Putting Brains in Muscles

Towards an Accessible Web-Based Health Education Platform on Neuromuscular Diseases

Motor Neurone Disease

What is Motor Neurone Disease?

Motor Neurone Disease (MND), also known as amyotrophic lateral sclerosis (ALS), is a disease that damages the nerve cells that control the muscles. The affected muscles become wasted and weak. The patients are getting worse gradually until they fail to move, swallow, speak and breath; and they finally die.

The cause of MND is unknown, but some patients are affected by genetic factors. Most people with MND can only live for a few years after the onset of symptoms. If difficulty in swallowing or breathing is an early symptom, they may even die within several months.

What are the symptoms of Motor Neurone Disease?

The common symptoms that are getting worse by themselves include:

  • Muscle weakness and wasting of the arms or legs, usually starting from one arm or one leg and then spreading to other parts of the body (Figure 1)
    (Figure 1) Wasting of the hand muscles.
    (Figure 1) Wasting of the hand muscles.
  • Speaking or swallowing difficulties
  • Slow, stiff or clumsy movements
  • Muscle twitching (fasciculation) (Video 1, 2)

(Video 1) Muscle wasting and fasciculation of the tongue

(Video 2) Muscle wasting and fasciculation of the upper limb

If a person’s hand or arm muscles are weak, he or she may encounter problems in the followings:

  • Using buttons or zippers
  • Handling coins or other small things
  • Writing

If a person’s leg muscles are weak, he or she may encounter problems in the followings:

  • Walking and turning
  • Balance
  • Climbing stairs
  • Standing up from a chair or the floor

If a person’s head, neck or chest muscles are weak, he or she may have the following problems:

  • Jaw being stiff and mouth difficult to be opened
  • Trouble in swallowing
  • Speech problem
  • Drooling
  • Eyes not able to close fully
  • Head drop
  • Difficulty in breathing

Some MND patients have mood changes. They may laugh, cry or yawn without reasons. Some others may have problems in normal thinking.

How are Motor Neurone Disease diagnosed?

Your doctor might be able to tell if you have MND by learning more about your symptoms and performing physical examination. Your doctor might arrange some tests to help the diagnostic process. These might include:

  • Electromyography (EMG) – This test is done by using a small amount of current to stimulate your nerves in your hands and feet, and putting a sterile recording needle into your muscles to check if they function normally.
  • Magnetic Resonance Imaging (MRI) – This is an imaging test that takes pictures of your brain and spinal cord.
  • Blood or urine tests

What are the recommended treatments for Motor Neurone Disease?

MND does not have a cure or an effective treatment for nerve cell regeneration. Medical research has found that two drugs may slightly prolong the life expectancy of patients with MND. The first one, riluzole (brand name: Rilutek ®), is an oral medicine that helps some patients live a few months longer by slowing down the progression of the disease a bit. The other drug, edaravone (brand name: Radicava®), is given by intravenous infusion and might also help slow the worsening in some early diseased patients. However, none of these treatments can effectively improve the quality of life of the affected patients.

 

Although MND cannot be cured, some therapies might be helpful to relieve the distressing symptoms of the patients. These therapies include:

  • Non-invasive breathing support – For this treatment, you wear a tight-fitting mask on your face or nose. Air and oxygen flow through the mask from a small ventilator to help you breathe.
  • Feeding tube – This is a flexible tube that goes through the skin over your belly or through your nose into your stomach. You can then get special liquid food and fluids through the tube directly into your stomach to improve your nutrition and body weight.
  • Devices to help with walking and talking, such as:
    I. A cane, crutches, walking frames or powered wheelchair.
    II.Picture cards or instruments that help communication with people.
  • Drugs to treat muscle cramps, drooling, sleep problems, pain and depression.