Pediatric Diseases and ConditionsGuillain-Barre Syndrome in Children
Inflammatory and Infectious Neurological Disorders
Guillain-Barré syndrome (GBS) is a neurological disorder in which the body's immune system attacks part of the peripheral nervous system. The onset can be quite sudden and unexpected. According to the National Institute of Neurological Disorders and Stroke (NINDS), the disorder can develop over a few days, or it may take up to several weeks. A person experiences the greatest weakness within the first two weeks after symptoms appear. By the third week, 90 percent of all patients are at the weakest point in their illness.
Although rare, the NINDS says that GBS afflicts about one person in 100,000. The syndrome can affect people at any age and both men and women equally. The disorder usually occurs a few days or weeks after a person has had symptoms of a respiratory or gastrointestinal viral infection.
Currently, it is not known why Guillain-Barré strikes some people. What is known is that 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 Guillain-Barré syndrome, the immune system starts to destroy the myelin sheath that surrounds the axons of many nerve cells, and, sometimes, the axons themselves.
When this occurs, the nerves cannot send signals efficiently, the muscles lose their ability to respond to the commands of the brain, and the brain receives fewer sensory signals from the rest of the body. The result is an inability to feel heat, pain, and other sensations.
Guillain-Barré syndrome can occur after a viral infection, surgery, trauma, or a reaction to an immunization.
The following are the most common symptoms of Guillain-Barré syndrome. However each individual may experience symptoms differently.
The first symptoms include varying degrees of weakness or tingling sensations in the legs, which, sometimes, spreads to the arms and upper body. The symptoms may increase in severity until the following does or does not occur--in these cases, the disorder is considered a medical emergency:
Muscles cannot be used at all
The patient becomes nearly paralyzed
It may become difficult to breathe
Blood pressure and heart rate are affected
Although symptoms can become life-threatening, partial recovery is possible from even the most severe cases of Guillain-Barré syndrome. However, some degree of weakness may still be present.
The symptoms of Guillain-Barré syndrome may resemble other medical conditions or problems. Always consult your doctor for a diagnosis.
According to the NINDS:
GBS is called a syndrome rather than a disease because it is not clear that a specific disease-causing agent is involved. A syndrome is a medical condition marked by a collection of symptoms, or what the patient feels, and signs, or what a doctor can observe or measure. The signs and symptoms of the syndrome can be quite varied, so doctors may, on rare occasions, find it difficult to diagnose GBS in its earliest stages.
Signs and symptoms that may differentiate Guillain-Barré from other disorders include the following. With Guillain-Barré:
Symptoms appear on both sides of the body.
Symptoms appear quickly--days or weeks as opposed to months.
Reflexes are usually lost.
Cerebral spinal fluid that bathes the spinal cord and brain contains more protein than usual.
Other diagnostic procedures include the following:
Spinal tap (also called a lumbar puncture). A special needle is placed into the lower back, into the spinal canal. This is the area around the spinal cord. The pressure in the spinal canal and brain can then be measured. A small amount of cerebral spinal fluid (CSF) can be removed and sent for testing to determine if there is an infection or other problems. CSF is the fluid that bathes the brain and spinal cord.
Electrodiagnostic tests, such as electromyography (EMG) and nerve conduction velocity (NCV). Studies that evaluate and diagnose disorders of the muscles and motor neurons. Electrodes are inserted into the muscle, or placed on the skin overlying a muscle or muscle group, and electrical activity and muscle response are recorded.
Specific treatment for Guillain-Barré syndrome will be determined by your doctor based on:
Your age, overall health, and medical history
Extent of the syndrome
Your tolerance for specific medications, procedures, or therapies
Expectations for the course of the syndrome
Your opinion or preference
Currently, there is no known cure for Guillain-Barré syndrome. The goal of treatment is to prevent breathing problems and provide supportive care (relief of symptoms). Medications are used to control pain and other conditions that may be present. In addition, treatments, such as plasmapheresis or immunoglobulin administration, may be used to suppress the immune system and/or reduce inflammation caused by the immune system’s response to the disease.
Plasmapheresis is a procedure that removes the plasma (liquid part of the blood) and replaces it with other fluids. Antibodies are also removed with the plasma, which is thought to help reduce the symptoms of the disease. Another treatment is the administration of immunoglobulin, a blood product that helps to decrease the immune system’s attack on the nervous system.
Other therapies include hormonal therapy and physical therapy (to increase muscle flexibility and strength).
Through research, new treatments for Guillain-Barré syndrome are continually being identified.