What is Guillain-Barre Syndrome?
Guillain-Barré syndrome, or GBS, is the leading cause of sudden muscle weakness or paralysis in the U.S. This illness was identified over 100 years ago. In that time, medical scientists have learned a lot about the disease, including how it affects the body, how it appears in patients, how to diagnose it, predict its course, and how to treat it to improve patient outcomes. Since GBS can lead to severe health problems if not treated, it’s important for all doctors to know about this relatively rare condition.
What Causes Guillain-Barre Syndrome?
Guillain-Barré syndrome (GBS) and similar conditions are believed to be caused by infections that trigger the immune system to also affect the body’s nerves. This happens because certain infections can trick the immune system. An example of this is an infection caused by a bacteria called Campylobacter jejuni. This bacteria has a molecule on its outer layer that looks a lot like another molecule found in our nerves. When the immune system fights off the bacteria, it mistakingly might also attack the nerves because they look similar.
Many different kinds of infections have been associated with GBS. The most typical ones are either stomach or respiratory infections. Interestingly, up to 70% of people with GBS have experienced an illness 1 to 6 weeks before the first symptoms of GBS were noticed. During outbreaks of the Zika virus, many cases of GBS were reported. Other instances suggest that certain medications and even surgeries may also trigger GBS.
Also, it’s been observed that flu vaccines can increase the chances of GBS. In 1976, a flu vaccine targeting a specific type of flu virus, known as A/H1N1, was linked to an increase in GBS cases. However, later studies show that the risk of this happening is extremely low: about one in a million. Additionally, it’s important to know that getting the flu itself can increase the chances of developing GBS seven times more than getting the flu vaccine.
Risk Factors and Frequency for Guillain-Barre Syndrome
Guillain-Barré syndrome (GBS), although not very common, significantly impacts the healthcare system. The number of cases can range from 0.4 to 2 for every 100,000 people. The medical care cost for a single GBS patient can go up to $318,966. In total, treating all GBS patients can cost around $1.7 billion dollars each year. Men are slightly more likely to get GBS than women. Every year, about 100,000 patients worldwide are estimated to contract GBS.
- Though uncommon, Guillain-Barré syndrome (GBS) poses a significant burden on the healthcare system.
- The number of cases fluctuates between 0.4 to 2 out of every 100,000 people.
- Medical care for one GBS patient can cost as much as $318,966.
- The yearly cost to treat GBS patients is approximately $1.7 billion.
- Men are slightly more likely to get GBS than women.
- About 100,000 people around the world are estimated to get GBS each year.
Signs and Symptoms of Guillain-Barre Syndrome
Guillain-Barré syndrome, often called GBS, is a condition where people typically experience a rapidly worsening set of symptoms. The main signs include muscle weakness that tends to rise up the body and abnormal sensations not linked to the length of nerves involved. Symmetric involvement, affecting both sides of the body, is a common feature. Ordinarily, the worst point of the disease is reached within four weeks, and GBS usually follows a single-episode course, with repeat episodes being quite rare. If the condition worsens beyond eight weeks, other causes should be considered.
Many GBS patients (up to 70%) experience the onset of the disease 1 to 6 weeks after having an illness. Certain vaccines, surgeries, traumas, or other infections can precede and be linked to GBS.
Commonly, people with GBS suffer from both upper and lower body weakness that can be severe if they need hospital treatment. Significant neck weakness might predict the need for a breathing tube. Usually, they have absent or decreased reflexes. Unlike common chronic nerve diseases like diabetic neuropathy, GBS patients usually report abnormal sensations in the hands before the feet. They may experience weakness in both sides of the face or trouble swallowing due to involvement of the nerves controlling these functions. Disruptions to autonomic nerves can lead to serious illness, including irregular heartbeat or unstable blood pressure. That’s why doctors often advise that these patients be monitored in a specialized care unit. In certain cases, when the nerves controlling respiration are impacted, patients may need machine-assisting breathing. Up to 30% of patients can develop breathing failure, leading to long hospital stays and recovery.
