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Motor neurone disease explained

Professor Stephen Hawking overcame a debilitating rare disease
Professor Stephen Hawking overcame a debilitating rare disease

Professor Stephen Hawking overcame a debilitating rare disease to become one of the world's best known physicists.

He survived for around half a century after being diagnosed with motor neurone disease (MND), despite being told he had just years to live when he was diagnosed.

The disease kills a third of people within a year and more than half within two years of diagnosis, according to the Motor Neurone Disease Association.

In Ireland, approximately 140 people are diagnosed with MND each year and there are about 360 people living with the disease.

Yet Prof Hawking was diagnosed with the condition in his early 20s and lived until he was 76.

The condition is fatal, and usually progresses rapidly, affecting the brain and spinal cord.

What is motor neurone disease?

MND is the collective name for a group of diseases that affect the nerves in the brain and spinal cord that control movement.

The condition progressively damages parts of the nervous system which leads to muscle weakness, stiffening and waste.

It can lead to people being unable to move, talk, swallow and eventually breathe.

Some patients suffer cognitive changes as well.

Four in five sufferers will have communication difficulties.

But the MND Association states that the condition affects everyone differently - not all symptoms will affect everyone and symptoms progress at varying speeds.

Cause?

The cause of MND is not known. There may be environmental factors that trigger the damage in people who are susceptible to the disease.

On going research is necessary to find out the nature of these environmental factors, and what makes one person more susceptible than another.

According to The Irish Motor Neurone Disease Association (IMNDA), "In the vast majority of cases of MND, there is no history of the disease in any of the immediate family members (grandparents, parents, brothers or sisters). However, in about 20% of cases, family history of the disease indicates that inherited genetic damage causes the condition; this is termed "familial MND".

What are the symptoms of motor neurone disease?

Signs of the disease will begin gradually.

Typically, this will occur on one side of the body only, before progressively worsening where swallowing and breathing may also become a difficult task.

In the UK, the NHS outlines initial symptoms to look out for…

  • a weakened grip, which can cause difficulty picking up or holding objects
  • weakness at the shoulder that makes lifting the arm difficult
  • a "foot drop" caused by weak ankle muscles
  • dragging of the leg
  • slurred speech (dysarthria)

Is there a cure?

There is no cure for MND and there is not a clear answer about what causes the condition.

"MND is a rare disease that generally affects adults. Most people with MND are aged 50 years or older but occasionally people in their 20s, 30s and 40’s develop MND," said the IMNDA

In the UK and Ireland, MND is used as an umbrella term for all forms of the disease. In the USA, Amyotrophic Lateral Sclerosis (ALS) is used as the umbrella term. 

More information on MND can be found on www.imnda.ie

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