There are three main types of motor neurone disease, which affect different groups of nerves.
- Amyotrophic lateral sclerosis is the most common type. It often affects the muscles in your arms, legs or face first.
- Progressive bulbar palsy affects about one in five people with motor neurone disease. It mainly affects the muscles you use to talk, chew and swallow.
- Progressive muscular atrophy is a less common type. It often affects the muscles in your hands and feet first before it spreads to the rest of your body.
Motor neurone disease develops at different speeds and ways in everyone who has it. It usually begins very gradually – at first feeling tired may be the only symptom you have.
The exact symptoms you get will depend on what type of motor neurone disease you have. Often, the first symptoms of problems with your muscles are clumsy fingers and a weak grip. Other symptoms may include:
- your muscles wasting away
- muscle cramps, spasms or twitching – these are called fasciculations
- difficulty swallowing
- slurred speech
- producing too much saliva or having difficulty swallowing it
- coughing after swallowing, or feeling like you're choking
If you have any of these symptoms, see your GP.
You may have symptoms in only certain parts of your body to begin with but they will eventually spread to other areas. As the disease progresses, you may develop the following problems.
- Weak muscles in your arms, hands and legs may make everyday tasks more difficult, such as turning taps or door handles 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, which can make it difficult to swallow. You may have difficulties with speech too.
- You may laugh or cry more easily. This may be because of 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.
Motor neurone disease may affect your memory, and your personality may change later on as it progresses. However, this doesn't happen to everybody.
Your GP will ask about your symptoms and examine you. If your GP suspects you may have motor neurone disease, he or she will refer you to see a neurologist. This is a doctor who specialises in conditions that affect the nervous system, including the brain and spinal cord.
There isn't a single diagnostic test for motor neurone disease. Your doctor will usually make a diagnosis based on your symptoms and what he or she finds when examining you. Your doctor will also try to rule out other conditions that can affect your nervous system.
You may need to have a number of tests, which may include the following.
- Blood tests. These will assess how well your organs are working and will rule out other conditions that might be causing your symptoms.
- An electromyogram (EMG). This test uses fine needles to measure the electrical activity of your muscles.
- Nerve conduction test. This measures the speed at which your nerves carry electrical signals by applying an electrical impulse through a small pad on your skin.
- Transcranial magnetic stimulation (TMS). This test measures the activity of nerves that run from your brain to your spinal cord.
- MRI or CT scan. These will help to rule out other conditions that may be causing your symptoms.
There isn’t a cure for motor neurone disease but there are medicines that can slow down its progress and manage your symptoms. We’ve described some of them here.
- Riluzole has been shown to slow down the progress of motor neurone disease by around a few months. The National Institute for Health and Care Excellence (NICE) has recommended riluzole as a treatment for amyotrophic lateral sclerosis.
- Muscle relaxants can help reduce muscle stiffness.
- Medicines (such as baclofen) can treat muscle cramps.
- Anti-inflammatory medicines and painkillers can help relieve pain and discomfort.
It’s important to talk to your doctor about your symptoms so he or she can help you to manage them.
If the muscles that you use for breathing are affected by motor neurone disease, your doctor may offer you a mask that attaches to a ventilator system. You wear this at night while you’re asleep. It should help you to breathe more easily and may slow the progress of motor neurone disease. The machines are small and easy to carry.
Researchers are continually striving to improve their understanding of motor neurone disease and investigating new treatments. If you’re interested in taking part in a clinical trial, ask your doctor for more information.
Scientific discoveries that may lead to future treatments include the following.
- Certain genes may be involved in the development of motor neurone disease. This means that one day it may be possible to stop these genes causing motor neurone disease.
- Stem cell research. Stem cells have the ability to divide and develop into any type of cell. In effect they could be used to create new motor neurones (the nerve cells that control movement).
Complementary therapies won’t stop the progression of motor neurone disease but you may find they help with some of your symptoms, such as stress.
Complementary therapies include:
Talk to your doctor before you start any complementary therapy. Also make sure any practitioner you see is fully qualified and registered with or affiliated to a recognised body.
The exact reasons why you may develop motor neurone disease aren't fully understood at present. Research is underway to try to find out what causes it.
A small number of people with motor neurone disease have a family history of the condition so you may inherit it. See our FAQs for more information. Environmental factors, such as smoking or working with chemicals, may also affect your chances of developing motor neurone disease. However, for most people with the disease, it appears for no apparent reason. This is called sporadic motor neurone disease.
A multidisciplinary team of health professionals will help support you and your family. Here we’ve explained the roles of some of the people who can help you manage your symptoms.
- A speech and language therapist can help you with any speech and swallowing problems.
- A physiotherapist is a health professional who can help you to stay able to move around. They can help you with any physical symptoms, such as pain from stiff joints and muscle spasms.
- An occupational therapist can give practical assistance to help you manage with everyday tasks and increase your independence. They can advise you about using equipment that has been adapted to help you with tasks around the home.
- A dietitian can give you advice on a healthy diet and maintaining a healthy weight.
The support of family and friends can be invaluable if you have motor neurone disease. There are also a number of patient associations, which provide information and services. For details, see Resources. It may help to contact other people who have motor neurone disease through charities and patient groups for support and advice.
I have motor neurone disease. How can I manage the problem of too much saliva?