Apart from this typical presentation of GBS, there are several variations. Some versions predominantly affect motor functions, such as the “acute motor axonal neuropathy” variant that’s frequently seen in Asian countries. Other variants predominantly affect specific regions, like the “pharyngeal-cervical-brachial” variant that majorly impacts the throat, neck and upper arm muscles. Some can even involve the brain and spinal cord, termed “Bickerstaff Encephalitis.” There is a variant called the Miller-Fisher syndrome that is primarily characterized by eye muscle weakness, areflexia and incoordination of movements.
Testing for Guillain-Barre Syndrome
Guillain-Barré syndrome (GBS) is usually identified through physical examination and an evaluation of symptoms. However, extra tests may be needed for unusual cases or versions of the disease. These tests can help to differentiate GBS from other conditions with similar symptoms.
Electricity-based tests, like electromyography and nerve conduction studies, can help doctors distinguish between different types of nerve damage. These tests can also help reveal how recent a patient’s symptoms are. Sometimes, these studies can help doctors consider other possible conditions, like nerve-muscle connection disorders or nerve damage caused by diabetes. These electric-based tests should typically be done 10 to 14 days after symptoms first appear because it takes time for nerve damage to occur. However, studies have shown that these tests may be useful in diagnosing GBS as early as 3 to 7 days after symptom onset.
The most common early signs seen in these electrical tests for GBS include delayed or missing reflex responses. In GBS, doctors often find a specific pattern in the tests where a sensory nerve in the lower back is normal while other sensory responses are abnormal. Other findings may depend on which version of GBS the patient has. For example, certain types of GBS would more likely have issues like partial blockage of muscle conduction, slow conduction speeds, delayed reflex responses and prolonged distal latencies, which is the delay in electrical energy being conducted along the nerve.
Another phenomenon in some types of GBS is that some nerves can show partial or complete blockage in the nerve conduction study. This can happen due to something known as “reversible conduction failure,” where the body’s natural defense system mistakenly attacks parts of peripheral nerves. This can cause the nerves to have prolonged axonal damage or reverse, causing failure in conducting signals.
Spinal fluid test can also reveal features suggesting GBS. Typically, this test would show a normal amount of white blood cells but elevated protein levels. However, this is only found in 80% of patients at 2 weeks after symptoms start. So, absence of this does not rule out the diagnosis. If the white blood cell count is high, the doctor might consider other infections that mimic GBS.
Body’s immune response to gangliosides (fatty acid compounds in nerve tissues) in the form of antibodies have been found associated with GBS. However, these lab tests often require time before the results are available and thus, may not help immediately during the time of hospital admission.
Magnetic resonance imaging (MRI) of the spine might reveal inflammation of nerve roots in GBS, but its primarily used to rule out other diseases that could cause similar symptoms.
Finally, doctors should perform a negative inspiratory force (NIF) test on those suspected with GBS. This test measures the strength of breathing muscles. If patients are unable to perform a NIF of a certain level, they are considered to be at very high risk and require close monitoring.
Treatment Options for Guillain-Barre Syndrome
Guillain-Barré syndrome (GBS) is a condition where the immune system mistakenly attacks the nerves. In medical research, two treatments are considered to be the standard for managing this syndrome. These are either intravenous immunoglobulin (IVIG) or plasma exchange.
IVIG is a blood product that is rich in antibodies. It is believed to work by moderating the activity of the immune system. However, the exact way in which IVIG helps in treating GBS is still not fully understood. IVIG is typically given via an injection into a vein.
Plasma exchange, on the other hand, works by removing certain substances from the blood. It’s believed that this treatment may act by getting rid of harmful antibodies and other elements which contribute to GBS. Just like IVIG, the exact process of how plasma exchange helps in the treatment of GBS is not yet known. This procedure is typically done over several sessions.
Research studies have shown that both IVIG and plasma exchange are equally effective. Their effects are more pronounced if the treatment is started within two to four weeks after the onset of GBS symptoms.
Interestingly, certain commonly used anti-inflammatory drugs like corticosteroids (including oral prednisone and intravenous methylprednisolone) have not been shown to provide any benefit over not taking any drugs or using in combination with IVIG and plasma exchange.
On the bright side, these treatments have been shown to speed up the recovery process for patients dealing with GBS. For example, patients who received treatment were able to start walking unassisted almost a month earlier compared to those who did not receive treatment.
