What Is Guillain-Barre Syndrome? What Causes Guillain-Barre Syndrome?

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Main Category: Neurology / Neuroscience
Article Date: 20 Oct 2009 - 0:00 PDT

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Guillain-Barre syndrome is a rare but serious autoimmune disorder that affects the peripheral nervous system. It is usually caused by an acute infectious process. The peripheral nervous system is the portion of the nervous system that is outside the brain and spinal cord. The word Barre may also be spelled Barré (accent on the "e").

Autoimmune means the patient's immune system goes wrong and attacks good tissue, which in this case are nerve cells which communicate with muscles. The individual with Guillain-Barre syndrome will initially experience a tingly and numbing sensation in the limbs, as well as weakness in the same areas. If these sensations spread, which they often do, the entire body eventually becomes paralyzed. Typically, symptoms start in the lower limbs and eventually spread upwards.

In 1859, the disorder was first described by the French physician Jean Landry. In 1916, Georges Guillain, Jean Alexandre Barre, and Andre Strohl diagnosed two soldiers with the illness. They discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.

Recent reports reveal that U.S. President, Franklin D. Roosevelt was not paralyzed because of polio - it was caused by Guillain-Barre syndrome. Other well known people who developed Guillain-Barre syndrome include Andy Griffith (Matlock, the Andy Griffith Show), Tachel Chagall (actress), Markus Babbel (international footballer), Hans Vonk (Dutch music conductor), Tony Benn (UK politician), Len Pasquerelli (ESPN sports writer), Hiroko Mima (Miss Universe Japan 2008, diagnosed at 13 years of age), Saituni Onyango (paternal aunt of President Obama).

According to Medilexicon's medical dictionary:

Guillain-Barre syndrome is: an acute, immune-mediated disorder of peripheral nerves, spinal roots, and cranial nerves, commonly presenting as a rapidly progressive, areflexive, relatively symmetric ascending weakness of the limb, truncal, respiratory, pharyngeal, and facial musculature, with variable sensory and autonomic dysfunction..

We don't know exactly what causes Guillain-Barre syndrome, but experts say it is preceded by an acute infectious process in a significant number of cases.

According to the Centers for Disease Control and Prevention (CDC), USA, approximately 1 to 2 individuals per 100,000 are affected by the syndrome. The UK National Health Service (NHS) reports that about 1,500 British people are affected annually, out of a population of 61 million. It is a little more common in men than women and can affect humans of all ages.

In a significant number of cases, patients make a full recovery and have no symptoms later on.

What are the signs and symptoms of Guillain-Barre syndrome?

A symptom is something the patient feels or reports, while a sign is something other people, including the doctor detects. For example, a headache may be a symptom while a rash may be a sign.

In most cases symptoms develop rapidly within a question of days. More rarely the progress of symptoms may take up to a month.

Typically, signs and symptoms emerge a short time after a sore throat or some minor infectious process.

First symptoms - symptoms usually start in the feet and hands and may eventually make their way further along the limbs. There is more commonly symmetrical weakness in the lower limbs initially, which rapidly progress in an ascending fashion. Initial signs and symptoms may include:

• Instability - unsteadiness. Patients often complain of rubbery legs
• D ysesthesias - numbness or tingling
• Muscle weakness, which gets progressively worse
• Stinging
• Weakness or tingling sensations in legs that spread to upper body

Possible later symptoms - In some people, the muscle weakness progresses to:

• Bladder control problems - usually transient (brief). If bladder control problems are severe, the doctor may suspect a spinal cord disorder.
• Digestive system problems
• Oropharyngeal dysphagia - drooling, or difficulty swallowing and/or maintaining an open airway
• Difficulty with eye movement and speech
• Facial weakness
• Slow heart rate or low blood pressure
• Temporary paralysis of the legs, arms and face
• Respiratory problems - some patients may require ventilatory assistance.
• Severe pain in lower back
• Pain - a common symptom. Pain is usually a deep aching one, located in the weakened muscles.
• Sensory loss - less common. If present, it usually takes the form of position sense (proprioception) and complete loss of deep tendon reflexes (areflexia). Any loss of pain or temperature sensation is generally very mild.
• No fever - if there is one, the physician should suspect another cause

Severe paralysis is rare, but may occur, and is thought to be linked to sodium channel blocking factor in the cerebrospinal fluid.

