Motor neurone disease

Published by Bupa’s Health Information Team, January 2011.

This factsheet is for people who have motor neurone disease, or who would like information about it.

Motor neurone disease (MND) is the name given to a group of rare diseases that affect nerve cells in the brain and spinal cord leading to muscle weakness and wasting.

About motor neurone disease

Your brain sends instructions to muscles that control voluntary movement (such as walking and swallowing) along nerve cells called motor neurones. MND is a disease that gradually destroys these nerve cells leading to weakness and wasting of your muscles.

Around three in every 100,000 people in the UK develop MND each year. It tends to occur as you get older – usually between the age 50 and 70. Men may be up to twice as likely as women to be affected.

Types of motor neurone disease

There are three main types of MND, which affect different groups of nerves.

• Amyotrophic lateral sclerosis is the most common type of MND. This mainly causes muscle weakness and stiffness.
• Progressive bulbar palsy affects about one in four people with MND. This makes speaking and swallowing difficult.
• Progressive muscular atrophy is a less common type of MND. It causes muscle weakness, wasting and twitching.

Symptoms of motor neurone disease

MND develops at different speeds in different people and affects individuals in different ways.

The disease usually begins very gradually and you may just feel tired to start with. Clumsy fingers and a weak grip are often the first symptoms of muscle problems.

Other symptoms include:

• pain and discomfort
• muscle cramps, spasms or twitching – called fasciculations
• stiff joints
• constipation – due to restricted movement or change in diet
• excess saliva or difficulty swallowing saliva
• coughing or a feeling of choking
• breathing problems
• depression

These symptoms aren’t always caused by MND but if you have them, see your doctor.

Although thinking and reasoning aren’t usually affected, it’s possible you may have some level of intellectual difficulty or a change in personality.

As the disease progresses you may have the following problems.

• Muscle weakness in your arms, hands and legs may make everyday tasks more difficult, such as turning taps or door handles, brushing your hair or fastening buttons. Raising your foot with each step may be difficult, which may cause your feet to drag on the floor. You may find it difficult to hold your head upright if your neck muscles weaken.
• Your throat muscles may be affected and this may lead to difficulties swallowing and with speech.
• You may laugh or cry more easily. This may be due to physical changes caused by the disease, rather than changes in how you’re feeling.
• The muscles of your chest wall may be affected, which can make it difficult to breathe.

Causes of motor neurone disease

Research is underway to understand the causes of MND.

A small number of people with MND have a family history of the condition, indicating a genetic link. However for most people with MND, the disease appears for no apparent reason. This is called sporadic MND.

Diagnosis of motor neurone disease

Your doctor will ask about your symptoms and examine you. He or she may also ask you about your medical history. If your doctor suspects you may have MND he or she will refer you to a neurologist (a doctor who specialises in conditions of the nervous system).

There is no single diagnostic test for MND. The diagnosis is usually made based on symptoms and what a neurologist finds when examining you. Your neurologist will also try to rule out other neurological conditions.

Tests to help diagnose MND include the following.

• An electromyography – this test uses fine needles to measure the electrical activity of your muscles.
• Nerve conduction tests – an electrical impulse is applied through a small pad on your skin; this measures the speed at which your nerves carry electrical signals.
• Transcranial magnetic stimulation – this measures the activity of the nerves that run from your brain to your spinal cord.
• Magnetic resonance imaging (MRI) scan – this uses magnets and radiowaves to produce images of the inside of your body.

Treatment of motor neurone disease

Medicines

Currently there is no cure for MND, however there are medicines available to slow down the progress of the disease and manage your symptoms.

• Riluzole has been shown to slow down the progress of the disease by a few months. The National Institute for Health and Clinical Excellence (NICE) has recommended riluzole for the treatment of one of the types of MND - amyotrophic lateral sclerosis.
• Muscle relaxants can help reduce the flow of saliva and treat muscle cramps.
• Anti-inflammatory medicines and painkillers can help reduce pain and discomfort.

Discuss your symptoms with your GP so that they can be effectively managed.

You may also be offered a mask ventilator system to wear at night while you’re asleep. This should help you to breathe easier and may slow the progress of MND. The machines are small and easy to carry.

Newer treatments

Researchers are continually improving their understanding of MND and investigating new treatments. There are treatments currently being investigated in clinical trials, but doctors won’t know if they help MND until the trials are finished.

Recent scientific discoveries that may lead to future treatments include:

• faulty genes that may be involved in the development of MND
• stem cell research – stem cells are the body’s ‘master cells’ and have the ability to divide and develop into any type of cell, in effect they could be used to create new motor neurones

Complementary therapies

Complementary therapies can help reduce anxiety and stress and may help to make day-to-day life more comfortable. These can include:

• acupuncture
• meditation
• massage
• reflexology

Talk to your GP before starting any complementary therapy to make sure that it isn’t harmful and is compatible with any other medicines you’re taking. Make sure that the practitioner is fully qualified and registered. Your GP may be able to advise you.

Help and support

A team of professionals will help support you, and your family. This team may include your neurologist, physiotherapists, occupational therapists, speech therapists and palliative care specialists.

The support of your family and friends is often invaluable and can help with anxiety and stress.

Patient associations, such as the Motor Neurone Disease Association, provide information and services that can make life easier and more comfortable. It may help to contact other people who have MND through charities and patient groups as they can be a good source of support and advice.

For answers to frequently asked questions on this topic, see FAQs.

For sources and links to further information, see Resources.