Motor Neurone Disease

Motor neurone disease is thankfully reasonably rare, with it affecting only around two in every 100,000 people in the UK. Nevertheless, with our population, this is still quite a significant number.

The disease is a progressive neurodegenerative disease. In normal terms, this means that the disease progressively damages the nervous system and stops our specialist nerve cells, or our motor neurones, from working properly. These motor neurones send instructions from the brain to the muscles and bones to produce movement and they also help regulate many of our automatic processes. Therefore interference with the motor neurones will affect many of our vital activities such as walking, speaking, breathing and swallowing.

There is a lot of research still to be done on the causes of motor neurone disease although the latest thoughts are that a combination of specific genetic, environmental and lifestyle factors building up over the years can help cause the disease. In around 10 per cent of cases, the person will have a close family relative who has already developed the condition and here there is obviously a genetic link.

Generally, motor neurone disease usually develops when a person is middle ages, perhaps in their late 40s or early 50s, and it is slightly more common in men than women.

There are three main types of the disease which often begin with similar symptoms but then progress in different ways. These are:

• limb-onset disease which begins with muscle weakness and loss of function in the arms and legs. You can find your arms and legs might begin to feel weak, your grip weakens, muscles can twitch or cramp up, and you can feel tired and even clumsy as the disease progresses. This is the most common type.
• bulbar-onset disease which begins with problems such as slurred speech and difficulties in swallowing. Your tongue may begin to twitch a little and even become smaller. Difficulties in swallowing may also occur at quite an early stage.
• respiratory-onset disease which shows by a shortness of breath. This is far less common than the other two types of motor neurone disease but the symptoms can include waking up frequently during the night because of lack of oxygen.

Diagnosing motor neurone disease can be difficult because the early symptoms could also indicate other diseases and health problems. To rule this out, there are various tests that can be undertaken to confirm a diagnoses. Blood tests assess towards general chemical composition; an MRI scan will check aspects of your brain and nervous system; EMG will measure the electrical activity in the muscles; and there is also a nerve conduction test which measures how your nerves conduct electrical signals and a transcranial magnetic stimulation test where a magnetic coil measures the electrical activity in the brain.

Often, the diagnoses won’t be confirmed until after quite a few months.
As motor neurone disease progresses to an advanced stage, the differences between the various types of disease are less noticeable as more parts and functions of the body are affected.

Eventually, because of the development of the disease, people usually become drowsy and fall into a deep sleep. Most usually die peacefully in their sleep.
The outlook for those with motor neurone disease is at the moment very poor with survival rates being only a handful of years after the symptoms start. However, this isn’t always the case and some do live for several decades – the famous British physicist Stephen Hawking has had the disease for many decades.

For anyone who wants to know more about this disease, including carers involved in looking after someone with the disease, there is a motor neurone disease association; visit www.mndassociation.org

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