Also called: Acute Inflammatory Polyneuropathy, GBS, Infectious Polyneuritis, Landry’s Ascending Paralysis, Acute Idiopathic Polyneuritis
Guillain-Barré syndrome (GBS) is a rare neuromuscular condition in which the body’s immune system attacks the nerves of the peripheral nervous system – nerves outside the brain and spinal cord. GBS can cause sensory abnormalities such as tingling in the hands and feet, loss of body reflexes, loss of motor functioning, paralysis and even death.
Scientists are not yet sure what causes GBS. More than half of all patients with GBS reported having a recent bacterial or viral infection. Recent studies also have suggested a link between the bacterium Campylobacter and GBS. In rare cases, GBS has been reported in people who recently have received certain vaccines. However, this link has not been clearly established. Other medical conditions or situations (e.g., recent surgery) have also been associated with GBS.
The first symptoms associated with GBS often include general weakness and tingling in the legs. This may quickly progress to a loss of reflexes and temporary paralysis that usually begins in the feet and legs and ascends to the arms and hands. If muscle weakness or paralysis affects the breathing muscles and heart, life-threatening complications may develop. In very rare cases, patients may remain permanently paralyzed.
Most patients with GBS recover with little or no loss of mobility. Some patients may take as long as a year to recover. Persistent weakness may linger for more than three years in about 30 percent of patients, according to the National Institutes of Health. Some patients with GBS may experience a relapse.
Symptom severity and duration can vary significantly from person to person. The signs and symptoms of GBS are similar to those of other neurological disorders and may make an accurate diagnosis difficult.
Although there is no way to definitively diagnose GBS, three different tests are often used to help make a GBS diagnosis. These include electromyography (measures the electrical activity of a muscle in response to nerve stimulation), nerve conduction velocity tests (measures the speed of signals along the nerve) and a spinal tap (determines the pressure of cerebrospinal fluid and helps reveal other signs of illness).
There are no known preventive methods for GBS. Sometimes GBS may be cured with intravenous immunoglobulin (IVIG) or plasmapheresis. However, most treatment is focused on alleviating symptoms and reducing the duration of the illness. Many patients diagnosed with GBS are hospitalized and their conditions closely monitored because the progression of the disease can become suddenly and unexpectedly severe.
About Guillain-Barré syndrome
Guillain-Barré syndrome (GBS) is a rare neuromuscular condition in which nerve damage inhibits muscle movement. This loss of mobility is usually temporary, although its severity and duration can vary greatly from person to person. In a small number of cases, permanent paralysis or death may result.
GBS is considered an autoimmune disorder, where the body’s immune system attacks its own cells, causing tissue damage. In patients with GBS, this damage involves the nerves of the peripheral nervous system (those outside the brain and spinal cord).
GBS affects the protective coating of nerves, the myelin sheath, and the nerve extensions called axons. The myelin sheath helps protect the nerve and speed transmission of signals between the brain and muscles. This aids motor functioning and physical coordination. The axons help conduct electrical impulses to other nerves, muscles and cells throughout the body.
GBS causes inflammation that can damage the nerves. Portions of the myelin sheath may be stripped away, reducing their ability to insulate and protect the axons. Myelin sheath damage of peripheral nerves can slow or prevent the nerves from transmitting signals between the brain and muscles. This causes the muscles to respond inefficiently, if at all. Likewise, the brain receives fewer signals from the nerves, resulting in the inability to feel certain sensations, or the experience of abnormal sensation. Because signals to the arms and legs must travel the farthest, they are more susceptible to interruption when nerve damage occurs. Damage to the axons can cause the nerves to stop working altogether and result in paralysis.
Nerve damage can cause a wide variety of symptoms such as paresthesia (burning or tingling in the hands or feet), numbness, weakness, paralysis that are common in patients with GBS. Symptoms usually begin in the lower extremities (e.g., legs) before spreading to the upper extremities (e.g., arms).
GBS affects only about one or two people out of every 100,000. The illness is known as a syndrome rather than a disease because scientists do not know whether or not a disease-causing agent is involved in the disorder. Thus, GBS is a medical condition characterized by a collection of certain signs and symptoms.
Complications of GBS may include lingering muscle weakness or long-term or permanent paralysis. If muscle weakness or paralysis associated with GBS spreads to the breathing muscles, a patient may experience respiratory failure. Significant damage may occur to the muscles and nerves, which can weaken the lungs and heart. This can result in a dangerous slowing of the heart rate and lowering of blood pressure.
GBS is fatal in about 5 percent of cases, according to the U.S. Centers for Disease Control and Prevention. Causes of death related to GBS may include respiratory failure, pulmonary embolism or cardiac arrest. Older adults are at the greatest risk of fatality. About 3 percent of patients who survive and recover from GBS may be prone to future relapse of the illness, according to the National Institutes of Health.
