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Guillain-Barre Syndrome
Sunday, 08 August 2004
Monday, 29 November 2004

What

Guillain-Barre Syndrome (GBS) is a disorder caused by inflammation and damage to the peripheral nervous system. The peripheral nervous system is made up of nerves that are found outside of the brain and spinal cord. These nerves convey sensory information (pain and temperature) from the body to the brain and motor (movement) signals from the brain to the body. Damage to the peripheral nervous system is what causes the severe weakness, numbness and paralysis that are characteristic of Guillain-Barre Syndrome.

Who

Guillain-Barre Syndrome is rare, affecting only about one out of every 100,000 people. GBS can occur in children from all ethnic backgrounds. This disorder seems to occur more often in males than females and the reason for this is not clear. While children are at lower risk than adults, children younger than 4 years of age may be at slightly higher risk than children of other ages.

Signs and Symptoms

Children with Guillain-Barre may have the following symptoms, many of which are sudden and unexpected:

  • Progressive ascending weakness-this is the hallmark of GBS. Weakness typically starts in the legs and ascends upward to the arms.
  • Absence of reflexes (areflexia)-another hallmark of GBS
  • Pain
  • Tingling; “pins and needles” sensation in the feet
  • Increased heart rate
  • Dizziness due to orthostatic hypotension (a form of low blood pressure that occurs when standing)
  • Unsteadiness
  • Incoordination
  • Dysfunction of the pupils
  • Abnormal sweating
  • Weakness in the muscles that control the face, eye movement, speaking, chewing, and swallowing
  • Severe breathing problems

Possible Causes

The exact cause of Guillain-Barre Syndrome is unknown. It is not inherited or contagious. It is thought to be an autoimmune disorder, which means the body’s immune system begins to attack the body itself. Normally, the cells of the immune system attack only foreign material and invading organisms, but in GBS the immune system’s cells attack parts of the peripheral nerves. Specifically, the immune system starts to destroy the myelin sheath (a fatty substance that surrounds the axons of the peripheral nerves) or even the axons themselves (axons are the long extensions of the nerve cells that carry the nerves signals). The myelin sheath acts as an insulator around the axon, speeding up the transmission of nerve signals and allowing the signals to travel over long distances. Because the myelin sheaths around the peripheral nerves are damaged, the nerves cannot transmit the signals effectively. This is why the muscles begin to lose their ability to respond to the brain's commands and the brain receives fewer sensory signals and inappropriate sensory signals from the rest of the body. The result is muscle weakness, and an inability to feel textures, heat, pain, and tingling sensations. Because the signals to and from the arms and legs must travel the longest distances they are most vulnerable to interruption from the damage to myelin. This is why muscle weakness and tingling sensations usually first appear in the hands and feet and progress upwards. Several infections (respiratory and gastrointestinal) have been known to precede Guillain-Barre Syndrome. Occasionally, surgery or vaccinations can trigger the syndrome.

Diagnosis

A diagnosis of Guillain-Barre Syndrome is typically made based on the presence of a progressive, ascending weakness with absent reflexes (areflexia). The following tests can confirm the diagnosis: 1. Lumbar puncture (spinal tap) to collect a sample of cerebrospinal fluid (the fluid that surrounds the brain and spinal cord). An elevated level of protein in the fluid is characteristic of Guillain-Barre Syndrome. 2. Electromyogram (EMG) to test muscle activity. This test can show the loss of reflexes due to the disease's characteristic slowing of nerve responses. 3. Nerve conduction study to test the speed at which signals travel along the nerves.

Treatment

There is no known cure for Guillain-Barre Syndrome, but therapies can lessen the severity of the illness and accelerate the recovery in most patients. Currently, two treatments have benefit children affected by Guillain-Barre Syndrome: plasmapheresis, and intravenous immunoglobulin. Plasmapheresis is a method that removes harmful substances from the blood. In intravenous immunoglobulin therapy, high doses of immunoglobulins from healthy donors (immunoglobulins are cells of the immune system that attack harmful, foreign organisms) are given to GBS patients via intravenous injections. This has been shown to lessen the attack on the nervous system. 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.

Prognosis

The prognosis for children with Guillain-Barre Syndrome can be good. In general, children with GBS have a more favorable outcome than adults. Most children recover from even the most severe cases of GBS, although some may continue to have some weakness and long-term impairment. The recovery period can be as short as a few weeks or as long as a few years. Very few children die as a result of this syndrome. The overall mortality rate is about 5%. Death usually results from the heart and respiratory problems associated with the syndrome.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Guillain-Barre Syndrome Foundation International
Great site, contains information and support for GBS, also contains links to discussion forums and chat rooms.

Guillain-Barre Syndrome Association
Foreign based association. Website has great information on GBS and support group information.

Google Search for Guillain-Barre Syndrome

References and Sources

1. Mack, K. (2001) Guillain-Barre Syndrome in Childhood http://www.emedicine.com/neuro/topic598.htm 2. Fanion, D. (2001) Guillain-Barre Syndrome http://www.emedicine.com/EMERG/topic222.htm 3. Andreoli et.al. (2001) Cecil Essentials of Medicine 5th Ed., Saunders, p.551-559