Looking at the broader picture, majority of the GBS patients, about 85%, manage to regain their ability to walk unassisted. However, there is still a significant portion of patients (20%) who experience long-term effects of the disease. Future research studies are currently looking at the possibility of using plasma exchange followed by IVIG or IVIG concurrently with steroids, but they have not shown any significant improvement yet.
Medical researchers are currently conducting trials with complement inhibitors, drugs that block certain immune system processes, in patients who don’t respond to the standard treatments. Additionally, they are also conducting trials involving two courses of IVIG. The results of these studies are expected in the coming year.
What else can Guillain-Barre Syndrome be?
Guillain-Barré syndrome (GBS) is now the main cause of sudden, severe muscle weakness around the world after polio has been wiped out. However, it might be confused with other disorders. In critically ill patients experiencing this kind of weakness along with multiple organ problems, it could be due to nerve or muscle problems related to their critical condition. Other health issues that can appear similar to GBS include:
- Tick paralysis
- Neuromuscular junction disorder
- Acute intermittent porphyria
- HIV infection
- Spinal cord disorders
- Toxic neuropathies
- Infections (like West Nile virus or rabies)
Some unusual symptoms like an early impact on the bowel and bladder, asymmetric features, and excessively active or normal reflexes can cue doctors to look for other diagnoses.
To tell GBS apart from similar conditions, doctors have to watchfully review the patient’s history, symptoms, and additional test results. For instance, if the patient has dilated pupils, it might be a sign of tick paralysis or botulism. Electromyography and nerve conduction studies, along with the patient’s history and symptoms, might assist doctors to differentiate between GBS and nerve/muscle problems associated with critical illness. If the cerebrospinal fluid test shows an abnormality called pleocytosis instead of the typical scenario in GBS, doctors might need to consider potential infections such as HIV or West Nile Virus.
What to expect with Guillain-Barre Syndrome
After the severe initial stage of Guillain-Barré syndrome (GBS – a neurological disorder that attacks the nervous system), a majority of patients (over 80%) can walk independently after six months. During this acute stage, less than 5% of patients lose their lives. However, less than 20% of patients continue to experience significant disabilities even after receiving standard GBS care.
Research is going on to identify these patients early on. Quick identification of factors suggesting poor prognosis (i.e., a high chance of the disease worsening) could allow clinicians to initiate further treatment targeting this specific group. A tool in use among Dutch patients, the Erasmus GBS Outcome score, uses a patient’s physical check-up, age, and history of diarrhea to predict their ability to walk in the near future.
Patients with a high risk of long-term disability could be most suitable for additional therapeutic trials. However, studies which investigated whether combining plasma exchange (a blood purification technique) with intravenous immunoglobulin (IVIG – a treatment procedure involving giving patients healthy antibodies through their veins) showed additional benefits were not significant.
Similarly, studies about adding corticosteroids (anti-inflammatory medicines) to IVIG were also not significant; however, they might benefit patients with worse prognosis, as indicated by factors like age and GBS disability score. According to a small study, giving two rounds of IVIG has been recommended as a possible intervention.
However, there might be some limitations to using IVIG due to possible side effects. Currently, a controlled test is ongoing to assess the benefits of two rounds of IVIG in GBS patients not responding to standard treatment. Also, since complement activation (an immune system response that contributes to destroying unwanted substances in the body) plays a critical role in GBS’s progression, a controlled trial is currently studying a medicine called eculizumab in GBS patients.
Several clinical features can indicate a need for artificial breathing support (ventilation) during the illness. These include a sudden onset of the disease (less than 7 days from onset to hospital admission), weaknesses in the throat and neck muscles. These factors could advise doctors to admit patients in intensive care units instead of less intensive facilities.
Finally, after recovery, patients might still deal with lasting fatigue, pain, and unusual touch sensations (paresthesias) for up to several years.
Possible Complications When Diagnosed with Guillain-Barre Syndrome
The complications that are most worrisome include difficulties with breathing and certain types of nerve paralysis.
Common Complications:
- Difficulties with breathing
- Certain types of nerve paralysis
