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Guillain-Barré Syndrome

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This entry was not written by a medical professional, and as such should not be taken as an authoritative statement of fact. One of the Researchers, however, is a long term sufferer of the illness.

A Brief History

The first (more or less) accurate description of Guillain-Barré Syndrome was made in 1859 by a man called Jean Baptiste Octave Landry de Thezillat. By 1876, the phrase 'Landry's ascending paralysis' became the standard name for the disease.

Georges Charles Guillain and Jean-Alexandre Barré, together with Andre Strohl, worked on the disease throughout the First World War. In 1916, the three published a classic paper on the illness.

In 1927, the paper was used by two doctors who coined the term 'Guillain-Barré Syndrome'. They failed to acknowledge Strohl's contribution to the research, and his name was not listed as an author on the 1916 paper.

Today, there are several recognized forms of Guillain-Barré Syndrome, though most medical professionals take Guillain-Barré Syndrome to mean AIDP, as does this entry.

Depending on the country, the incidence of GBS ranges from 1 in every 25,000 to 1 in every 170,000 people.

It's most common in middle-aged and older people, but has been observed to occur at every age, from infancy on. It's also somewhat more common in men than in women, although the ratio varies by country and by age. (The ratio can vary from 2:1 to 1.1:1).

Terminology

  • GBS - Guillain-Barré syndrome (this is a blanket term for the illness, which has various forms).

  • AIDP - Acute ideopathic demyelinating poly(radiculo)neuropathy (the acute form of Guillain-Barré syndrome).

  • Myelin - The protein sheath that insulates nerves, much like the coating on electrical wiring.

  • Autoimmune disorder - A condition where the immune system is mistakenly triggered into attacking the body's own cells, as if they were invaders like bacteria. Common autoimmune disorders include lupus, multiple sclerosis, and type I diabetes

  • Neuropathic pain - this is the result of an injury or malfunction in the peripheral or central nervous system. Neuropathic pain is often caused by an injury, but this injury may not involve actual damage to the nervous system. Nerves can be compressed by tumours, strangulated by scar tissue, or inflamed by infection.

Causes

GBS is caused by an immune system response to a trigger. Most cases are triggered by an infection; Campylobacter (a common cause of food poisoning) is one of the biggest culprits. Certain hepatitis virii and chlamydia are also known to trigger GBS. Certain vaccinations can also trigger GBS, such as the 'swine-flu' vaccinations of 1976 which left over 500 people paralysed with GBS.

It is worth noting that, because different people have different immune systems, one person may be pre-disposed to developing GBS, whereas another may not.

The Illness

GBS is an autoimmune disorder. It occurs when the immune system accidentally attacks the nerves in the lower back, stripping them of their protective myelin sheath. This causes these long nerves to 'short circuit' and cause changes in the sensation and function of the body.

The first- and often worst-affected parts are the extremities. Toes and fingers are normally the first affected parts of the body. The original name, 'Landry's ascending paralysis', was for good reason. The arms and legs can progressively fail to work and the weakness can spread into the chest. Breathing can also be affected by weakening the muscles of the chest wall and diaphragm; in these cases, mechanical ventilation is required immediately. Those with breathing difficulties have the worst prognosis, and overall, as many as 7-8% of sufferers may die.

The autonomic nervous system which controls, among other things, the heart, doesn't seem to be affected, as the major organs keep functioning.

Symptoms

Symptoms may not appear immediately after the triggering infection: weeks may pass before the first signs of GBS appear.

The first thing a person may notice is tingling or numbness in the fingers and toes. It may become difficult to hold a pen or cutlery, then function changes: hands and feet can stop working entirely. Facial muscles can twitch uncomfortably and fail to work.

Once the symptoms first begin to appear, the time taken for them to peak can vary, usually this happens within four weeks. Some patients reach the point of maximum weakness within a few days, but this is uncommon.

As the disease peaks, the patient progressively loses function to some greater or lesser extent. GBS is often painful. Some of the pain is neuropathic, some may be caused by stiff joints due to immobility. At worst, the patient can be totally paralysed and experience breathing difficulties. Some 30% of cases require ventilation.

A 'plateau' stage is then reached, during which time the disease neither improves nor worsens. This again can last for days or weeks, depending on the individual case. The patient is mostly left to rest at this time, except perhaps for having a physiotherapist move all their joints through a full range to prevent them stiffening up.

Diagnosis

History and Symptoms

GBS is diagnosed mainly by looking at the symptoms and clinical history. Joint reflexes may be absent, and GBS may follow a viral infection, for example. In this way, other conditions, with which it may be confused, can be ruled out.

A good indicator of how well the patient may recover is the time taken for the symptoms to peak.

A lumbar puncture is often performed, where a needle is inserted between the bones of the lower spine and fluid is drawn off for analysis.

An electromyogram, or EMG, is used to ascertain nerve malfunction: it is a test of nerve conductivity, performed by running electricity through the patient and measuring the output at certain points.

Treatment and Recovery

Symptomatic Treatments

Neurontin/gabapentin or carbamazepine are often used to relieve neuropathic pain and discomfort, which can cause untold suffering to the patient and hamper physiotherapy.

Systemic Treatments

  • Immunoglobulin is a human blood product which 'soaks up' the harmful antibodies and helps bring their rampage through the body to a swifter close.

  • Plasma Exchange - the patient is connected to a machine which separates the plasma from their blood, since the antibodies are contained in this plasma, they are discarded. 'Clean' plasma is added to the remaining blood cells and this is returned to the patient. Not all hospitals have the equipment available for this, however, so immunoglobulin is often favoured.

  • Corticosteroids - some hospitals still use corticosteroids for GBS, in a vain attempt to stop the inflammatory process of the autoimmune reaction itself, even though it has been shown repeatedly not to work, and may actually be counter-productive.

  • Physiotherapy is a vital tool in nursing a sufferer back to full health while attempting to prevent too much loss of muscle mass through inactivity. It is the main treatment, and the most effective. The patient will be exercised daily in many cases, often in a rehabilitation centre or specialist ward. This can be a long and difficult journey for the patient, who will need much emotional support from family and friends.

Recovery

Most sufferers make a complete recovery, though some are left with a permanent weakness. Some varieties can exhibit recurring symptoms. Recovery time varies wildly and is difficult to guess. It can be as little as a fortnight, or take many years.

After recovering from GBS, an increased risk of recurrence exists. Some estimates put this at around 3%.

One follow-up study found that while most people with GBS recovered quite well, about 21% had to go on disability or take early retirement because they were no longer able to do their previous jobs. Many of these people, however, had had physically demanding jobs.

Further Information

There is much information that is not in the scope of this guide, for example, the rarer variants of Guillain-Barré Syndrome. For more information, visit the Guillain-Barré Syndrome Support Group UK, and the Guillain-Barré Syndrome International Foundation.


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