What is Guillain-Barre Syndrome?
Guillain-Barre syndrome (GBS) is a neurological disorder in which the body’s immune system inappropriately attacks healthy nerve cells. In the most common type of GBS, the action of the immune system damages a protective coating on the nerve cells called myelin. Myelin insulates nerve cells and allows them to function. As the cells’ myelin sheath breaks down, the symptoms of GBS result.
Types of Guillain-Barre Syndrome
GBS can be classified into several different types, including:
- Acute inflammatory demyelinating polyradiculoneuropathy (AIDP). This is the most common type of GBS in the United States and Europe. It is characterized by weakness that begins in the lower body before progressing to other parts of the body.
- Miller Fisher syndrome (MFS). This form of GBS begins with paralysis of the eyes and also includes problems with walking. It is more common in Asia.
- Acute motor axonal neuropathy (AMAN)
- Acute motor-sensory axonal neuropathy (AMSAN)
Symptoms of GBS
Symptoms of GBS can include:
- Tingling in the fingers, toes, ankles, or wrists
- Muscle weakness or paralysis in the legs and lower body that spreads to the upper body
- Difficulty chewing or swallowing
- Difficulty speaking
- Unsteady walking
- Problems moving the eyes
- Difficulty breathing
- Rapid heart rate
- Abnormal blood pressure (low or high)
- Pain or aches, especially at night
- Problems with bowel or bladder control
What Causes Guillain-Barre Syndrome?
Scientists don’t know precisely what causes the immune system to damage nerve cells. However, the abnormal immune response follows a viral or bacterial infection in the majority of cases. These infections are likely the triggering event that leads to an inappropriate reaction by the immune system. Infections that have been associated with GBS include:
- Zika virus
- Epstein Barr virus
- Campylobacter jejuni (a bacteria that causes diarrhea)
- Other respiratory or gastrointestinal infections
In rare cases, the onset of GBS has been associated with some vaccines. Doctors stress, however, the benefits of vaccinations far outweigh the small risk of GBS associated with the vaccines.
Is Guillain-Barre Syndrome Hereditary?
In the majority of cases, GBS does not appear to be inherited. Scientists have noted reports of cases that seem to cluster in families, but research has not yet discovered any clear genetic link to GBS. Some gene or genes may increase a person’s risk of developing the disorder, but developing GBS is likely to involve an interplay of multiple environmental and genetic factors.
How Is Guillain-Barre Syndrome Detected?
The most common early sign of GBS is tingling that begins in the toes or feet before spreading to the upper body. You should seek emergency medical care if you experience this kind of tingling or other potential signs of the disorder, including:
- Difficulty breathing while lying down
- Rapidly spreading tingling in any part of your body
- Choking on saliva or difficulty swallowing
How Is Guillain-Barre Syndrome Diagnosed?
When symptoms suggest the possibility of GBS, a doctor will conduct diagnostic tests and exams to look for the characteristic features of the disorder and to rule out other possible causes of the symptoms.
Diagnostic steps may include:
- Evaluation of the patient’s medical history
- Physical and neurological exams
- Electromyography (EMG) to measure electrical interaction between muscles and nerves
- Nerve conduction studies to measure the nervous system’s ability to send signals through the body
- Lumbar puncture (spinal tap) to look for abnormalities in the cerebrospinal fluid
PLEASE CONSULT A PHYSICIAN FOR MORE INFORMATION.
How Is Guillain-Barre Syndrome Treated?
GBS has no cure. However, some therapies and treatments may reduce the severity of symptoms and aid in a faster recovery.
Commonly used treatments include:
- Plasmapheresis. This therapy involves removing some of the liquid component of the patient’s blood (plasma). The treatment is thought to work by removing disease-causing antibodies in the plasma. The body then generates new plasma that does not contain the antibodies.
- Immunoglobulin therapy. This therapy involves the intravenous introduction of healthy donor antibodies into the patient’s bloodstream.
- Medications to alleviate pain or prevent blood clots
- Physical therapy
How Does Guillain-Barre Syndrome Progress?
Most people with GBS recover fully with treatment. During the disease’s typical progression, GBS symptoms usually develop and worsen over the course of about two weeks before plateauing at about four weeks. After that, recovery is gradual, taking anywhere from 6 months to 3 years.
About a third of people with GBS still experience some weakness after three years, and about 15% experience long-term weakness. Some people require the use of a wheelchair, walker, or other mobility assistance indefinitely.
How Is Guillain-Barre Syndrome Prevented?
There is no known way to prevent GBS.
Guillain-Barre Syndrome Caregiver Tips
Some people with GBS also suffer from other brain and mental health-related issues, a condition called co-morbidity. Some of the brain-related disorders that may be associated with GBS include:
- People with GBS may be at increased risk of depression.
- Anxiety appears to be associated with GBS in some people.
- Sleep disruptions are sometimes associated with GBS.
- People with GBS may be at increased risk of developing dementia.
Guillain-Barre Syndrome Brain Science
Myelin is a fatty substance that surrounds nerve axons, long extensions of nerve cells that stretch throughout the body, connecting nerve cells to other nerves, muscles, and organs. Myelin acts as an insulator, allowing electrical signals to travel effectively along the axons and pass from one cell to another. When a nerve cell’s myelin sheath is lost or damaged, the cell is unable to transmit signals properly.
