What Is Amyotrophic Lateral Sclerosis, The Root Of Stephen Hawking’s Death?

What Is Amyotrophic Lateral Sclerosis, The Root Of Stephen Hawking’s Death? - Stephen Hawking was probably the most famous man to have ALS, and I sort of understand what it is. But what does ALS stand for?

ALS stands for Amyotrophic Lateral Sclerosis. It is also called Lou Gehrig’s disease, after the famous American baseball player who had it.

It is a type of motor neuron disease that is progressive, and slowly destroys nerve cells and causes disability.

What is motor neuron disease? Actually, what is a motor neuron in the first place?

A motor neuron is a nerve cell that has a cell body located in either the spinal cord, brainstem or motor cortex of the brain.

The word “motor” here denotes action. Motor neurons control the organs and muscles that act or do things that involve “action”, e.g. walking, talking, swinging your arms, producing saliva etc.

This is opposed to sensory neurons that sense touch, heat, cold, pressure and vibrations coming from your skin.

A motor neuron constitutes a cell body that projects into an axon (nerve fibre).

Motor neuron disease constitutes a group of conditions that affect these motor neurons.

There are five main types of motor neuron disease, and ALS is one of them.

What are the other types of motor neuron disease?

ALS is the most common type, and it affects the muscles in your arms, legs, mouth and respiratory system.

It usually causes death in three to five years because it ultimately shuts down the muscles of your breathing system, but some people – like Hawking – live for years and years.

Another type is Progressive Bulbar Palsy (PBP). This one involves the brainstem, leading to choking, difficulty in speaking, eating and swallowing.

Many people with ALS have PBP too.

Yet another type is Progressive Muscular Atrophy (PMA). This one is a variation of ALS that slowly causes your arm, leg and mouth muscles to waste.

The rarest of them all is Primary Lateral Sclerosis (PLS), which advances very slowly. Luckily, this one does not kill you.

Lastly, there is Spinal Muscular Atrophy (SMA), that affects the trunk, arms and legs of children.

What causes ALS? Is it inherited?

Only 5% to 10% of cases are inherited.

But, to this day, doctors still don’t know what causes most cases of ALS.

Researchers have postulated that it may be caused by gene mutations, or a chemical imbalance, or even a “disorganised” body immune response.

However, there seems to be some risk factors for ALS. They are:

• Hereditary: If you have ALS, there’s a 50/50 chance your children may have ALS.

• Age: ALS is commonest between the ages of 40 and 60.

• More men than women get it.

• Smoking, especially for women.

• Exposure to environmental toxins.

• Military service. It’s unclear exactly how this can trigger ALS, but there seems to be a link.

How do the symptoms of ALS start?