Guillain-Barré Syndrome (GBS)

otherwise known as
"Acute Inflammatory Demyelinating Polyneuropathy" or "Getting Better Slowly"

GBS Articles please select from list For the medically orientated GBS - a Carer's guide GBS - a GP's guide to diagnosis GBS - an overview for the layperson GBS Patient in Intensive Care (Red book) Guillain-Barré Syndrome (Blue book) "Classic" Guillain-Barré Syndrome

IF YOU'VE FOUND THIS PAGE: then you or someone close to you are probably a victim of Guillain-Barré Syndrome or one of it's variants like CIDP, AIDP or the Miller-Fisher variant.  This website contains only a brief introduction; your own further investigation is warranted and strongly recommended.  Guillain-Barré Syndrome, also known as Landry's ascending paralysis, acute idiopathic polyradiculoneuritis and acute idiopathic polyneuritis, is an inflammatory disorder of the peripheral nerves. It paralyses the nerves of the arms, legs, lungs and neck, as well as those controlling the eyes, throat and heart. It frequently follows a viral infection. In 1859, French physician Jean Landry first described the symptoms of the disorder, and soon several other reports of a similar disorder appeared. In 1916, Georges Guillain, Jean Alexander Barré and Andre Strohl documented the disease's characteristic abnormality of increased spinal fluid protein production, but normal cell count.

Guillain-Barré is called a syndrome rather than a disease because it is not clear that a specific disease-causing agent is involved.  Guillain-Barré Syndrome affects 10 to 20 million people each year and is neither hereditary nor contagious.  Mortality rates range from 5%-10%

GBS is short for 'Guillain-Barré syndrome' (pronounced ghee-yan bah-ray). It is an acute disease of the peripheral nervous system in which the nerves in the arms and legs become inflamed and stop working. The first symptoms of this disorder include sudden weakness leading to limb paralysis, and a loss of sensation (tingling), sometimes with pain. GBS is normally an acute illness but about ten per cent of patients have a chronic variant called CIDP. These symptoms can increase in intensity until certain muscles cannot be used at all and, when severe, the patient is almost totally paralysed.

CIDP, GBS, and Miller-Fisher variant are all diseases which can best be described as a process in which the myelin sheath surrounding the peripheral nerves throughout one's body are damaged.  This damage is done through an "attack" on this insulation surrounding the nerves going from the brain to the muscles. 

GBS has a short, severe course. It causes inflammation and destruction of the myelin sheath, and it disturbs multiple nerves. Therefore, it is considered an acute inflammatory demyelinating polyneuropathy.  These nerves send messages from the brain to the muscles, instructing the muscles to move, and also carry sensations such as pain from the body to the brain.  In these cases the disorder is life threatening – potentially interfering with breathing and, at times, with blood pressure or heart rate – and is considered a medical emergency. Such a patient is often put on a respirator to assist with breathing and is watched closely for problems such as an abnormal heart beat, infections, blood clots, and high or low blood pressure. 

(a) top. Normal myelinated fibre with nerve impulse being conducted from node to node, causing muscle contraction.
(b) bottom. Autoimmune attack on myelin causes a block in nerve conduction, preventing muscle contraction. Plasmapheresis and intravenous immunoglobulin inhibit immune-mediated attack on myelin, allowing remyelination to commence and nerve conduction to be restored. Illustration for Modern Medicine by Chris Wikoff

Anyone: young or old, male or female. The illnesses are neither hereditary nor contagious. GBS affects about 1500 people every year in the United Kingdom; the incidence of CIDP is perhaps one tenth that of GBS.  It can strike at any age and both sexes are equally prone to the disorder. The syndrome is rare, however, afflicting only about one person in 100,000. Usually Guillain-Barré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection. Occasionally surgery or vaccinations will trigger the syndrome. The disorder can develop over the course of hours or days, or it may take up to 3 to 4 weeks. Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear, and by the third week of the illness 90 percent of all patients are at their weakest.  

