Guillain-Barre Syndrome

What is Guillain-Barre syndrome?

Guillain-Barre syndrome is a condition in which the body's immune system damages parts of neurons. Guillain-Barre syndrome usually occurs after an infection or other triggering event. It is believed that the event leads to an abnormal immune response in which the body produces antibodies, specific immune particles that attack neurons or the material lining the neurons.

Symptoms include progressive weakness, numbness or tingling, decreased or loss of reflexes, pain in the arms, leg, or back, double vision, loss of balance, abnormal heart rhythms or blood pressure levels, and difficulty breathing.

Treatment primarily involves intravenous immunoglobulin therapy or plasma exchange therapy, along with monitoring in a hospital or intensive care unit and supportive care for pain and abnormal vital signs.

You should seek immediate medical care at an ER. Nerve damage can potentially impair your ability to control your heart and lungs. You may need to be admitted to the hospital.

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder that is closely related to Guillain-Barré syndrome (GBS). While GBS is typically an acute condition, CIDP is considered its chronic counterpart. CIDP is characterized by progressive weakness and impaired sensory function in the limbs, developing over a period of at least eight weeks. The key differences between CIDP and GBS lie in their progression and duration. GBS symptoms typically peak within four weeks, whereas CIDP symptoms continue to worsen beyond this timeframe. Additionally, CIDP may have a relapsing-remitting course or show steady progression over time. Nerve conduction studies play a crucial role in diagnosing both GBS and CIDP. These tests measure the speed and strength of electrical signals traveling along nerves. In CIDP, nerve conduction studies often reveal evidence of demyelination, such as reduced conduction velocities, prolonged distal latencies, and conduction blocks. These findings may be more pronounced and widespread in CIDP compared to GBS, reflecting the chronic nature of the condition. Proper diagnosis and differentiation between GBS and CIDP are essential, as the treatment approaches and long-term management strategies may differ. While both conditions may initially respond to similar treatments like intravenous immunoglobulin or plasma exchange, CIDP often requires ongoing therapy to prevent relapses and manage symptoms over an extended period.

Guillain-Barre syndrome symptoms

There are a few different variants of Guillain-Barre syndrome, which differ in their disease processes and symptoms. These include acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), and Miller Fisher Syndrome. The diagnosis for this condition is made by clinical evaluation, although electrophysiologic studies and cerebrospinal fluid testing can support the diagnosis.

Main symptoms

Symptoms that can generally be seen in Guillain-Barre syndrome include:

• Muscle weakness: This weakness usually starts in the legs and classically progresses up the body, but in some cases can start in the arms or face. The weakness is usually symmetric on both sides of the body. Overall, muscle weakness and other symptoms of Guillain-Barre syndrome usually worsen over the course of days to weeks. Seventy-three percent of people will reach maximum symptoms by one week and 98 percent will reach maximum symptoms by four weeks; symptoms will then plateau and begin to improve in a few days, although may persist for weeks.
• Numbness or tingling: This usually affects the hands or feet.
• Decreased or loss of reflexes in the arms or legs: This may be noticed when your physician examines you with a reflex hammer.

Other symptoms

Other symptoms that are also likely with this condition include the following.

• Pain in the arms, legs, or back: About one-half of patients with Guillain-Barre syndrome will develop pain in the arms, legs, or back. The pain is usually described as a deep, aching pain and may be accompanied by muscle cramps.
• Double vision and/or loss of balance: This is classically seen in the Miller Fisher Syndrome variant of Guillain-Barre syndrome.
• Abnormal heart rhythms or blood pressure levels: This can occur if the inflammation affects the autonomic nervous system, the part of the nervous system that controls heart rate and blood pressure. This may cause you to feel chest discomfort, lightheadedness, or loss of consciousness.
• Difficulty breathing: This occurs due to weakness in the muscles that are important for breathing. In some cases, the respiratory muscle weakness may be severe enough that you need to be intubated. This occurs in approximately 30 percent of cases.

Guillain-Barre syndrome causes

Guillain-Barre syndrome usually occurs after an infection or other triggering event. It is believed that the event triggers an abnormal immune response in which the body produces antibodies, specific immune particles that attack neurons or the material lining the neurons. The risk of developing Guillain-Barre syndrome increases with increasing age and is slightly more common in men than in women. Specific causes of Guillain-Barre syndrome include infections and vaccination.

Infection

Approximately two-thirds of cases of Guillain-Barre syndrome occur after an infection, and infections are thought to potentially trigger Guillain-Barre syndrome through an immune-mediated process. The preceding infection is usually a respiratory or gastrointestinal infection, and symptoms of Guillain-Barre syndrome usually develop within one month.

• Campylobacter jejuni: This is the most common infection associated with Guillain-Barre syndrome, which a bacterium that causes diarrhea and can be acquired from eating raw meat or contaminated dairy or produce. It is seen in 25 to 50 percent of adult patients.
• Other infections: Other associated infections include HIV, influenza, hepatitis viruses, and Zika virus, among others.

Vaccination

Studies have shown that certain vaccinations, including certain types of influenza vaccines, may be associated with a very slight increased risk of developing Guillain-Barre syndrome, with a risk of approximately one to two cases per 1 million people immunized. Therefore, this risk must be weighed against the much greater risks of developing the disease that the vaccine protects against. A surveillance system administered by the Centers for Disease Control (CDC) and the U.S. Food and Drug Administration (FDA) concluded that the risk of developing vaccine-associated Guillain-Barre syndrome is less than the risk of severe influenza.

Treatment options and prevention

Although Guillain-Barre syndrome will eventually resolve on its own, it often causes serious symptoms such as paralysis and difficulty breathing that need to be treated with supportive therapies in an intensive care unit. In addition, certain treatments have been shown to shorten the duration of symptoms in Guillain-Barre syndrome and are generally recommended to improve outcomes. Therefore, most people diagnosed with Guillain-Barre syndrome are admitted to the hospital for close monitoring and supportive treatment. Specific treatment for Guillain-Barre syndrome includes the following.

Intravenous immunoglobin (IVIG) therapy

This therapy involves administering a collection of antibodies collected from healthy donors. This is thought to help treat Guillain-Barre syndrome by reducing inflammation and blocking the effect of abnormal antibodies.

Plasma exchange therapy

A component of your blood is removed and replaced with donor blood components. This therapy is thought to help by removing antibodies that may be attacking the nerves. This therapy usually involves multiple treatments over eight to 10 days. Studies have shown that plasma exchange therapy is as effective as intravenous immune globin therapy for treating Guillain-Barre syndrome.

When to seek further consultation

If you develop any symptoms of Guillain-Barre syndrome such as weakness, numbness or tingling, pain in the back or extremities, you should go to your physician. If you are experiencing difficulty breathing, you should go to the emergency department or call an ambulance right away.