Guillain-Barré syndrome

Guillain-Barré (pronounced ghee-lan bar-ray) syndrome is a rare and serious condition of the peripheral nervous system.

Information written and reviewed by Certified Doctors.

Contents

Introduction

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Guillain-Barré (pronounced ghee-lan bar-ray) syndrome is a rare and serious condition of the peripheral nervous system. It occurs when the body's immune system attacks part of the nervous system.

The exact cause of Guillain-Barré syndrome is unknown. However, most people (around 60%) develop the condition shortly after having a viral or bacterial infection. It is thought infection may trigger the immune system to attack nerve roots and peripheral nerves.

Read more about causes of Guillain-Barré syndrome.

The symptoms of Guillain-Barré syndrome usually develop one to three weeks after a minor infection, such as a cold, sore throat or gastroenteritis (an infection of the stomach and bowel).

Symptoms often start in your feet and hands before spreading to your arms and then your legs. Initially, you may have:

  • pain, tingling and numbness
  • progressive muscle weakness
  • co-ordination problems and unsteadiness (you may be unable to walk unaided)

The weakness usually affects both sides of your body, and it may get worse over several days.

Read more about the symptoms of Guillain-Barré syndrome.

The peripheral nervous system

The peripheral nervous system is the network of nerves that lie outside the central nervous system, including the motor nerves, which the brain uses to control the muscles.

In Guillain-Barré syndrome, the immune system (the body's natural defence against infection and illness) attacks these nerves, causing them to become inflamed and stop working.

Inflammation of the peripheral nerves causes a tingly, numbing sensation in the arms and legs. This may lead to impaired feeling and limb weakness. Sometimes, respiratory muscles are also involved.

Diagnosing Guillain-Barré syndrome

It can sometimes be difficult to distinguish the symptoms of Guillain-Barré syndrome from other brain and nervous system disorders. Therefore, two tests are usually used to confirm the diagnosis. They are:

  • nerve conduction studies and electromyography (EMG) – tests that measure nerve and muscle function
  • lumbar puncture – where a small needle is used to remove a small amount of fluid from the spinal canal for analysis

You may also have blood tests to rule out other possible causes.

Read more about how Guillain-Barré syndrome is diagnosed.

Treating Guillain-Barré syndrome

Guillain-Barré syndrome is treated in hospital in a general ward, a neurology ward or an intensive care unit (ICU), depending on the severity of the condition.

There are two main treatments:

  • intravenous immunoglobulin
  • plasma exchange (plasmapheresis)

These treatments target the antibodies (infection-fighting proteins) produced by your immune system to prevent them further damaging your peripheral nerves.

Intravenous immunoglobulin is slightly safer and easier to give than plasma exchange. However, both treatments are equally effective.

Read more about how Guillain-Barré syndrome is treated.

Recovery

Most people (about 80%) with Guillain-Barré syndrome make a full recovery. However, you may need to spend several weeks in hospital and it may take a year or more to fully recover.

Read more about recovering from Guillain-Barré syndrome.

Complications

About one or two people in every 10 will not recover completely from Guillain-Barré syndrome. Possible long-term complications include:

  • not being able to walk unaided – for example, needing a wheelchair
  • loss of sensation (sensory ataxia) that may cause a lack of co-ordination
  • loss of balance
  • muscle weakness in your arms or legs
  • problems with your sense of touch known as dysaesthesia – often felt as a burning or tingling sensation

Some people with Guillain-Barré syndrome also experience persistent fatigue (extreme tiredness).

Read more about the complications of Guillain-Barré syndrome.

Symptoms

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The symptoms of Guillain-Barré syndrome can develop quickly over a few hours. The muscle weakness often gets progressively worse within a few days.

The symptoms usually develop one to three weeks after a minor infection, such as a cold, sore throat or gastroenteritis (an infection of the stomach and large intestine).

Symptoms often start in your feet and hands before spreading to your arms and then your legs. Initially, you may have:

  • pain, tingling and numbness
  • progressive muscle weakness
  • co-ordination problems and unsteadiness (you may be unable to walk unaided)

The weakness usually affects both sides of your body and may get worse over a period of several days.

In mild cases of Guillain-Barré syndrome, your muscles may only be slightly weakened. However, in more severe cases, the muscle weakness can progress to:

  • temporary paralysis of the legs, arms and face
  • temporary paralysis of the respiratory muscles
  • blurred or double vision
  • difficulty speaking
  • difficulty chewing or swallowing (dysphagia), resulting in the need to be fed through a tube
  • difficulty with digestion or bladder control
  • fluctuations in heart rate or blood pressure

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

When to seek immediate medical assistance

See your doctor if you notice any of the above symptoms. However, seek immediate medical assistance if you experience any of the following symptoms:

  • difficulty breathing
  • difficulty swallowing
  • lightheadedness when you stand
  • fainting

Causes

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Guillain-Barré syndrome is an autoimmune condition (where the immune system attacks healthy cells and tissue). It is not known what causes the immune system to do this.

Most people develop Guillain-Barré syndrome after having another illness. This is usually a viral infection, such as the common cold, influenza, a throat infection, or sometimes a bacterial infection (see below). It is thought the infection may trigger the immune system to attack nerve roots and peripheral nerves.

It is not possible for Guillain-Barré syndrome to be transmitted from one person to another and it cannot be inherited.

Immune response

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 antibodies usually attack the bacteria or virus causing the infection. However, in Guillain-Barré syndrome the antibodies react to the cells of the protective covering (myelin sheath) of the peripheral nerves.