No one is sure. The classic scenario in 60% of GBS, involves a patient who has just recovered from a typical, seemingly uncomplicated viral infection. a throat or intestinal infection or influenza in the previous two weeks. These infections seem to trigger an incorrect response in the immune system which attacks the nerves.  The immune system becomes confused, and is not able to distinguish between foreign invaders and the body itself. Elements of the immune system are unleashed against areas of the body, resulting in damage and destruction. For some reason, in the case of GBS, the myelin sheath appears to become a target for the body's own immune system.

The cause of the weakness and paralysis of GBS is the loss of myelin, which is the material that coats nerve cells (the loss of myelin is called demyelination).  Myelin is an insulating substance which is wrapped around nerves in the body, serving to speed conduction of nerve impulses. Without myelin, nerve conduction slows or stops.  The nerve damage often causes muscle weakness (often to the point of paralysis), and can cause abnormalities of sensation, including pain, tingling, "crawling skin," or a certain amount of numbness.  That is why the muscles begin to lose their ability to respond to the brain's commands, commands that must be carried through the nerve network. The brain also receives fewer sensory signals from the rest of the body, resulting in an inability to feel textures, heat, pain, and other sensations. Alternately, the brain may receive inappropriate signals that result in tingling, "crawling-skin," or painful sensations. Because the signals to and from the arms and legs must travel the longest distances they are most vulnerable to interruption. Therefore, muscle weakness and tingling sensations usually first appear in the hands and feet and progress upwards.

First symptoms are usually tingling and numbness in the fingers and toes with progressive weakness in the arms and legs during the next few days.  In some cases the weakness progresses and leads to complete paralysis of the legs, the arms may also be affected.  Symptoms affect both sides of the body simultaneously, a characteristic that helps distinguish GBS from other causes of weakness and paresthesia. Normal reflexes are first diminished, then lost. The weakness eventually affects all the voluntary muscles, resulting in paralysis. When those muscles necessary for breathing become paralysed, the patient must be placed on a mechanical ventilator which takes over the function of breathing. This occurs about 30% of the time. Very severely ill GBS patients may have complications stemming from other nervous system abnormalities which can result in problems with fluid balance in the body, severely fluctuating blood pressure, and heart rhythm irregularities.  In a quarter of cases the paralysis progresses up the chest and the patient is unable to breathe on his or her own and needs to rely on a mechanical breathing machine (ventilator). The throat and face may be affected making swallowing impossible and so the patient needs to be fed by tube up the nose or directly into the stomach.

What makes Guillain-Barré syndrome a medical emergency is that the weakness can affect the chest muscles responsible for breathing. If chest muscles become paralysed, the patient can die from lack of oxygen. The patient must be carefully monitored, usually in a hospital, to make sure that breathing and other vital functions are maintained.  Very severely ill GBS patients may have complications stemming from other nervous system abnormalities which can result in problems with fluid balance in the body, severely fluctuating blood pressure, and heart rhythm irregularities.

Since laboratory tests can not specifically diagnose GBS, doctors must recognize the disease form its pattern of symptoms. The signs and symptoms of GBS are quite varied, so it can be quite difficult to diagnose in early stages.  Diagnosis is difficult because the symptoms may be confused with those of other conditions.   Diagnosis of GBS is made by looking for a particular cluster of symptoms (progressively worse muscle weakness and then paralysis), and by examining the fluid that bathes the brain and spinal canal through cerebrospinal fluid (CSF) analysis.

Two confirmatory tests may be helpful and are performed in most cases:

• lumbar puncture (spinal tap) — under a local anaesthetic, a needle is inserted between the third and fourth lumbar vertebrae or into the subarachnoid space to obtain cerebrospinal fluid (CSF) or analysis

• electromyogram (EMG) — an electrical recording of nerve conduction and muscle activity.