What are the causes of Guillain-Barre syndrome?

Nobody knows precisely what the exact cause is. We know it is due to an immune response to foreign antigens, such as infectious agents (bacteria, etc.) that the body's immune system mistargets - attacks good tissue by mistake. Experts believe our immune system mistakenly attacks gangliosides - compounds which are naturally present in nerve tissues.

The most common infection which precedes the development of Guillain-Barre syndrome is Campylobacter jejuni - one of the most widespread causes of human gastroenteritis. Even so, in over half of all cases no previous infection was present; in other words, there was nothing to which the doctor could link the syndrome.

It is believed the influenza virus may also trigger an autoimmune response which causes the syndrome.

Experts believe the foreign agent (bacterium/virus) causes the body's immune system to attack the myelin sheath of the peripheral nerves. The myelin sheath is a cover that protects the nerve, a bit like the plastic that covers electrical wires. The sheath becomes damaged, causing nerve damage, resulting in faulty sending of signals between nerves and muscles. This faulty wiring causes muscle weakness, numbness and tingling, and eventually paralysis (usually brief).

How is Guillain-Barre syndrome diagnosed?

Most people with symptoms will visit their GP (general practitioner, primary care physician, family doctor) first, who might be able to diagnose Guillain-Barre syndrome after testing the patient's knee-jerk reflex (little hammer on the knee with legs crossed), carrying out a physical examination, and asking the patient some question regarding symptoms.

However, as Guillain-Barre syndrome symptoms overlap those of other nervous system disorders, diagnosis may not be a straightforward process. The following diagnostic tests may be ordered:

• Electromyography (EMG) - this determines how healthy the muscles and the nerves that control them are. A electrode is inserted through the skin and into the muscle to give an electrical recording of muscle activity to see if the muscles respond after certain nerves are stimulated. In Guillain-Barre syndrome, nerve damage may cause the nerves not to respond properly.


• Lumbar puncture (spinal tap). A needle is inserted into the spinal canal (usually in the lower back). This test helps determine the pressure of the spinal fluid inside. A sample of the spinal fluid is taken analysed to determine whether there is any bleeding in the nervous system. Spinal fluid protein levels are checked, as well as any presence of infectious agents (bacteria, etc.).


• Blood test - this may be ordered to determine whether the patient's symptoms might not be caused by something else.

What are the treatment options for Guillain-Barre syndrome?

Patients with Guillain-Barre syndrome will be hospitalized initially (medical emergency). It is important to monitor the individual's respiration carefully. If breathing problems are severe he/she may be placed in an ICU (intensive care unit) and put on a ventilator.

According to the National Health Service, the two main initial treatment options for Guillain-Barre syndrome are intravenous immunoglobulin, which is safer and easier to give, or plasmapheres (plasma exchange).

Intravenous immunoglobulin - concentrated antibodies is injected straight into a vein. The antibodies are extracted from healthy donors.

Plasmapheresis - the aim here is to deplete the body of blood plasma without depleting it of its blood cells. Plasma is the liquid part of blood. In the case of Guillain-Barre syndrome, the aim is to remove the cells that are attacking the nerves.

After the acute phase of the syndrome, the patient may need rehabilitation to regain functions that were lost. Treatment concentrates on improving activities for daily living, such as brushing teeth, washing, getting dressed and performing some other everyday tasks.

What is the prognosis for Guillain-Barre syndrome?

In the vast majority of cases the patient starts to recover about four weeks after the first symptoms appeared. About four-fifths of all patients with the syndrome make a full recovery within a year, a significant number of them within a few months.

In up to 10% of cases there may be some long-term disability - with sensory and motor (movement) problems. The patient may need a wheelchair to get about, there may be long-term breathing difficulties, numbness and tingling.

Written by Christian Nordqvist
Copyright: Medical News Today
Not to be reproduced without permission of Medical News Today

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