Have you ever felt like your legs were going numb when you were climbing stairs, your words were getting slurred when you were talking, or something suddenly falling off your hand? You might think these are normal, but if these symptoms persist, it's a good idea to be a little concerned. Because these could be early signs of a condition called Motor Neuron Disease (MND) . So, let's talk about this in a little more detail today.
What is motor neurone disease (MND)?
Simply put, motor neurone disease (MND) is a group of diseases that affect our nervous system. It causes our motor neurons to gradually die. Now you might be wondering what these motor neurons are. These are the nerve cells that control our muscles. These motor neurons are essential for all of our functions, from breathing, talking, swallowing, and walking.
Imagine, from our brain (which we call upper motor neurons ) messages come to our spinal cord. From there, those messages go through lower motor neurons to the muscles in our body. These upper motor neurons are what tell the lower motor neurons, "Okay, now move this muscle like this."
Now, what happens when these lower motor nerves can't send messages to the muscles? The muscles gradually begin to weaken and shrink (muscle atrophy) . Also, when the upper motor nerves can't send signals to the lower motor nerves, the muscles become stiff and overreactive ( hyperreflexia ). This makes it difficult for us to make voluntary movements, and they happen very slowly. Over time, we may even lose the ability to walk and control other movements.
The important thing is that there is currently no cure for motor neurone disease. These are progressive diseases that get worse over time. However, there are treatments that can help reduce the impact of the condition.
What types of MND are there?
Doctors classify MND according to whether it affects the upper motor nerves, the lower motor nerves, or both. There are several main types of MND:
`Amyotrophic lateral sclerosis (ALS)`
This is the most common type of MND. Some people also call it Lou Gehrig's disease . ALS affects both the upper and lower motor nerves. This can cause rapid loss of muscle control, eventually leading to paralysis . Many doctors sometimes use the terms ALS and motor neurone disease interchangeably.
`Progressive bulbar palsy (PBP)`
This attacks the lower motor nerves that are connected to our brainstem (bulbar region) . Our brainstem controls the muscles needed for things like speaking, chewing, and swallowing. Another name for PBP is ``progressive bulbar atrophy``.
`Primary lateral sclerosis (PLS)`
This affects only the upper motor nerves. Most often, this disease first affects the legs. Then it affects the arms, hands, and trunk. Finally, it can affect the muscles used for speaking, swallowing, and chewing food.
`Progressive muscular atrophy (PMA)`
It affects only the lower motor nerves. The condition is more common in men, and it tends to develop at a younger age than other adult-onset MNDs. The first symptoms are weakness in the arms, which then spreads to the lower body. It can also affect the trunk and breathing.
Spinal muscular atrophy (SMA)
This is a hereditary condition that affects the lower motor nerves. It is also considered a leading genetic cause of infant mortality . Mutations in the `SMN1` gene result in the loss of the `SMN` protein . This gradually destroys the lower motor nerves, causing muscle wasting and weakness, and ultimately death.
Kennedy's disease
It is linked to a gene on the X chromosome in men. It is caused by mutations in the androgen receptor gene. Over time, weakness develops in the arms and legs, often starting in the pelvic or shoulder areas. Pain and numbness in the arms and legs may also occur.
Post-polio syndrome (PPS)
It occurs in people who have recovered from polio. It can occur up to four decades after their original illness. Polio can cause significant damage to the motor nerves. Symptoms of PPS include muscle and joint weakness, fatigue, pain that increases over time, muscle twitching and wasting, and an inability to tolerate cold.
What are the symptoms of MND?
The symptoms of MND do not come on suddenly, but gradually. In the early stages, they may not be very obvious.
Early symptoms
- Difficulty climbing stairs or frequent stumbling due to weakness in the legs or ankles.
- Slurred speech (dysarthria) .
- Difficulty swallowing food .
- Weakening of the grip on something you are holding. For example, it may be difficult to button a shirt, open a bottle, or things in your hand may constantly fall to the floor.
- Muscle twitches and cramps .
- Weight loss due to thin arms and legs.
- Unable to stop laughing or crying at inappropriate times (pseudobulbar affect) . This is like laughing when you feel sad, or feeling sad when you feel happy.
Other symptoms
In addition to these initial symptoms, other symptoms may appear:
- Difficulty breathing (breathlessness) .
- Difficulty breathing while lying in bed.
- Frequent chest infections .
- Sleep disorders (disturbed sleep) .
- Attention and memory impairments .
- Confusion .
- Morning headache .
- Fatigue .
What causes MND?
As we've talked about before, MND is caused by problems with our motor neurons. These cells gradually stop working properly over time. However, researchers still don't know exactly why this happens .
