Guillain-Barré syndrome

Guillain-Barré (pronounced Ghee-lan Bar-ray) syndrome is a rare but serious condition of the peripheral nervous system. It is named after two of three French doctors who first described the condition in 1916, Georges Charles Guillain and Jean-Alexander Barré.

The peripheral nervous system

The peripheral nervous system is the network of nerves that control the body's senses and movements. In Guillain-Barré syndrome, the immune system (the body's natural defence against infection and illness) attacks these nerves, causing them to become inflamed.

Inflammation of the peripheral nerves leads to a tingly, numbing sensation in the arms and legs. This can eventually result in a short-term loss of feeling and movement (temporary paralysis). See Guillain-Barré syndrome - symptoms for more information.

Link with infection

The exact cause of Guillain-Barré syndrome is unclear. There is no way of identifying who is most at risk.

However, in most cases of Guillain-Barré syndrome, the person affected had a viral or bacterial infection a few weeks before getting the condition. It is likely that the infection causes the immune system to attack the body's nerves.

How common is Guillain-Barré syndrome?

Guillain-Barré syndrome affects 50-100 people in Ireland each year. It is slightly more common in men than women. It can affect people of any age, including children.

Outlook

Most people (about 8 out of 10) with Guillain-Barré syndrome make a full recovery within a few weeks or months, and do not have any further problems. Some people may take longer to recover, and there is a possibility of permanent nerve damage. See Guillain-Barré syndrome - complications for more information.

Inflammation
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.

 

Guillain-Barré syndrome usually develops quickly, over a few days. However, it can sometimes develop gradually, over a period of up to four weeks.

Once the symptoms are at their worst point, they usually remain at this level for a few days before you gradually begin to recover.

The symptoms first appear one to three weeks after a minor infection, such as a cold or sore throat.

Initial symptoms

The initial symptoms of Guillain-Barré syndrome tend to start in the feet and hands. They include:

  • tingling
  • numbness
  • unsteadiness
  • progressive muscle weakness

These symptoms soon spread to the limbs, affecting the legs and arms a few days later.

You may find that you only have a mild form of the condition and that your symptoms do not get any worse than a general feeling of weakness. If this is the case, you may need a walking frame or a crutch to help you walk.

Possible later symptoms

In some people, the muscle weakness progresses to:

  • temporary paralysis of the legs, arms and face
  • temporary paralysis of the respiratory muscles, which control breathing (see below)
  • difficulty with eye movement and speech
  • difficulty with chewing or swallowing, resulting in feeding through a tube
  • difficulty with digestion or bladder control
  • slow heart rate or low blood pressure

Some people with Guillain-Barré syndrome do not have any pain, but others feel severe pain in their spine, arms and legs.

Paralysis

The extent of paralysis varies with each case of Guillain-Barré syndrome.

Guillain-Barré syndrome can be life-threatening because it can paralyse your respiratory muscles and stop you breathing. If this happens, you will need to be put on a ventilator (a machine that helps you to breathe). About one in four people need to be artificially ventilated.

If you start to feel a tingling sensation in your toes, feet or legs, followed by muscle weakness, visit your GP immediately .

See the Health A-Z topic about Paralysis for more information.

 

It is unclear what causes Guillain-Barré syndrome. However, most people develop the condition following a recent illness. This is usually a viral infection, such as a throat infection or flu, or sometimes a bacterial infection.

It is thought that the infection may trigger the immune system (the body's natural defence against infection and illness) to attack the body.

The immune system

The immune system uses white blood cells to produce antibodies. Antibodies are infection-fighting proteins that travel around the body in the blood vessels and lymphatic vessels (part of the lymphatic system).

The role of antibodies is to attack the bacteria or virus that is causing the infection. However, in this case, the antibodies react to the cells of the protective covering (myelin sheath) of the peripheral nerves (see box, right).

This reaction damages the nerves and prevents them from sending signals to your muscles. This causes the tingling, weakness and eventual short-term paralysis that you might experience.

Possible triggers

Although some cases occur without a known cause, some cases of Guillain-Barré syndrome are related to:

  • a virus, such as the cytomegalovirus (a member of the herpes group) or the HIV infection
  • a bacterial infection, such as infection from Campylobacter bacteria
  • Hodgkin's lymphoma - tumour of the lymph glands (the small, bean-sized organs that carry white blood cells)

Vaccination has also been linked to Guillain-Barré syndrome. However, studies suggest a very low risk of developing the syndrome after receiving a vaccine.

For example, since the swine flu pandemic in 2009, around 6 million doses of the vaccine Pandemrix have been given in the UK. The Medicines and Healthcare products Regulatory Agency (MHRA), which monitors the safety of vaccines, received 15 suspected reports of Guillain-Barré syndrome during the pandemic. The diagnosis was not confirmed in any of these cases. A similar situation applies in Ireland.