Types/differences of Guillain-Barré syndrome
Guillain-Barré syndrome (GBS) can take several different forms, including:
- Acute inflammatory demyelinating polyneuropathy (AIDP). Condition that damages the myelin sheath, the fatty protective covering surrounding the nerves’ axons. Motor and sensory nerves of the peripheral nervous system may be affected, slowing transmission of signals to and from the brain. Symptoms include muscle weakness and paralysis that begins in the legs and feet and moves up to the arms and hands. The vast majority of cases of GBS in the United States are of this type. Often, when people speak of GBS, they are referring to AIDP.
- Miller Fisher syndrome (MFS). MFS affects facial and cranial nerves and includes a distinctive triad of ataxia (loss of coordination), eye muscle paralysis (ophthalmoplegia), and the absence of body reflexes (e.g., knee-jerk). MFS may also include generalized weakness as well as difficulty speaking or swallowing. Unlike other forms of GBS, weakness associated with MFS typically begins in the upper body and descends to the lower body.
- Acute motor axonal neuropathy (AMAN). AMAN usually involves damage to the nerve axons, especially those affecting movement. Symptoms are similar but more severe than those of AIDP and recovery can take much longer. For example, patients with AMAN are more likely to experience weakness or paralysis of breathing muscles, resulting in respiratory failure. Outbreaks of this type of GBS have occurred in northern China.
- Acute motor-sensory axonal neuropathy (AMSAN). This type of GBS is similar to AMAN, except that this type also includes sensory symptoms, such as loss of reflexes and burning or tingling sensations (paresthesia). The axons of both motor and sensory nerves may be damaged. This type of GBS may last longer than AMAN.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is similar to AIDP, although CIDP is not considered a type of GBS. Unlike AIDP, which is characterized by an acute (sudden and severe) onset of symptoms, CIDP develops slowly and lasts longer.
Risk factors and causes of Guillain-Barré syndrome
Scientists are not yet sure what causes Guillain-Barré syndrome (GBS).
In more than half of all cases of GBS, minor respiratory or gastrointestinal illnesses precede GBS. Some experts believe that when these illnesses activate the immune system to defend the body, the immune system mistakenly attacks healthy nerve cells as well. This may occur when certain viruses or bacteria change the cell structure of healthy nerves in the body, so the immune system is unable to properly recognize them and treats them as if they were foreign cells. Certain pathogens may also somehow affect the immune system itself so that it is unable to recognize certain types of cells as its own.
Conditions and situations associated with the onset of GBS include:
- Bacterial infections. A variety of different bacterial infections (including Lyme disease and Hib infection) have been associated with the onset of GBS. Infection with the bacterium Campylobacter is the most commonly identified illness preceding onset of GBS. Campylobacter is often found in undercooked food, especially poultry.
- Viral infections. A wide variety of viral illnesses are associated with the onset of GBS. These include colds, the flu, mononucleosis, HIV, cytomegalovirus infections, and herpes simplex viral infections (the cause of cold sores).
- Vaccinations. In rare cases, GBS has been reported in people who recently have received certain vaccines (e.g., influenza, meningococcus, swine flu). However, this link is not clear and experts generally believe that people who receive vaccinations are at little or no added risk of contracting GBS.
- Recent surgery or childbirth. These have also been associated with GBS. GBS may also occur with certain systemic medical conditions such as lupus and Hodgkin’s disease.
GBS can strike people at any age, but is more likely to occur among teens and young adults (15 to 35) or among older adults (50 to 75). It occurs in both genders, although just slightly more frequently in men. GBS does not usually occur in young children under the age of two years. This may indicate that some maturation of the immune system is required before GBS can occur.
Diagnosis methods for Guillain-Barré syndrome
Currently, there is no test that can definitively diagnose Guillain-Barré syndrome (GBS). Patients who suspect they may have GBS or another neurological condition may be referred to a neurologist for diagnosis and treatment.
A physician who suspects GBS will likely begin by compiling a medical history of the patient. The physician may ask questions such as whether the patient has recently experienced any type of respiratory or gastrointestinal illness. The patient’s symptoms will be discussed, including whether certain symptoms (e.g., tingling sensations, weakness, paralysis) are affecting both sides of the body, whether symptoms are worsening or spreading to other areas of the body and how quickly this is occurring. If a fever is present, it may indicate another medical condition instead of GBS.
A physician will also perform a complete physical examination of the patient. This examination may include a blood pressure reading and heart rate check. A knee-jerk reflex test may be performed to look for diminishment or absence of this reflex, which may indicate nerve conduction problems and GBS.
In previous years, the muscle weakness and inability to move of GBS had to be distinguished from polio, another condition that affects the nervous system and may cause paralysis. However, polio has been essentially eradicated in developed countries, making GBS the most common condition with flaccid paralysis of the muscles.
