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Motor neurone disease
This factsheet is for people who have motor neurone disease, or who would like information about it.
Motor neurone disease 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
Symptoms of motor neurone disease
Causes of motor neurone disease
Diagnosis of motor neurone disease
Treatment of motor neurone disease
Help and support
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. Motor neurone disease (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 get 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 (ALS) is the most common type of MND. This mainly causes muscle weakness and stiffness.
- Progressive bulbar palsy (PBP) affects about one in four people with MND. This makes speaking and swallowing difficult.
- Progressive muscular atrophy (PMA) 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, indicating an inherited 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 (EMG) – 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 (TMS) – this measures the activity of the nerves that run from your brain to your spinal cord.
- 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 and your life expectancy will depend on how your symptoms progress. 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 the ALS form of the disease.
- 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.
This section contains answers to common questions about this topic. Questions have been suggested by health professionals, website feedback and requests via email.
How can I manage the problem of too much saliva?
Does motor neurone disease run in families?
Are there any new medicines being developed for motor neurone disease?
How can I manage the problem of too much saliva?
Your GP can prescribe medicines to reduce the amount of saliva you produce. Improving your posture and changing your diet can help to reduce saliva. You may find that a suction pump also helps.
Explanation
Saliva makes swallowing easier and keeps your mouth healthy. If you have difficulty swallowing, watery saliva can build up in your mouth and result in dribbling. Your GP can prescribe medicines to reduce the amount of saliva you produce. Improving your posture may also help stop saliva pooling in your throat. A reclining chair or chin support can help support your head.
You may get thick saliva if you’re dehydrated or have difficulty breathing through your mouth. Your GP may prescribe medicines such as carbocisteine to reduce the thickness of your saliva. Always ask your doctor for advice and read the patient information leaflet that comes with your medicine.
If you have problems with thick saliva, make sure that you drink enough fluid. Drinking citrus fruit juices such as grapefruit and orange as well as apple and pineapple juices can help to break down the protein in the mucus. These can be sipped or frozen into icy sticks.
Your GP or district nurse may provide you with a suction pump. This has a small tube attached that you place in your mouth to suck out excess saliva. It can be operated by you or a carer.
Further information
- Motor Neurone Disease Association
08457 626 262
www.mndassociation.org
Sources
- NINDS Motor Neuron Diseases Information Page. National Institute of Neurological Disorders and Stroke. www.ninds.nih.gov, published October 2010
- Carbocisteine. Joint Formulary Committee. British National Formulary. 60th ed. www.bnf.org, accessed 7 October 2010
- Saliva control. Motor neurone disease association. www.mndassociation.org, published November 2010
Does motor neurone disease run in families?
Yes, motor neurone disease (MND) can run in families, although it is rare. Around five in 100 with people MND will have an affected family member. This is called familial motor neurone disease (FMND).
Explanation
If you have MND and also have a parent, aunt, uncle or sibling with the disease, it’s called FMND.
In FMND, it’s thought that a faulty gene causes the disease. The faulty gene can be passed from parent to child. Children of a parent who has FMND have a one in two chance of inheriting the faulty gene, so the disease isn’t passed on to all family members.
In the majority of families with FMND, the faulty gene is unknown. However, around two in 10 people with FMND have a faulty gene called SOD1. It isn’t yet known how this causes the disease but motor neurones have higher levels of SOD1 than other nerve cells, which may be why they are vulnerable if this gene is faulty.
If you have FMND, a blood test is available to see if you have the faulty SOD1 gene. If you do, other family members may request a blood test (genetic testing) to see if they carry the faulty gene too. Having the faulty SOD1 gene doesn’t automatically mean that you will get MND.
Further information
- Motor Neurone Disease Association
08457 626 262
www.mndassociation.org
Sources
- Motor neurone disease; the use of non-invasive ventilation in the management of motor neurone disease, CG 105. National Institute for Health and Clinical Excellence (NICE). 2010. www.nice.org.uk
- Benatar M, Kurent J, Moore DH. Treatment for familial amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews 2009, Issue 1. doi: 10.1002/14651858.CD006153.pub2
- Is MND inherited? Motor neurone disease association. www.mndassociation.org, published February 2009
- Vance C, Rogelj B, Hortobágy T, et al. Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science 2009; 323(5918):1208–11
Are there any new medicines being developed for motor neurone disease?
