Motor neurone disease 

Introduction 

Motor neurone disease Julie's story

Julie has motor neurone disease. Find out how she copes with this incurable condition, and how she finds the strength to continue working as a primary school teacher. Sadly, since the making of this film, Julie Genovese has died.

Media last reviewed: 20/08/2013

Next review due: 20/08/2015

Who is affected?

Motor neurone disease is a rare condition that affects around 2 in every 100,000 people each year in the UK. There are about 5,000 people living with the condition in the UK at any one time.

Most cases first develop in people in their early 60s, but people as young as 18 and as old as their 90s can also develop the disease. Motor neurone disease affects slightly more men than women.

In 5% of cases, the person has a family history of motor neurone disease or the related condition frontotemporal dementia. This is known as familial motor neurone disease, and relatives of the person may be at an increased risk of developing the disease in later life.

Motor neurone disease is the name for a rare condition where parts of the nervous system become damaged. This causes progressive weakness, usually with muscle wasting.

Motor neurone disease, also known as amyotrophic lateral sclerosis (ALS), occurs when specialist nerve cells in the brain and spinal cord called motor neurones stop working properly. Motor neurones control important muscle activity, such as:

  • gripping
  • walking
  • speaking
  • swallowing
  • breathing

As the condition progresses, people with motor neurone disease will find these activities increasingly difficult – and eventually impossible – to do.

Exactly what causes motor neurones to stop working properly is unclear, but there is not currently thought to be a link with factors such as race, diet and lifestyle.

In about 5% of cases there is a family history of either motor neurone disease or a related condition called frontotemporal dementia. In most of these cases faulty genes have been identified as contributing to the condition.

There is no single test to diagnose motor neurone disease and diagnosis is based mainly on the opinion of a neurologist. Sometimes specialised tests are needed to rule out other possible conditions.

Read more about the causes of motor neurone disease and diagnosing motor neurone disease.

Progression of symptoms

The symptoms of motor neurone disease begin gradually over weeks and months, usually only on one side of the body initially, and get progressively worse. Common early symptoms are:

  • 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

The condition is not usually painful.

As damage progresses, symptoms spread to other parts of the body and the condition becomes more debilitating.

Eventually, a person with motor neurone disease may be unable to move. They may also find communicating, swallowing and breathing very difficult.

In 10-15% of cases, motor neurone disease is associated with a type of dementia that can affect things such as personality and behaviour. This is called frontotemporal dementia.

Read more about symptoms of motor neurone disease.

Treating motor neurone disease

There's currently no cure for motor neurone disease. Treatment therefore aims to:

  • make the person feel comfortable and have the best quality of life possible
  • compensate for the progressive loss of bodily functions such as mobility, communication, swallowing and breathing

For example, a breathing mask can help reduce shortness of breath, and the insertion of a small feeding tube (called a gastrostomy) helps maintain nutrition and overall comfort. Medication is used to help control oral secretions if necessary.

A medication called riluzole has shown a very small improvement in patients' overall survival, but it is not a cure and does not stop the progression of the disease.

Read more about treating motor neurone disease.

Survival rates

Motor neurone disease is a severely life-shortening condition for most people. Life expectancy for about half of those with the condition is three to four years from the start of symptoms. However, some people may live for up to 10 years, and others even longer.

Living with motor neurone disease can be frustrating, challenging and sometimes a terrifying possibility, but it's not necessarily as bleak as many people imagine.

With strong community and specialist support, many people lead a relatively independent lifestyle and enjoy a quality of life they may not have imagined was possible at the time of their diagnosis.

In most cases, motor neurone disease is fatal because of the eventual involvement of the breathing muscles, which typically makes someone with the condition gradually more drowsy and more susceptible to chest infections.

The end of life in someone with motor neurone disease is not usually distressing and is most often in their own home. In a few cases, a person with motor neurone disease may die suddenly but painlessly during their sleep.

