Guillain-Barre Syndrome (GBS)
Overview
Guillain-Barre syndrome (GBS) is an inflammatory disorder of the peripheral nerves. The peripheral nerves convey sensory information (e.g., pain, temperature) from the body to the brain and motor (i.e., movement) signals from the brain to the body. GBS is characterized by weakness and numbness or tingling in the legs and arms, and possible loss of movement and feeling in the legs, arms, upper body, and face.
Chronic inflammatory demyelinating polyradicalneuropathy (CIDP), is considered to be a related form of Guillain-Barre syndrome. It is much less common than GBS, and evolves much more slowly and usually is longer lasting. Some CIDP patients experience periods of worsening and improvement, and individual relapses are often confused with GBS.
Incidence
Guillain-Barre syndrome is a rare disorder; its frequency is about 1 to 2 cases in every 100,000 people per year in the United States. Men and women, young and old, are equally prone to contracting GBS.
Causes
Guillain-Barre syndrome is not hereditary or contagious. What causes GBS is not known; however, in about half of all cases the onset of the syndrome follows a viral or bacterial infection, such as the following:
- flu, common cold
- gastrointestinal viral infection
- infectious mononucleosis
- viral hepatitis
- campylobacteriosis (usually from eating undercooked poultry)
- porphyria (rare disease of red blood cells)
A small number of cases have been known to occur after a medical procedure, such as minor surgery.
Guillain-Barre syndrome may be an autoimmune disorder in which the body produces antibodies that damage the myelin sheath that surrounds peripheral nerves. The myelin sheath is a fatty substance that surrounds axons. It increases the speed at which signals travel along the nerves.
Signs and Symptoms
The first symptoms of GBS are usually numbness or tingling (paresthesia) in the toes and fingers, with progressive weakness in the arms and legs over the next few days. Some patients experience paresthesia only in their toes and legs; others only experience symptoms on one side of the body.
The symptoms may stay in this phase, causing only mild difficulty in walking, requiring crutches or a walking stick. However, sometimes the illness progresses, leading to complete paralysis of the arms and legs. About one quarter of the time, the paralysis continues up the chest and freezes the breathing muscles, leaving the patient dependent on a ventilator. If the swallowing muscles are also affected, a feeding tube may be needed.
In CIDP, the course of illness is longer and respiratory failure is much more unlikely.
Diagnosis
Because its symptoms vary and its cause is unknown, GBS can be difficult to diagnose. If the symptoms occur uniformly across the body and progress rapidly, the diagnosis is easier.
Observation of the patient's symptoms and an evaluation of the medical history provide the basis for diagnosis of Guillain-Barre syndrome, although no single observation is suitable to make the diagnosis.
Tests
Three tests can confirm a diagnosis of Guillain-Barre syndrome.
Lumbar puncture (spinal tap)-The patient is given local anesthetic. Once the anesthetic has taken effect, a needle is inserted between two lower (lumbar) vertebrae and a sample of cerebrospinal fluid is drawn. An elevated level of protein in the fluid is characteristic of GBS.
Electromyogram (EMG)-This is an effective diagnostic tool because it records muscle activity and can show the loss of reflexes due to the disease's characteristic slowing of nerve responses.
Nerve conduction velocity (NCV)-This test is performed with EMG, and together, they are often referred to as EMG/NCV studies. NCV records the speed at which signals travel along the nerves.
Treatment
GBS is considered a medical emergency and most patients are admitted to intensive care soon after diagnosis. Though GBS can improve spontaneously, there are a number of treatments that facilitate recovery.
Like GBS, CIDP can improve spontaneously. However, recovery may be very slow and the illness can either get progressively better or worse, or can follow a relapsing/remitting course.
Most patients with GBS and CIDP are treated with plasmapheresis or immunoglobulin. Corticosteroids may be used to treat CIDP but are not used to treat GBS, as it worsens rather than improves the condition.
Plasmapheresis
Patients diagnosed early in the course of the disease and those who are acutely ill often respond well to blood plasma exchange (plasmapheresis). In this procedure, blood is withdrawn and passed through a series of filters that separate the different types of blood cells. The blood cells are then suspended in donor or synthetic plasma and returned to the patient's body.
Plasmapheresis is thought to remove the substances that damage myelin. It can shorten the course of GBS, alleviate symptoms, and prevent paralysis.
Immunoglobulin
Large doses of immunoglobin given intravenously can help shorten the duration of symptoms. This treatment is recommended when plasmapheresis is not available, when patients do not respond to it, and when patients are not good candidates.
Medications
Muscle and joint pain can be treated with over-the-counter analgesics such as aspirin. If necessary, stronger pain medication (e.g., acetaminophen with codeine) may be prescribed. Muscle spasms can be controlled with relaxants such as diazepam (Valium®).
In the later stages of rehabilitation, lingering sensation problems can be treated with tricyclic antidepressants or anticonvulsants such as gabapentin (Neurontin®).
Corticosteroids, which often effectively treat the symptoms of autoimmune disorders, actually worsen Guillain-Barre syndrome and should not be used. They are sometimes used to treat CIDP.
Physical therapy
Before recovery begins, caregivers move the patient's arms and legs to prevent stiffness. After symptoms subside, the rehabilitation team will prescribe an active exercise routine to help regain muscle strength and independence. Training with adaptive devices, such as a wheelchair or braces, give the patient mobility.
Hydrotherapy
Whirlpool therapy (hydrotherapy) may help relieve pain and be useful in retraining the movement of affected limbs.
Counseling
Counseling often is suggested to reassure patients diagnosed with GBS or CIDP and to help them feel positive about their treatment and recovery.
Prognosis
Patients may remain in the hospital for several months and recovery may take a year or more. Most patients recover completely, but some have residual weakness, numbness, and occasional pain. A small number are unable to resume their normal occupation.
Fewer than 5% of GBS patients die. Those fatalities usually result from cardiovascular or respiratory complications. Death resulting from CIDP is rare.