Motor Neuron Disease

Motor Neurone Disease

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If you’re not familiar with the term "Motor Neurone Disease", perhaps you've heard of one of the following:

  • Also known as Amyotrophic Lateral Sclerosis, in the USA
    • Do you remember last summer’s Facebook craze, the ALS Ice Bucket Challenge, for ALS (motor neurone disease) awareness?

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The ALS Ice Bucket Challenge, was a popular social media challenge, aimed to promote awareness for ALS, and encourage donations for research.

 

  • Also known as Lou Gehrig’s Disease
    • Lou Gehrig was a very successful American baseball player who played for the New York Yankees in the 1920’s and 30’s. He retired from the sport aged 36, after suffering with Motor Neurone Disease, dying just two years later.

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Lou Gehrig, pictured playing baseball for the New York Yankees in 1923. 


What Motor Neurone Disease?

  • MND is characterised by the progressive degeneration of nerve cells and pathways, in the brain and spinal cord.
  • The death of these essential nerve cells leads to the loss of voluntary muscle control, which causes:
    • Motor difficulties, including walking
    • Speech difficulties
    • Eventually, paralysis
  • Around 10% of MND patients may experience some form of dementia; however most MND sufferers’ minds are usually unaffected

 

How common is MND?

  • Around 5,000 people in the UK have motor neurone disease
  • The condition can affect adults of any age
  • However, MND sufferers usually first develop symptoms in their 60’s.
  • Slightly more men than women are affected.

 

What causes MND?

  • There is no clear cause – although a lot of research is being done to investigate the cause, and an effective treatment.
  • 5% of cases are thought to be genetic – known as familial MND
  • Recent research from the University of Glasgow found that those who had served in the UK armed forces are 50% more likely to develop MND.

 What’s the prognosis?

  • Sadly, there’s no cure to MND
  • Treatments aims to:
    • Relieve the discomfort,
    • Maintain a good quality of life,
    • Compensate for the bodily disabilities caused, especially with regard to:
      • Motility
      • Communication
      • Swallowing
  • 50% of patients die within two years of diagnosis
  • Estimated survival range from 2-5 years post-diagnosis, depending on type of MND

  • Disappointingly, a recent survey revealed that 20% of patients diagnosed with MND wait over a year to see a neurologist.
  • Around 40% of MND patients claimed that their GP only offered referring them to a neurologist after their 4th GP consultation.

 

 

  • A notable British MND sufferer is Cambridge University theoretical physicist Stephen Hawking:
    • Hawking suffers from a rare type of MND:
      • Early onset – he was diagnosed aged just 21, and doctors gave him just two years to live.
      • Slow progressing – 20 years since his initial diagnosis, Hawking is still living with his disease, aged 74!
    • Since losing the use of his hands, Hawking now communicates via a device detecting movements in his cheek muscles – at the rate of a single word per minute.
    • Despite his diagnosis of motor neurone disease, Stephen Hawking has had an outstanding career in theoretical physics, and still maintains his position as Director of Research in his department at the University of Cambridge to this day!

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Stephen Hawking: pictured at NASA in the 1980's.

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