Page last reviewed: 15/01/2013

Next review due: 15/01/2015

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Comments

The 9 comments posted are personal views. Any information they give has not been checked and may not be accurate.

rosexelex said on 11 October 2014

Is MND related to Guilliame Barre Syndrome as that produces sudden total paralyis but is easily cured with calcium and Vitamins that rebuild the insulation on the nerves between the brain and the muscles?

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pinklollie said on 27 July 2014

Hi my Mum is 69 years old and has just been diagnosed with motor neurone disease. We are a very close family who will support each other through this but we are all very very scared at the moment. My Mum is a very young 69 who has a zest for life and a better social l life than me so this is the cruelest disease she could have. I cant bear to think of her not being able to communicate, eat or breathe.It's breaking my heart and we're only 3 days in to the diagnosis. Mum is,still in hospital I can't wait for her to get home but I'm really worried about how she'll cope. My Dad is 77 and useless bless him! I've devoured the MNDA website which is really good but I'm feeling overwhelmed, I have 3 brother's but I've a feeling I will bear the lions share of what's to come. I love my Mum so much.

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keep cool said on 25 April 2014

livelifewhileucan did you take the test to find out whether you will get MND or not. can you please tell me the name of the test. Thanks

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livelifewhileucan said on 05 February 2014

hi i m 20 years old mnd had took my dad grandad and my uncle now in 2014 there is a test that i m having to tell me if i got it :L i m really unlucky to get this test 5years when my dad died i ask about tests and there wasnt one. i m really scared because all my family are dying younger 48 ,46,44 hope this helps people

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Eileenjo said on 19 November 2013

Hi it is with much interest that I read the comments written I have had two sister die from motor neuron disease. They both followed very similar patterns. Initially both had sore throats and within days speech became slurred, over a very short period they lost the ability to speak other that grunty noises. They lost the use of the left arm and were unable to eat without choking. Both had the tube inserted into the stomach on my elder sister this worked well, unfortunately on the other this caused her a lot of pain and discomfort. The duration was four to five years. It is a cruel disease and I hope and pray that I don't suffer the same fate as my two beautiful sisters. I miss them so.

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Orangepig said on 23 October 2013

My dad died this March of bulbar onset MND. This disease swiftly denied him speech and the ability to eat. We were given a scant 6 months with him since diagnosis. To see this highly intelligent man reduced to grunting and dribbling, shuffling around the house was heartbreaking. Yes, at 84 he had had a good innings, but this disease is one of the cruellest - leaving your mind still active, but taking away everything else.

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fordy 40 said on 02 April 2013

I've just come back from the burial of my father-in-law who was diagnosed with this horrible illness a year and a half ago. He was the most active man you could possibly wish to meet, right up until his final days. MND took a hold of him big time in his final few months, it was horrible to see. He died peacefully in his sleep a couple of weeks ago, but I sure wish there was a cure for this wicked disease. God bless Chris. x

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Auto Spa said on 03 January 2013

My father died 16 years ago of MND when i was 28. I still worry now, if one day i might end up with the same disease. I'll be 45 this year and get worried if i get any sort of twitch. I remember when my dad was first diagnosed (although we the rest of the family knew something major was wrong with him, way before he went to the docs) ! He came home looking very scared. He showed me his tongue which was something i'll never forget. His tongue was jumping around like it had a mind of its own, like it was electric! (one of the symptoms) That was in the summer of1995....We went on a family holiday to New Zealand in the febuary 1996 and my dad had gone down hill so fast, that looking back it probably wasn't a good idea to go all that way, but it was somewhere he wanted to visit. Then by the september of 96 aged only 53, dad was gone. He must have been hiding the disease for at least 2 years, because he got so ill within 1 year before he died... STILL MISS YOU DAD X

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martinharlow said on 28 January 2012

I found this quite helpful apart from the section relating to the causes of motor neuron which seemed to me rather technical and did not given any real information as to why/how such conditions arose e.g. lifestyle, diet etc.

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