GBS causes the loss of myelin in the peripheral nervous system, the nerves that branch off from the central nervous system (the brain and spinal cord) to carry signals to and from the extremities and the rest of the body. In some types of GBS, the disease causes damage to the nerve axons themselves. The damage interferes with the signals between the peripheral nerves and the central nervous system, resulting in difficulties with movement, the sense of touch, and other body functions.
In the case of GBS, scientists believe that the immune system mistakes chemicals in nerve cells for similar chemicals in infection-causing invaders such as viruses or bacteria. In an attempt to fight off the invaders, immune cells also attack similar-looking nerve cells. Researchers are still unsure how infections trigger these inappropriate immune reactions, and the mechanism may vary among the different types of GBS.
Guillain-Barre Syndrome Research
Title: National Vaccine Adverse Event Reporting Survey and Etiology (NVAERS)
Principal investigator: Donald C Cooper, PhD
Vaccinations are typically administered to healthy persons and are often mandated by states or federal authorities as a condition for school attendance or military service to prevent the spread of infectious diseases. Because vaccines are either mandated or recommended for vulnerable groups, such as children or the elderly, vaccines are often held to a higher standard of safety by the FDA than other medical products.
Due to the vast number of vaccine doses administered in the US, there is a need to gather quality information on serious vaccine-associated adverse events leading to serious injury. Vaccine-induced adverse events are typically reported in the CDC Vaccine Adverse Event Reporting System (VAERS) database, which contains information on unverified reports of adverse events (illnesses, health problems, and/or symptoms) following immunization with US-licensed vaccines. Reports are accepted from anyone and can be submitted electronically at www.vaers.hhs.gov.
The primary goal of this observational study is to establish an independent national database to classify vaccine-associated serious adverse events/injury data from vaccinated individuals. The plan is to gather survey data and health information from newly vaccinated individuals who have experienced serious adverse effects to help understand the possible causal relationships and plausible biological mechanisms underlying serious adverse events/injuries. The project aims to identify the genetic determinants of vaccine-induced adverse responses by studying host genetics. We plan to use Nebula Genomics’ platform for whole genome sequencing to identify single nucleotide polymorphisms associated with cardiovascular, neurological, gastrointestinal, musculoskeletal, and immunological symptoms induced by vaccine administration.
The secondary goal is to gather medical history and survey data from recently vaccinated individuals to develop a vaccine-injury risk assessment tool that may be used to predict individual vulnerability to vaccine adverse events/injury.
The tertiary goal is to establish a database to gather detailed long-term adverse reaction data from subjects enrolled in FDA Emergency Use Authorized vaccine clinical trials.
Title: Assessment of Chronic Guillain-Barre Syndrome Improvement With Use of 4-aminopyridine
Rehabilitation Institute of Michigan at Detroit Medical Center
In developed countries, Guillain-Barre Syndrome (GBS) is the most common cause of acute neuromuscular paralysis, afflicting about 5,000 persons annually in the United States. Over 20% of GBS patients have permanent residual motor deficits affecting their daily activities.
This study aims to assess the potential usefulness and safety of 4-aminopyridine (4-AP) in patients who suffer chronic functional deficits from GBS. This medication is a potassium channel blocker that has the potential to improve nerve conduction, particularly across partially demyelinated axons. It is felt that, by increasing nerve conduction, there will be improved motor performance for walking and activities of daily living, as well as decreased fatiguability. This medication has demonstrated potential usefulness in central demyelinating diseases such as multiple sclerosis. Because the peripheral nervous system is much more accessible to systemic medication delivery, it is hypothesized that this medication may improve the functional status of those patients who are suffering from the residual side effects of this medication.
Title: International Guillain-Barré Syndrome Outcome Study (IGOS)
Principal investigator: Dr. Bart Jacobs
Barrows Neurological Institute
GBS is a post-infectious immune-mediated polyradiculoneuropathy with a highly diverse clinical course and outcome despite partially effective forms of treatment (immunoglobulins and plasma exchange). The outcome in patients with GBS has not improved in the last two decades. At present, about 10 to 20% of patients remain severely disabled, and about 5% die. One explanation for this stagnation is the highly variable clinical course of GBS and the lack of knowledge about the factors determining the clinical course in individual patients with GBS. GBS may consist of distinct pathogenic subgroups, in which disease onset and progression are influenced by different types of preceding infections, anti-neural antibodies, and genetic polymorphisms. The optimal treatment of individual patients may depend on the pathogenesis and clinical severity. Patients with severe forms of GBS may need more intensive treatment to recover. Patients with a milder course that fully recover after standard therapy could suffer from possibly more side effects of more aggressive forms of treatment. This could only be possible if prognostic models accurately predict the clinical course in individual patients. Ideally, such models should be based on clinical and biological predictors strongly associated with the disease course and known as early as possible in the acute phase of illness, when treatment with immunomodulatory therapy is most effective. Prognostic models could help to guide selective trials in specific GBS subtypes. Because of this, it will be possible to treat GBS with more effective and individual therapy.
This study aims to identify clinical and biological determinants and predictors of disease course and outcome in individual patients with Guillain-Barré syndrome as early as possible after the onset of the disease. This information will be used to understand the diversity in clinical presentation and response to the treatment of GBS. This information will also be used to develop new prognostic models to predict the clinical course and outcome accurately in individual patients with GBS.
To address these research questions, it is required to conduct a prospective study with a standardized collection of clinical data and biomaterials from a large group of well-defined GBS patients during a long follow-up period. Such an extensive study in a relatively rare disease such as GBS can be addressed only by intensive international collaboration.