your brain and the nerve fibers that extend from those neurons to the spinal cord. These are also called the upper motor neurons ( UMN).
Imagine, when you want to move your leg, your brain sends a message. This message travels along nerve fibers to neurons in the spinal cord, and from there to the voluntary muscles in your leg. When the muscles receive this message, they move.
In PLS, these UMN nerve cells gradually die (degeneration). Then, the muscles do not receive the necessary messages properly, so the muscles do not function as expected.
Is this a hereditary disease?
PLS is not usually hereditary. This means that you can develop it even if no one in your family has had it. Most of the time, it occurs randomly, with no family history of the disease.
Who is at higher risk of developing PLS?
PLS can actually affect anyone. It is usually diagnosed around the age of 50. However, it can affect people under the age of 50, older people, and even children. It is slightly more common in men.
What complications can occur due to PLS?
As PLS progresses, you may find it difficult to walk without assistance. You may need to use a cane, walker, or wheelchair. In addition, as your muscles weaken, you may lose your balance and fall more easily. If you do, you may be injured.
How is PLS diagnosed?
A doctor diagnoses PLS after a physical examination, a neurological examination, and other specialized tests. They will review your symptoms and try to rule out other conditions that have similar symptoms, such as ALS or multiple sclerosis. Tests may include:
- Blood tests.
- Electrodiagnostic examinations - These check how well your nerves and muscles are working. Examples include nerve conduction studies and needle electrode studies.
- An MRI (Magnetic Resonance Imaging) test of the brain and spinal cord.
- Cerebrospinal fluid (CSF) analysis. This is done by taking a small amount of fluid from your spine (a lumbar puncture or spinal tap) to look for abnormalities specific to neuromuscular diseases.
What are the treatments for PLS?
Treatment for PLS is aimed at helping you manage your symptoms. This may include:
- Medications to reduce muscle stiffness, limping, and difficulty swallowing.
- Physical therapy to reduce muscle weakness, improve muscle flexibility, and improve joint range of motion.
- Devices that help with daily activities and mobility. For example, a cane, a walker, or a wheelchair.
- Speech therapy or devices to help with speech difficulties.
Medications given for PLS symptoms:
Some of the medications commonly prescribed for PLS symptoms include:
- For muscle stiffness: `(Baclofen)` and `(Tizanidine)`
- Muscle cramps: `(Quinine)`
- To relax muscles: `(Diazepam)`
Are there any side effects of the treatment?
Yes, like all medications, this one can have side effects. Side effects vary depending on the medication. Therefore, it is very important to talk to your doctor about the possible side effects before starting any medication.
Can PLS be prevented or cured?
Since the exact cause of PLS is not known, there is currently no way to prevent it from developing.
Also, sadly, there is currently no treatment that can completely cure PLS.
What is the life expectancy of someone with PLS? What can they expect?
PLS does not directly affect your life expectancy. A person with PLS can have a normal lifespan, just like someone without the disease.
How quickly your symptoms appear and how quickly they worsen varies from person to person. In general, primary lateral sclerosis progresses very slowly, over years, sometimes decades. But if you suddenly feel your symptoms getting worse, see your doctor right away.
Medications can help reduce your symptoms and help you carry out your daily activities without interruption. You may want to consider using a cane, walker, or wheelchair to help you feel more secure and confident when getting around.
When should you see a doctor?
If you notice any gradual changes in your muscles, such as stiffness or weakness, talk to your doctor. If you have been diagnosed with PLS, if any medication you are taking is causing your symptoms to worsen, or if you are experiencing side effects, tell your doctor about that as well.
Most importantly: If you fall or are injured in an accident, go to the emergency room immediately.
Questions to ask your doctor
If you have PLS, you will have many questions to ask your doctor. For example:
- What treatments can help control my symptoms?
- What are the side effects of the treatment?
- How do I know if my condition will get worse over time?
- What exercises can I do to maintain my muscle function?
- Do I need to use a cane or a walker?
Finally, a Take-Home Message
Adjusting to a progressive condition that affects your ability to move your body can be really difficult. Some of the things you used to enjoy doing can gradually become difficult. Although primary lateral sclerosis (PLS) progresses slowly, it can affect your daily life.
If these physical changes are affecting your mood, you may find it helpful to talk to a mental health professional . It's nothing to be ashamed of, and many people need help at times like these.
If you notice a change in your muscle strength, see a doctor right away. They can prescribe medications or mobility devices to help you become more independent and comfortable. Remember, you are not on this journey alone.
` Primary lateral sclerosis, PLS, muscle weakness, neuropathy, motor neurons, ALS, symptoms
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