Yes. There are a number of treatments for motor neurone disease (MND) currently being investigated in clinical trials.
Explanation
Clinical trials help find out if new treatments are safe and effective. Clinical trials are usually done in three stages – phase I, phase II and phase III.
- Phase I are the earliest trials in the life of a new treatment. The medicine is tested in up to 30 (but usually far fewer) patients with the advanced stage of disease to find out the safe dose range, side-effects and how the body copes with the treatment.
- Phase II clinical trials test the medicine in up to 100 patients with the disease to find out more about the safe dose range, side-effects and how the body copes with the treatment.
- Phase III clinical trials compare the new treatment with the best currently available treatment and check the new treatment is safe and effective. Sometimes phase 3 trials involve thousands of patients in many different hospitals and even different countries.
Treatments for MND currently being investigated in clinical trials include the following.
- Ceftriaxone (an antibiotic) is currently being tested in a phase III trial in America to find out if it improves survival in people with amyotrophic lateral sclerosis (ALS) form of MND.
- Tauroursodeoxycholic acid is an anti-oxidant that acts to ‘mop up’ waste molecules before they damage nerve cells. This medicine is currently being investigated in an Italian clinical trial.
- Pioglitazone is a medicine used for the treatment of diabetes. A phase II trial in Europe is currently looking to see if it benefits people with MND.
Further information
- Motor Neurone Disease Association
08457 626 262
www.mndassociation.org
Sources
- Treatment trials. Motor neurone disease association. www.mndassociation.org, published November 2010
- Andrews J. Amyotrophic lateral sclerosis: clinical management and research update. Current Neurology and Neuroscience Reports 2009; 9:59–68
Related topics
- Acupuncture
- MRI scan
Further information
- Motor Neurone Disease Association
08457 626 262
www.mndassociation.org
- Scottish Motor Neurone Disease Association
0141 945 1077
www.scotmnd.org.uk
- Brain and Spine Foundation
0808 808 1000
www.brainandspine.org.uk
Sources
- Motor neurone disease; the use of non-invasive ventilation in the management of motor neurone disease, CG 105. National Institute for Health and Clinical Excellence (NICE), 2010. www.nice.org.uk
- What is MND? Motor neurone disease association. www.mndassociation.org, published December 2009
- Motor neurone disease (MND) statistics. Motor neurone disease association. www.mndassociation.org, published September 2010
- Different types of MND. Motor neurone disease association. www.mndassociation.org, published May 2010
- Benatar M, Kurent J, Moore DH. Treatment for familial amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews 2009, Issue 1. doi: 10.1002/14651858.CD006153.pub2
- CG105 Motor neurone disease - non-invasive ventilation: understanding NICE guidance. National Institute for Health and Clinical Excellence (NICE). 2010. www.nice.org.uk
- Motor neurone disease. Brain & Spine Foundation. www.brainandspine.org.uk, published August 2010
- Symptoms. Motor neurone disease association. www.mndassociation.org, published February 2010
- Diagnostic process. Motor neurone disease association. www.mndassociation.org, published March 2010
- Riluzole. Joint Formulary Committee. British National Formulary. 60th ed. www.bnf.org, accessed 7 October 2010
- Pastula DM, Moore DH, Bedlack RS. Creatine for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews 2010, Issue 6. doi: 10.1002/14651858.CD005225.pub2
- NINDS Motor Neuron Diseases Information Page. National Institute of Neurological Disorders and Stroke. www.ninds.nih.gov, published October 2010
- Guidance on the use of riluzole (Rilutek) for the treatment of motor neurone disease. Technical Appraisal Guidance TA20. National Institute for Health and Clinical Excellence (NICE), 2001. www.nice.org.uk
- Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review). Neurology 2009; 73:1218–26
- Vance C, Rogelj B, Hortobágy T, et al. Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science 2009; 323(5918):1208–11
- Ng L, Khan F, Mathers S. Multidisciplinary care for adults with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database of Systematic Reviews 2009, Issue 4. doi: 10.1002/14651858.CD007425.pub2
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