Your doctor can prescribe medicines to reduce the amount of saliva you produce. If you improve your posture and change your diet, it may help too. You may also find a suction pump helpful.
Saliva helps you to swallow and keeps your mouth healthy. If you have difficulty swallowing, it can build up in your mouth and you may dribble. Your doctor can prescribe medicines to reduce the amount of saliva you produce. If these don't work for you, your doctor may give you an injection of botulinum toxin into your salivary glands.
Keeping your posture as upright as possible may also help stop saliva pooling in your throat. Sitting in a reclining chair or using a chin support can help support your head.
You may get thick saliva if you’re dehydrated or have difficulty breathing through your mouth. If this becomes a problem for you, make sure that you’re drinking enough fluids. Fruit juices, in particular pineapple juice, can help to break down the protein in the mucus. You can sip these or freeze them into lollies.
You may be provided with a suction pump. This has a small tube that you place in your mouth to suck out excess saliva. You can operate it yourself, or a carer can do it for you.
Does motor neurone disease run in families?
Yes, motor neurone disease can run in families, although this isn’t very common. Around one in 20 people with motor neurone disease will have an affected family member. This is called familial motor neurone disease (FMND).
If you have motor neurone disease and also have a parent, aunt, uncle or sibling with the disease, you may have familial motor neurone disease.
It’s thought that a faulty gene causes familial motor neurone disease and this can be passed from a parent to their child. If you have a parent with familial motor neurone disease, you have a one in two chance of inheriting the faulty gene.
In the majority of families with familial motor neurone disease, it isn't known which gene is faulty. However, around two in 10 people with familial motor neurone disease (the amyotrophic lateral sclerosis type) have a faulty gene called SOD1. But doctors don't yet know how this causes the disease. A second gene called C9orf72 has also been identified, which may increase your risk of developing motor neurone disease. Around half of people diagnosed with familial motor neurone disease in Europe have been found to have this gene.
If you have familial motor neurone disease, you can have a blood test to see if you have these faulty genes. If you do, other members of your family may want to think about getting tested too. This is called genetic testing. Having the faulty SOD1 or C9orf72 gene (or both) doesn’t automatically mean that you will get motor neurone disease. Some people can have a faulty gene but never develop motor neurone disease. The reasons why this is aren’t fully understood yet but other genetic or non-genetic factors may in some way protect them.
If you’re concerned about motor neurone disease and your family, or would like information about genetic testing, contact your doctor.
Are there any new medicines being developed for motor neurone disease?
Yes. There are a number of treatments for motor neurone disease currently being investigated in clinical trials. However, it's important to remember that not all medicines work and so won't make it through the trial process.
Clinical trials are designed to find out if new treatments are safe and effective. Clinical trials are usually done in three stages.
- Phase I are the earliest trials in the life of a new treatment. The medicine is tested in small numbers of healthy volunteers or patients to find out what a safe dose is. The trials also check if there are side-effects and how the body copes with the treatment.
- Phase II clinical trials test the medicine in a larger number of patients with the disease. They aim to find out more about the safety of the medicine as well as if it has a positive effect on the disease.
- Phase III clinical trials compare the new treatment with the best currently available treatment to see if it works (and also tests safety). Sometimes phase III trials involve thousands of patients in many different hospitals and even different countries.
If you would like to know more about taking part in a clinical trial, ask your doctor.
- Motor neuron diseases fact sheet. National Institute of Neurological Disorders and Stroke. www.ninds.nih.gov, published 10 October 2014
- Pathology of motor neuron disorders. Medscape. www.emedicine.medscape.com, published 29 April 2014
- Motor neurone disease. PatientPlus. www.patient.co.uk/patientplus.asp, published 16 May 2012
- Motor neurone disease: the use of non-invasive ventilation in the management of motor neurone disease. National Institute for Health and Care Excellence (NICE), July 2010. www.nice.org.uk
- Amyotrophic lateral sclerosis. Medscape. www.emedicine.medscape.com, published 2 May 2014
- Amyotrophic lateral sclerosis and other motor neuron diseases. The Merck Manuals. www.merckmanuals.com, published March 2014
- Guidance on the use of riluzole (Rilutek) for the treatment of motor neurone disease. National Institute for Health and Care Excellence (NICE), January 2001. www.nice.org.uk
- Zoing M, Kiernan M. Motor neurone disease: caring for the patient in general practice. Aust Fam Physician 2011; 40(12):962–6. www.racgp.org.au
- Joint Formulary Committee. British National Formulary (online) London: BMJ Group and Pharmaceutical Press. www.medicinescomplete.com, accessed 11 December 2014
- Radunovic A, Annane D, Rafiq MK, et al. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews 2013, Issue 3. doi:10.1002/14651858.CD004427.pub3
- Complementary therapies. Motor Neurone Disease Association. www.mndassociation.org, published August 2010
- Swallowing difficulties. Motor Neurone Disease Association. www.mndassociation.org, published August 2013
- Saliva control. Motor Neurone Disease Association. www.mndassociation.org, published November 2010
- Andersen PM, Abrahams S, Borasio GD, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) – revised report of an EFNS task force. Eur J Neurol 2012; 19(3):360–75. www.guideline.gov/content.aspx?f=rss&id=38469
- Understanding clinical trials. National Institute for Health Research. www.crn.nihr.ac.uk, published October 2010
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