In addition, signs and symptoms of GBS may vary considerably from person to person. This makes an accurate diagnosis more difficult.
Diagnostic tests that may be used when GBS is suspected include:
- Electromyography (EMG). Measures the electrical activity of a muscle in response to nerve stimulation. This test involves inserting fine needles that contain electrodes into muscles and placing electrodes on the skin over peripheral nerves.
Nerve conduction velocity (NCV) test. Typically performed along with an EMG. This test measures the speed of signals that travel through the nerves.
- Spinal tap (lumbar puncture). Determines the pressure of cerebrospinal fluid (CSF), a clear fluid that bathes and protects the brain and spinal cord. A needle is inserted into the spinal canal and a sample of fluid is removed for laboratory analysis. Spinal taps can help find evidence of bleeding, determine the number and types of white blood cells (WBC) that are present, determine levels of glucose and levels and types of protein, and test for bacteria and fungi. Elevated levels of protein in the CSF are associated with GBS, although this may not be detected during the first week of illness. Other diseases may also cause an elevated rise in CSF protein levels.
If Miller Fisher syndrome (a form of GBS) is suspected, blood tests may be performed to detect the presence of certain antibodies that are typically present in patients with MFS. A nerve biopsy may be required to identify a similar but different condition called chronic inflammatory demyelinating polyneuropathy.
Blood tests and imaging tests may also be used to rule out other possible causes of the patient’s symptoms. Urine tests may help rule out heavy metal poisoning as the cause of symptoms.
Treatment and prevention
There is no prevention method for Guillain-Barré syndrome (GBS), because it is unclear exactly how the condition is caused. When GBS occurs, the goal of treatment is to control symptoms.
Early treatment of GBS is crucial to relieving a patient’s symptoms and accelerating recovery. Both treatments have been found to be equally effective, and there is no reported benefit to using both. They are:
- Blood plasma exchange (plasmapheresis). This blood-cleansing process helps to remove damaging antibodies from the bloodstream. It may be recommended for patients with more severe symptoms of GBS such as the inability to walk without help or requiring mechanical assistance to breathe.
- Intravenous immune globulin (IVIG). This provides the patient with a high-dose infusion of healthy antibodies that can block off damaging antibodies in the patient’s bloodstream. IVIG is easier to administer than a blood plasma exchange and often encouraged for patients with milder symptoms (e.g., able to walk, no longer worsening).
Treatment of GBS typically focuses on reducing inflammation of the peripheral nerves. A physician may suggest over-the-counter (OTC) or prescription pain relief and anti-inflammatory medications to reduce pain and inflammation associated with GBS. Medications that suppress immune system activity may also be prescribed. Corticosteroids were previously used to help treat GBS, but are no longer recommended because they may actually worsen a patient’s condition. Patients should not take any medication, whether OTC or prescribed, without first consulting their physician.
Because it is difficult to tell whether a patient with minor symptoms of GBS may rapidly progress into life-threatening symptoms, many patients diagnosed with GBS are hospitalized and their conditions carefully monitored. Hospitalized patients may require the assistance of a respirator to breathe normally, a feeding tube may be used to prevent choking during meals, and a heart monitor to track the patient’s condition. Anticoagulant medications may be prescribed to prevent blood clots.
About half of all patients with GBS will return to normal health within a year of being diagnosed, according to the American Association of Neuromuscular & Electrodiagnostic Medicine. After release from the hospital, some patients may require short-term supportive care for daily tasks such as eating and using the bathroom.
Other patients may require long-term rehabilitative therapy to help recover the loss of motor function. This may include physical therapy to help regain muscle coordination and strength. Hydrotherapy can help retrain the muscles of affected limbs and may help reduce pain. In addition, certain medical devices (e.g., wheelchair, braces, crutches, walkers) may be needed.
Counseling may help patients and their loved ones deal with the restricted physical abilities or paralysis that can occur with GBS. Lingering problems associated with GBS may also be treated with certain types of medications (e.g., antidepressants, anticonvulsants).
A few cases of GBS have been reported after receiving vaccinations. Patients who developed GBS after a vaccination may be advised to avoid future vaccination if possible. Patients are advised to discuss any concerns they may have with their physician.
Questions for your doctorPreparing questions in advance can help patients have more meaningful discussions with healthcare professionals regarding their condition. Patients may wish to ask their doctor the following questions related to Guillain-Barré syndrome (GBS):
How will I know if my symptoms are associated with GBS?
What tests will you perform to diagnose GBS?
What do you think caused my GBS?
What are my treatment options and their risks?
Will I need to be hospitalized?
What change in my symptoms will require that I seek emergency medical attention? What changes do you want me to report to you?
How long will my symptoms last?
Is there anything I can do to reduce the severity of possible residual effects of GBS?
What is my long-term prognosis?
Can I develop GBS again?
Are there certain vaccinations I should avoid?