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 (MND), 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 MND 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 MND 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 MND and diagnosis is based mainly on the opinion of a neurologist. Sometimes specialised tests are needed to rule out other possible conditions.
Progression of symptoms
The symptoms of MND 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 MND may be unable to move. They may also find communicating, swallowing and breathing very difficult. In 10-15% of cases, MND is associated with a type of dementia that can affect things such as personality and behaviour. This is called Frontotemporal Dementia.
Treating Motor Neurone Disease
There’s currently no cure!
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.
Survival rates
MND 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 MND 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, MND 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 MND is not usually distressing and is most often in their own home. In a few cases, a person with MND may die suddenly but painlessly during their sleep.