MND is hereditary
Some MND conditions are hereditary, meaning they are passed down through family members through genes. In these cases, the disease is caused by a change in a single gene. These can be inherited in several main ways:
- ``Autosomal dominant``: In this case, even if you inherit a single copy of the altered gene from only one of the affected parents, there is still a risk of developing the disease.
- ``Autosomal recessive``: In this case, in order to develop the disease, you must receive two copies of the altered gene from both parents.
- `X-linked inheritance`: In this, a mother carries this gene on an X chromosome and passes the disease on to her male children.
Other reasons
Non-hereditary MND conditions can be caused by a variety of factors. These can include viruses, toxins, genetics, and environmental factors .
Who is at higher risk of developing MND?
MND most often affects people between the ages of 60 and 70. However, it can affect children and adults of any age. If a close relative of yours has MND, or a similar condition called frontotemporal dementia , you are at increased risk of developing MND.
How do doctors diagnose MND?
MND can be difficult to diagnose early on, as there is no specific test for it. If you have gradual muscle weakness without pain or loss of sensation, your doctor may suspect MND.
Questions asked by the doctor
You can ask yourself questions like:
- What parts of the body are affected ?
- When did the symptoms start?
- What were the first symptoms ?
- Have the symptoms changed over time?
Tests performed
Several tests can be done to help diagnose the disease:
- Electromyography (EMG): This records the electrical activity of your muscles. This can help determine whether the problem is in the muscles, nerves, or neuromuscular junction.
- Nerve conduction studies: This measures how quickly nerves transmit impulses.
- Magnetic resonance imaging (MRI) scan: An MRI of the brain, and sometimes an MRI of the spinal cord, is done to look for other abnormalities that may be causing the same symptoms.
To rule out other medical conditions, the doctor may request further tests:
- Blood tests
- Urine tests
- Spinal tap (lumbar puncture)
- Genetic tests
Over time, the symptoms of MND become so obvious that there are times when the disease can be diagnosed without any tests.
What are the treatments for MND?
There is no specific cure or treatment for motor neurone disease. However, researchers continue to study safe and effective ways to treat the condition.
You will need to work with a team of doctors to help you manage your symptoms. MND treatments may include:
- Physical therapy: This helps maintain your muscle strength and keep your joints flexible.
- If you have difficulty swallowing , you may be given nutrition through a tube (gastrostomy tube) inserted through the abdominal wall into the stomach.
Medicines
Some medications help many people with MND to get relief from their symptoms:
- Baclofen: Reduce muscle stiffness and reduce muscle spasms.
- Phenytoin or quinine: Reduce muscle spasms.
- Glycopyrrolate: Reduce salivation.
- Antidepressants: Help with depression.
- Benzodiazepines: For pain that occurs as the disease progresses.
For people with ALS, there are two drugs that can help prolong life and control functional decline: riluzole and edaravone .
Many people also participate in clinical trials .
When should I see a doctor?
If you have something like muscle weakness, which could be an early sign of MND, you should definitely see a doctor. You may not have MND, but getting an accurate diagnosis as soon as possible is very important to get the care and support you need.
Also, if you have a close relative with MND or frontotemporal dementia and you are concerned that you may also develop the disease, it is a good idea to see a doctor. Your doctor may also refer you to a genetic counselor to discuss your risk.
What can you expect when living with this condition?
Motor neurone disease is a progressive disease, so it's important to find a doctor who understands your condition and can help you find the right treatment to manage your condition.
It's also important to have a support system . As your condition progresses, you may need more help from others to do things that are difficult for you. Talk to friends and family, or ask your doctor about ways to get support from your community. Remember, you are not alone.
What is the life expectancy of someone with MND?
Unfortunately, motor neurone disease can significantly shorten your life expectancy . These are diseases that progress over time and eventually lead to death. However, how long it takes to reach that point varies from person to person. Some people live for years, sometimes even decades, before dying from the effects of these diseases. Your doctor can give you a better understanding of the prognosis/outlook of your condition.
The most important thing - what you have to say
Finding out you have motor neurone disease (MND) can be a scary and overwhelming experience . Remember, it’s not your fault . You haven’t done anything wrong. And it doesn’t change who you are – but it can make life a little different. While you may face challenges as the disease progresses, there are treatments that can help you manage the effects. Lean on your friends and family for support. If you’re struggling to cope or need more support, talk to your doctor. They can help you find the resources you need, and make sure you get the help you need over time.
` Motor neurone disease, MND, neurological disease, muscle weakness, ALS, nerve cells, muscle wasting
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