What is Motor Neurone Disease?

Motor neurone disease involves the degeneration of parts of the nervous system and the wasting of muscles. Also known as amyotrophic lateral sclerosis, it occurs when motor neurones in the brain and spinal cord stop working properly – neurodegeneration. It is relatively rare, affecting 2 in every 100,000 people in the UK each year.

How does someone get MND – is it genetic?

It remains unclear what causes the motor neurones to stop functioning properly. A family history of MND has been recognised in only about 5% of cases. In the majority of these cases – known as familial motor neurone disease – faulty genes have been identified.

When the person with motor neurone disease doesn’t have a family history of the condition, it is known as sporadic motor neurone disease. Experts believe the motor neurones begin to lose function as a result of a combination of inter-related factors, including:

• Aggregates (abnormal clumps of protein) developing inside the motor neurones
• Cell transport disruption – when the normal movement of nutrients into the cell becomes disrupted, possibly due to an antioxidant deficiency
• Problems with the glial cells that relay information between nerve cells
• Abnormalities in the mitochondria – the cells’ “batteries”

What are the symptoms of MND?

The early symptoms of MND can include a weakened grip that will cause problems for someone picking up and holding objects. A weakened shoulder might make lifting the arms difficult. Weak ankle muscles can cause a “foot drop” or a dragging of the leg, while slurred speech (dysarthria) can also be a symptom.

Although MND isn’t normally painful, as the damage progresses it becomes increasingly debilitating, spreading to other parts of the body and leading to the person being unable to move, communicate, swallow or breathe without difficulty. In up to 15% of cases, MND is associated with a type of dementia.

How is MND diagnosed?

There isn’t a single test to diagnose MND and the diagnosis is normally based on the opinion of a neurologist (a brain and nervous system specialist). The diagnosis of MND is usually relatively clear to an experienced neurologist but sometimes, specific tests are needed to discount other conditions that have similar features.

Can MND be treated?

Although there isn’t a cure for MND, treatments are available to give the person the best possible quality of life and to make them feel comfortable.

How can MND affect your day to day life?

A person with MND will be able to look after themselves in the early stages but as the condition progresses, they will need care either at home or in a hospital. Muscle cramps can be helped with physiotherapy or by a medication called quinine, while a speech and language therapist can teach MND techniques to make the voice as clear as possible. The treatments are all intended to give the best quality of life and for as long as possible.

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