Guillain-Barré syndrome affects the nerves of the limbs and body and is usually triggered by an infection. The main symptom is weakness of the muscles that are supplied by the affected nerves. It requires immediate hospital admission as it can rapidly become very serious. With appropriate treatment and monitoring, most people make a full recovery.
Guillain-Barré syndrome (GBS) is a disease that affects nerves throughout the body and limbs. It is usually triggered by an infection. It is named after Dr Guillain and Dr Barré who were two of the doctors who first described this syndrome early in the last century.
GBS is rare but may affect 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 1,500 people develop GBS in the UK.
Most cases of GBS start within three weeks of an infection. Some of the infections that are known to be related to GBS include Campylobacter jejuni (which can cause bowel infection and diarrhoea), Epstein Barr virus (which causes glandular fever - also called infectious mononucleosis), cytomegalovirus (usually causes no symptoms) and Mycoplasma pneumoniae infection (a bacterial infection that can cause a cough, cold or pneumonia) - but there are probably others. For example, HIV and influenza A virus are also possible infections that have been linked to GBS. (Note: the vast majority of people who get these infections do not develop GBS.) Rarely, GBS has been reported after certain vaccinations.
GBS is an autoimmune disease. The immune system normally makes antibodies (small proteins) to attack bacteria, viruses, and other germs. In autoimmune diseases, the immune system makes antibodies that can attack tissues of the body and cause damage to them. In GBS, the preceding (earlier) infection stimulates the body to produce antibodies to attack the infecting germ. But, it is thought that a tiny part of the infecting germ and tiny parts of nerves in the body 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, causing inflammation of the nerves and leading to GBS. If a nerve is damaged, then the muscles which 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.
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 and when they peak can vary from person to person, but often symptoms can be severe. In many cases, both arms and legs become so weak that they cannot be used at all. In addition, in about 1 in 4 cases, the muscles of the chest become so weak that breathing is affected. If this occurs, you will need support to breathe with a ventilator (a breathing machine) in an intensive care unit. Swallowing may be affected and you may need to be fed via a tube passed into your 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. Then the symptoms gradually start to ease off as the damaged nerves begin to heal. The time it takes to recover varies greatly from person to person, but it is often several months.
Other tests including blood tests, spirometry (a test to show how well you breathe in and out) and an electrocardiogram (ECG - records the electrical activity of your heart) may also be suggested. These can help to check for complications of GBS and to monitor progress.
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 part of treatment. For example, you may 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 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 clear. It may work to alter your immune system and help to clear the abnormal antibodies. People with GBS who receive immunoglobulin, on average, have a quicker time to recovery compared with those who do not have this treatment. It is thought that the sooner treatment is started after symptoms begin, the better the effect this treatment is likely to have.
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 probably just as effective, is easier to give, and seems to cause fewer side-effects.
You will usually be given special stockings to wear and blood-thinning (heparin) injections to try to prevent a clot in your leg (DVT) which may occur as a result of being immobile.
This may be with particular drugs that work well for nerve-related pain, such as gabapentin and carbamazepine.
These may also be helpful treatments for GBS. Physiotherapy may help with pain relief and may also help to prevent or treat muscle stiffness that may develop. Occupational therapists help people achieve as much as they can for themselves. For example, they may help someone recovering from GBS to walk by themselves again with the aid of a walking stick.
Low mood can be a problem for many people with GBS. You may find it helpful to talk through some of your feelings with a counsellor and to get support from them. The Guillain-Barré Syndrome Support Group (contact details below) provides information and support to those affected by GBS.
In general, steroids are not thought to help much in the treatment of GBS and so are not routinely used. However, there is some research that suggests that steroids given intravenously (into a vein) at the same time as immunoglobulin treatment may help to speed up recovery. More research is needed about this.
Due to improvements in managing GBS, around 7-8 in 10 people will make a full recovery in 6-12 months. However, you may be in hospital for a few months. About 1-2 in 10 people with GBS 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. This 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.
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Helpline: 0800 374803 Web: www.gbs.org.uk