You cannot catch Guillain-Barré syndrome from other people or inherit it from your parents.

Lymphatic system
The lymphatic system is made up of a network of vessels (channels) and glands called lymph nodes that are distributed throughout the body. It helps fight infection.
White blood cells
White blood cells are the part of blood that fight infection and disease.

What is the peripheral nervous system?

The peripheral nervous system is the network of nerves that control the body's senses and movements. They extend out from the brain and spinal cord (the central nervous system) and carry impulses to and from the rest of the body, including the limbs and organs.

Your GP may be able to diagnose Guillain-Barré syndrome by examining you and checking your symptoms. They will usually test your knee-jerk reflex to see if this is lost. The knee-jerk reflex is where your lower leg 'kicks out' in response to your GP tapping your leg just below your knee cap. 

Guillain-Barré syndrome can sometimes be difficult to diagnose because the symptoms vary from person to person. The symptoms can also be very similar to those of other brain and nervous system disorders. Therefore, two tests are used to confirm the diagnosis of Guillain-Barré syndrome. They are:

  • electromyography 
  • lumbar puncture

You may also have a blood test to rule out any other possible causes.

Electromyography (EMG)

Electromyography is a test that measures the health of the muscles and the nerves that control them.

Testing the muscles

To test the muscles, a needle electrode is inserted through the skin and into the muscle to give an electrical recording of your muscle activity. This helps to determine whether your muscles respond when certain nerves are stimulated. In Guillain-Barré syndrome, the muscles may not respond due to nerve damage.

Testing the nerves

To test the nerves, a surface electrode (small metal disc) is stuck to your skin to stimulate the nerves with a small electric shock. The response from them is measured. It measures how quickly your nerves conduct electric signals. If you have Guillain-Barré syndrome, nerve conduction will be slower than normal.

Lumbar puncture (spinal tap)

A lumbar puncture involves inserting a needle into the spinal canal (usually in the lower back), under local anaesthetic (painkilling medication). This is done to:

  • determine the pressure of the spinal fluid inside
  • take a sample of the spinal fluid and test it for evidence of bleeding in the brain and spinal cord
  • check the protein levels in the spinal fluid (this is often raised in Guillain-Barré syndrome) and look for any bacteria or fungi

See the Health A-Z topic about Lumbar punctures for more information about this procedure.

Blood tests

Possible blood tests include: 

  • testing for antibodies - antibodies are infection fighting proteins that are produced by the immune system; in Guillain-Barré syndrome specific antibodies may be present that are known to react to your peripheral nerves
  • erythrocyte sedimentation rate (ESR) - this measures how long it takes the red blood cells in a sample of blood to sink to the bottom of a test tube; the time it takes indicates how much inflammation (swelling) there is in your body 
Antibodies
Antibodies are proteins that are produced by the body to neutralise or destroy disease-carrying organisms and toxins.
Immune system
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.
Inflammation
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.
Peripheral nerves
The peripheral nerves are the network of nerves that run from your brain and spinal cord and carry impulses to and from the rest of your body, such as the limbs and organs. They are responsible for senses and movements.

If you have Guillain-Barré syndrome, you will initially be treated in hospital as an emergency, and your respiration rate (breathing) will be carefully monitored. Depending on your condition, you may be treated in:

  • a general ward
  • a neurology ward - a ward specialising in neurological conditions (conditions affecting the nervous system) 
  • an intensive care unit (ICU) - a special ward that provides intensive care (treatment and monitoring) for people who are in a critically ill or unstable condition
  • a high-dependency unit - one step down from an ICU, more intense treatment is given in a high-dependency unit than in a general ward

If you have breathing problems, you may be put on a ventilator (a machine that helps you to breathe). Around a quarter of people with Guillain-Barré syndrome will need a ventilator.

There are two main treatments to reduce the severity of Guillain-Barré syndrome and help you recover more quickly:

  • intravenous immunoglobulin 
  • plasma exchange (plasmapheresis)

These treatments target the antibodies (infection-fighting proteins) in your blood that are reacting to your peripheral nerves and damaging them. See Guillain-Barré syndrome - causes for more information about the role that antibodies play in causing the condition.

Intravenous immunoglobulin is slightly safer and much easier to give than plasma exchange. However, both types of treatment are equally effective.

Intravenous immunoglobulin (IVIg)

Immunoglobulin is another name for antibodies. These are the proteins in your blood that are produced by your immune system (the body's natural defence against infection and illness) to destroy harmful bacteria and viruses.

During IVIg, healthy immunoglobulin is taken from blood donors and given to you intravenously (directly into a vein). The healthy antibodies block and destroy the harmful antibodies that are attacking your nerves. You will usually receive a dose of IVIg every day for around five days.

Plasma exchange (plasmapheresis)

Plasma is the yellowish fluid that is found in blood. It transports blood cells and platelets around the body and contains a number of substances, including proteins. Platelets are cell fragments that help the blood to clot.