• Nerve Conduction Velocity Test — In this test, electrodes are placed on the skin over the nerve to be tested. An electrical impulse is fed through one electrode, and the speed of transmission of the stimulus is picked up and measured by the other electrodes. Because Guillain-Barré syndrome affects the myelin sheath coating the nerves, nerves affected by the disorder will conduct signals more slowly than unaffected nerves.

What is the treatment for GBS?

With the lack of knowledge of the cause of these neurological diseases comes an uncertainty of how to go about treatment in order to both halt the damage that has occurred and prevent further deterioration of the body.  There is no direct treatment for GBS. Instead, treatments are used that support the patient with the disabilities caused by the disease. The progress of paralysis must be carefully monitored, in order to provide mechanical assistance for breathing if it becomes necessary. Careful attention must also be paid to the amount of fluid the patient is taking in by drinking and eliminating by urinating. Blood pressure, heart rate, and heart rhythm also must be monitored.

GBS improves spontaneously. However, certain factors can assist recovery:

• good nursing and medical/intensive care;

• immunoglobulin — the infusion of immunoglobulin proves successful with similar results to plasmapheresis; or immune serum globulin (gamma globulin) is a relatively small portion of the plasma which contains the antibodies or chemical substances which help fight disease caused by invading bacteria and viruses.

• plasmapheresis — the exchange of blood plasma generally reduces the duration of the disease in severe cases if carried out in the first few days; and removing some desired fraction then returning the red cells and repeating the whole process

• physiotherapy and hydrotherapy, therapies that relieve discomfort and prevent stiffness;

• counselling to reassure the patient and encourage the patient towards recovery.

The most critical part of the treatment for this syndrome consists of keeping the patient's body functioning during recovery of the nervous system.  This can sometimes require placing the patient on a respirator, a heart monitor, or other machines that assist body function. The need for this sophisticated machinery is one reason why Guillain-Barré syndrome patients are usually treated in hospitals, often in an intensive care ward. In the hospital, doctors can also look for and treat the many problems that can afflict any paralysed patient – complications such as pneumonia or bed sores.

Guillain-Barrè syndrome can be a devastating disorder because of its sudden and unexpected onset. In addition, recovery is not necessarily quick.   About 50% of GBS patients make reasonably good recoveries. However, 30% of adult patients, and a greater percentage of children, experience permanent neurological damage and never fully regain their previous level of muscle strength. Some of these patients suffer from residual weakness, others from permanent paralysis. About 10% of GBS patients begin to improve, then suffer a relapse. These patients suffer chronic GBS symptoms. About 5-8% of all GBS patients die, most from cardiac rhythm disturbances, sepsis or pulmonary emboli.

Although Guillain-Barré syndrome is a terrifying disorder, the long-term prognosis is generally good. Most patients experience full recovery, although it can take months — or even years — to regain pre-illness strength and movement. Guillain-Barrè syndrome patients face not only physical difficulties, but emotionally painful periods as well. It is often extremely difficult for patients to adjust to sudden paralysis and dependence on others for help with routine daily activities. Patients sometimes need psychological counselling to help them adapt.

See my links page for further details. 
Support groups are extremely useful and provide a range of useful services and contacts. 

• Provides emotional support to patients, families and friends of GBS and CIDP.
• Provides visits by former patients to those currently suffering to offer encouragement and support.  I am happy to visit people in hospital, I am located in Melbourne Australia.  Please contact me by e-mail.  There can be nothing more helpful to a patient's morale than to receive a visit from someone who has made his or her own recovery from the illness. I am available to visit hospitals within Australia and I provide information kits for parents/friends/relatives etc.
• Supplies literature about the illness and newsletters to members.
• Endeavours to educate the public and medical community about the Support Group and maintains their awareness of the illness.
• Encourages local branches nationwide.
• To support research into the cause & treatment of GBS & CIDP.
• To continue fund raising activity to support the groups continued existence.
• Improved counselling and support facilities for patients and their families.

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