The reaction damages the nerves and slows down the nerve signals sent to your muscles. This causes the symptoms of Guillain-Barré syndrome, such as tingling, numbness, weakness and, in some cases, temporary muscle paralysis.

Possible triggers

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

Vaccination

In the past, vaccinations (particularly the swine flu vaccination used during 1976-77) were thought to be a possible cause of Guillain-Barré syndrome. However, subsequent studies have shown there is virtually no risk of developing Guillain-Barré syndrome after being vaccinated.

Diagnosis

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Your doctor may be able to diagnose Guillain-Barré syndrome from your symptoms and by carrying out an examination.

If you have been ill recently and have progressive muscle weakness or paralysis, you may have the condition.

Your doctor may test your knee-jerk reflex to check whether it is lost. The knee-jerk reflex is where your lower leg "kicks out" in response to your doctor tapping your leg just below the knee cap.

Guillain-Barré syndrome can sometimes be difficult to diagnose because symptoms vary from person to person, and they can also be similar to those of other brain and nervous system disorders.

Two tests are used to confirm the diagnosis: electromyography and lumbar puncture (see below). You may also have blood tests to rule out other possible causes.

Electromyography (EMG)

Electromyography (EMG) measures the health of your muscles and the nerves that control them.

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

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

Lumbar puncture

A lumbar puncture is a procedure carried out under local anaesthetic that involves inserting a needle into the lumbar spinal canal. This is the space in the lower spine (below the end of the spinal cord).

It is used to measure the protein levels (a measure of inflammation) and cell count in the cerebrospinal fluid, and to exclude other causes of nerve inflammation.

Read more about lumbar puncture.

Treatment

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There is no cure for Guillain-Barré syndrome, but there are a number of treatments that can reduce your symptoms and help your recovery.

You will be treated in hospital, where you will have a number of tests. Your condition will be closely monitored, including your breathing. Depending on the severity of your condition, you may be admitted to:

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

If you are having difficulty breathing, you may be put on a ventilator (a machine that assists with breathing). Around a quarter of people with Guillain-Barré syndrome need to use a ventilator.

Two main treatments can be used to reduce the severity of Guillain-Barré syndrome and help you recover more quickly. They are:

  • 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.

Intravenous immunoglobulin is slightly safer and easier to give than plasma exchange. However, both treatments are equally effective.

Intravenous immunoglobulin (IVIg)

Immunoglobulin is another name for antibodies. Antibodies are proteins in the blood that the immune system produces to destroy harmful bacteria and viruses. The immune system is the body's natural defence against illness and infection.

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 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 (thicken).

During plasma exchange, you will be connected to a machine that removes some of your blood. The plasma will be separated from the blood cells and removed. The blood cells will then be returned to your body without the harmful plasma cells that attack the nerves. Your blood cells will produce healthy plasma to replace the harmful plasma that was removed.

Depending on the severity of your condition, you may need several sessions of plasma exchange.

Read more about plasma exchange.

Monitoring your condition

While being treated for Guillain-Barré syndrome, you may need to spend several weeks or months in hospital. This is so you can be closely monitored and your breathing, heart rate and blood pressure can be regularly checked.

You may be given painkillers if you are in pain, plus 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 hospital ward or a rehabilitation ward before being discharged. You will continue receiving treatment to help your recovery.

Read more about recovering from Guillain-Barré syndrome.

Corticosteroids

Corticosteroids are not recommended for the treatment of Guillain-Barré syndrome. Although corticosteroids are often used to reduce inflammation, there is no evidence to suggest they offer a significant benefit in treating this condition.

Complications

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It is estimated that one or two 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 (sensory ataxia) that may cause a lack of co-ordination
  • loss of balance
  • muscle weakness in your arms or legs
  • problems with your sense of touch known as dysaesthesia, which may be felt as a burning or tingling sensation

Some people with Guillain-Barré syndrome also have persistent fatigue (extreme tiredness).

Life-threatening complications

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

  • respiratory failure – where your lungs are unable to provide enough oxygen for the rest of your body
  • infections – particularly respiratory infections in people who are on a ventilator (a machine that assists with breathing)
  • heart rhythm disorders – including cardiac arrest
  • bowel obstruction

Recovery

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Most people (about eight out of 10) with Guillain-Barré syndrome make a full recovery.

It usually takes several weeks to recover after the start of symptoms. However, some people will need to spend longer in hospital or a neurological rehabilitation unit. While some people recover quickly, it can take a year or more before a full recovery is made.

You may not be able to walk unaided, have numbness or weakness in your arms or legs, and you may have persistent fatigue (extreme tiredness).

Read more about the complications of Guillain-Barré syndrome.

The following therapies may be used to help your recovery.

Counselling

Counselling is a talking therapy that you may find useful if you have Guillain-Barré syndrome, or while you are recovering from it. During counselling you will be able to:

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

Physiotherapy

Physiotherapy can help restore movement and function to a person's full potential.

A range of techniques are used to help relieve any pain, discomfort and muscle stiffness. These include:

  • movement and exercise – taking into account a person's current level of health and specific requirements
  • manual therapy – where the physiotherapist uses their hands to relieve pain and stiffness
  • aquatic therapy – a form of physiotherapy carried out in water
  • other techniques – such as heat, cold and acupuncture to ease pain

Occupational therapy

An occupational therapist will be able to identify any problems that Guillain-Barré syndrome causes in your everyday life, and help you work out practical solutions.

For example, if you have difficulty walking unaided, an occupational therapist will be able to help you find a suitable walking stick, walking frame or wheelchair.

Read more about occupational therapy.

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