Guillain-Barré Syndrome

What is Guillain-Barré syndrome?

Guillain-Barré syndrome (GBS) is a disease that affects nerves throughout the body and limbs. It is usually triggered by an infection. The syndrome is named after Dr Guillain and Dr Barré who first described this syndrome early in the last century.

Who gets GBS?

GBS is rare but can occur in anyone. It occurs more commonly in early adulthood and the elderly. It is also more common in women immediately after they have given birth. Every year about 1500 people are affected with GBS in Great Britain.

What causes GBS?

Most cases of GBS start within three weeks of an infection. The infections that are known to be related to GBS include Campylobacter jejuni (which can cause bowel infections and diarrhoea), Epstein Barr virus (which causes glandular fever, also called infectious mononucleosis) and Cytomegalovirus (usually causes no symptoms) – but there are probably others. For example, HIV and Hepatitis B are also possible causes.

GBS is an autoimmune disease. This means that the body makes proteins, called antibodies, that attach to and damage parts of the body. In GBS, the preceding (earlier) infection stimulates the body to produce antibodies which attack the infecting germ. But, what is thought to happen is that a tiny part of the infecting germ and tiny parts of the nerves have similar structures. In some people the antibodies that attack the germ also ‘mistake’ the nerves as germs. So, the antibodies also attach to and damage the nerves, which causes GBS. If a nerve is damaged, then the muscles that the nerve supplies stop working.

Note: there are various other autoimmune diseases. Most tend to run a chronic (persistent) course once the immune system is triggered. For example, rheumatoid arthritis. However, GBS is different in that the disease activity seems to be 'time limited'. That is, the immune system 'reacts' to a recent infection which can cause nerve damage, but then settles down again. This is why most people with GBS fully recover.

What are the symptoms of GBS?

• Weakness. This starts in the extremities such as the feet and hands, and moves upwards towards the centre of the body. The legs are affected more often than the arms. If the nerves in the head, neck and chest are affected, there may be difficulty with swallowing, eye movements and breathing. Reflexes, such as the way the leg moves when the knee is tapped, are also lost early in most people.
• Sensory problems. This may include tingling or numbness.
• Pain. About 1 in 2 people with GBS develop either nerve pain or deep aching muscle pain. This tends to be worse with movement and at night.
• Autonomic symptoms. The autonomic nervous system is responsible for body functions like sweating, breathing, heart beating and digestion. In GBS, nerves within this system may be affected causing problems with blood pressure, pulse, vision and lack of sweating.

How does the disease progress?

Usually, the weakness and sensory symptoms gradually become worse and spread up the body. Symptoms typically peak in severity 1-4 weeks after first starting. The severity of symptoms when they 'peak' can vary from person to person, but often they are severe. In many cases, both arms and legs become so weak they cannot be used at all. In addition, in about 3 in 10 cases, the muscles of the chest become so weak that breathing is affected. If this occurs, you will need support to breath with a ventilator (breathing machine) in an intensive care unit. Swallowing may be affected and you may require to be fed by via a tube passed into the stomach.

In some cases, the weakness does not progress much further than the lower legs. This may only cause some moderate difficulty in walking which may require a walking stick.

Once symptoms peak, they tend to remain in a stable 'plateau' phase for a while. Typically, this is for several days before the symptoms then gradually start to ease off as the damaged nerves begin to heal. The time it takes to recover varies greatly between people, but it is often several months.

How is GBS diagnosed?

• Examination. GBS is usually first suspected after an examination by a doctor. This usually reveals weakness in the arms and legs, no reflexes, and mild numbness. The signs are usually equal on both sides of the body.
• Cerebrospinal Fluid (CSF) sample. This is the fluid that surrounds the spinal cord and brain. A sample of this is collected through a needle in the back and will show a high amount of protein and not very many white blood cells (blood cells that increase in infection).
• Electrodiagnostic tests. These are tests of the electrical activity of the nerves and will usually show a typical pattern in GBS.

Blood tests and lung function tests are also done. This is usually to check for complications of the disease and to monitor progress.

What is the treatment for GBS?

If your doctor suspects GBS, you will be sent to hospital. This is because the disease may progress quickly within a few days to affect your breathing and heart. You need to be monitored closely in hospital so that these complications can be treated quickly if they occur. Good supportive care is the most important aspect of treatment. For example, if you need help and support with feeding and breathing until the symptoms ease.

Some specific treatments that may be given include one or more of the following:

Immunoglobulin

Immunoglobulin is an antibody that is present in blood and is collected from blood given by blood donors. It is given as an injection directly into a vein. The precise way that it works is not clearly known. It may work to alter the immune system and help to clear the abnormal antibodies. People with GBS who receive immunoglobulin, on average, have a quicker time to recovery compared to those who do not have this treatment. The sooner treatment is started after symptoms begin, the better the effect this treatment is likely to have.

Plasma exchange

Your blood is made up of blood cells and plasma. Plasma is where the damaging antibodies are present. Plasma exchange involves taking your blood out of your body and separating the blood into cells and plasma. The plasma is then removed and the blood cells are returned to the body with a plasma substitute. A special machine is used for this treatment and only a small amount of blood is actually outside the body at any time. Again, the sooner treatment is started after symptoms begin, the better the effect this treatment is likely to have. However, plasma exchange is done less commonly these days since treatment with immunoglobulin has been shown to be just as effective, easier to give, and to cause fewer side-effects.

Deep vein thrombosis (DVT) prevention

Special stockings are worn and blood thinning (heparin) injections are given to try to prevent a clot in the leg (DVT) which may occur as a result of being immobile.

Pain relief

This may be with particular drugs that work well for nerve related pain such as gabapentin and carbamazepine.

Steroids used to be given as a treatment. However, research trials have been shown that they make no difference in the treatment of GBS and are no longer recommended.

What is the outlook (prognosis) of the disease?

Due to improvements in managing GBS, more than 8 in 10 people will make a full recovery in 6-12 months. About 1 in 10 people will be left with some degree of permanent problems such as some weakness, muscle wasting, difficulty walking or pain. About 1 in 20 people with GBS will die. Death is usually due to severe breathing problems, problems with the rhythm of the heart, or infections (which you are more prone to get if you become immobile).

It is rare to have GBS a second time.

If you have a relative or friend in hospital with severe GBS you will inevitably be very worried by what is happening. But remember: total paralysis due to GBS is compatible with total recovery. Hang on to that fact through the grim days. Also, as appropriate, communicate this optimism to your relative or friend.

Further help and information

Guillain-Barré Syndrome Support Group

LCC Offices, Eastgate, Sleeford, NG34 7EB
Helpline: 0800 374803 Web: www.gbs.org.uk