During plasma exchange, you are connected to a machine that removes some of your blood. The plasma is separated from the blood cells and removed. The blood cells are then put back into your body without the harmful plasma cells that attack the nerves. Your blood cells will then go on to produce healthy plasma to replace what was taken.

You may have several sessions of plasma exchange depending on the severity of your condition.

See the Health A-Z topic about Plasma products - how they are used for more information about the procedure.

Monitoring your condition

If you are being treated for Guillain-Barré syndrome, you may need to spend several weeks in hospital. During this time you will be closely monitored to check your breathing, heart rate and blood pressure.

As well as the treatments explained above, you may be given painkillers if you are in pain, and any other necessary medication. For example, if you develop a problem with your heart rate or blood pressure, you may need further medication to treat this.

Once you start to recover, you may be moved to a general ward in the hospital before being discharged. You may continue to receive treatment to help your recovery. See Guillain-Barré syndrome - recovery for more information.

Corticosteroids

Corticosteroids are a type of medicine that contains steroids (a type of hormone). Hormones are powerful chemicals that have a wide range of effects on the body.

Corticosteroids are often used to reduce inflammation. In theory, this should reduce the nerve damage that occurs in Guillain-Barré syndrome. However, so far there is no evidence to show a significant benefit for using corticosteroids for this condition. 

One review looking at the evidence for corticosteroids found that intravenous corticosteroids (given directly into a vein) might speed up recovery if they are given with intravenous immunoglobulin. However, corticosteroid tablets delayed recovery. More research is still needed to find out whether corticosteroids are of real use for treating Guillain-Barré syndrome.

Antibodies
Antibodies are proteins that are produced by the body to neutralise or destroy disease-carrying organisms and toxins.
Immune system
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.
Inflammation
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.
Peripheral nerves
The peripheral nerves are a network of nerves that run from your brain and spinal cord and carry impulses to and from the rest of your body, such as the limbs and organs. They are responsible for senses and movements.

About 8 in 10 people with Guillain-Barré syndrome will make a complete recovery.

It usually takes a few weeks or months after the start of symptoms for most people to recover. However, some people will need to spend a few months in hospital. It may take up to a year for them to fully recover.

See Guillain-Barré syndrome - complications for information about what problems can occur.

The following therapies may be used to aid your recovery.

Counselling

Counselling, a type of talking therapy, can be reassuring and can help you to recover from Guillain-Barré syndrome. It aims to help you to:

  • discuss your problems honestly and openly
  • address any issues that are preventing you from achieving your goals and ambitions
  • achieve a more positive outlook on life

The Guillain-Barré Syndrome Support Group is a UK based charity that offers support and information to those affected by the syndrome in the UK and Ireland.

Physiotherapy

Physiotherapy can also help to relieve discomfort and prevent muscle stiffness. It involves a range of treatments, including:

  • manipulation
  • massage
  • exercise
  • electrotherapy - which uses different types of energy, such as electric currents
  • hydrotherapy - physiotherapy that is carried out in water, such as a warm, shallow swimming pool

See the Health A-Z topic about Physiotherapy for more information about this type of treatment.

Occupational therapy

An occupational therapist can identify any problems that Guillain-Barré syndrome causes in your everyday life. They will help you work out practical solutions. For example, if you have difficulty walking unaided, an occupational therapist can help you find a suitable walking stick, walking frame or wheelchair.

See the Health A-Z topic about Occupational therapy for more information about this type of treatment.

It is estimated that 1 or 2 people in every 10 will not recover completely from Guillain-Barré syndrome.

Long-term complications

Possible long-term complications include: 

  • not being able to walk unaided - for example, needing a wheelchair 
  • loss of sensation (numbness)
  • lack of co-ordination caused by the loss of sensation (sensory ataxia)
  • weakness - for example, in your arms or legs
  • loss of balance  
  • problems with your sense of touch (dysesthesia), which can cause a burning or tingling sensation in your skin; or abnormally sensitive skin that causes severe pain when you come into contact with objects, such as bedding or towels

Many people with Guillain-Barré syndrome also have persistent fatigue (long-term tiredness), and nearly two thirds of people still notice the effects of the condition three to six years after first having it.

Life-threatening complications

There is a small chance (about 1 in 20) of dying from Guillain-Barré syndrome. This usually occurs as a result of compilations that develop during the first few weeks of the condition. For example:

  • respiratory distress syndrome - the lungs are unable to provide enough oxygen for the rest of the body 
  • sepsis - the body develops a severe local or generalised infection
  • pneumonia - inflammation of the tissue in one or both of your lungs
  • cardiac arrest - the heart stops beating
Inflammation
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.

Content provided by NHS Choices www.nhs.uk and adapted for Ireland by the Health A-Z.

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