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What is Guillain-Barré Syndrome?
Guillain-Barré syndrome is a rare autoimmune disease that affects the peripheral nervous system through inflammation, causing peripheral nerve degeneration, specifically in the myelin sheath.
The Peripheral Nervous System (PNS) is composed of the nerves that go from the brain or spinal cord (both part of the Central Nervous System, or CNS) to the rest of the body. The SNP is made up of nerves that reach the muscles, carrying signals from the CNS or to the CNS.
In some cases, the disease can also affect the Autonomic Nervous System (ANS), which coordinates those muscular actions that we have no control over, such as heartbeat and breathing.
The myelin sheaths run along the nerves, making electrical impulses pass through them faster. As the sheaths are destroyed, the impulses slow down and may even stop passing.
The result of this is a weakening of the muscles, which often begins in the limbs and spreads over time, reaching the area of the body closest to the CNS.
Types
There are a few different types of the disease, which vary depending on the main region affected. Variations of the disease are very rare, especially considering that the classic version is already rare. The types are:
Classical Guillain-Barré syndrome
The classic version of the disease affects the entire body at once. However, in some situations, cranial nerves may not be affected.
Paraparética Guillain-Barré syndrome
The main difference between the classic and paraparetic versions is that in the latter, only the lower limbs are affected.
Pharyngo-cervical-brachial weakness
This type of the disease is more common in adolescents and begins in the bulbar region – the part of the brain that is closest to the nape, made up of the cerebellum and the beginning of the spinal cord – and spreads through the neck, arms and the region of the mouth and pharynx .
Bifacial weakness with paresthesias
This type of disease affects the cranial nerves, causing weakness mainly in the facial muscles of the forehead and oropharynx (mouth and pharynx). It can affect the eyelids.
Read more: Treatments for Paresthesia
Miller Fisher Syndrome
This version of Guillain-Barré Syndrome is very rare. It is estimated to affect 1 in 1 million people per year. Most cases appear after infection by the bacterium Campylobacter jejuni , which causes diarrhea.
The disease initially affects the eye muscles and in some cases can affect the Central Nervous System, causing changes in consciousness.
Causes
The mechanism by which Guillain-Barré syndrome is activated is not known. It is known that it is an autoimmune disease, that is, the person’s own immune system attacks its cells, causing damage.
Some types of infection can trigger the reaction in the human body, but all have Guillain-Barré as a rare consequence. Are they:
- Zika fever (caused by the Zika virus);
- Diarrhea caused by the bacterium Campylobacter jejuni ;
- Epstein-Barr virus (Cause of mononucleosis);
- Human Immunodeficiency Virus (HIV);
- Hepatitis A, B or C;
- Pneumonias.
In addition, it is possible to develop Guillain-Barré syndrome from other situations such as surgery, Hodgkin’s lymphoma and, very rarely, through immunization against influenza, for example.
From these triggering factors, the immune system is altered and begins to produce antibodies against the body itself, attacking the myelin sheath of peripheral nerves. An inflammatory process begins and demyelination of the nerve occurs, that is, its destruction.
Guillain-Barré e Zika syndrome
Although Guillain-Barré Syndrome is not exclusively caused by the Zika virus (several infections are triggered), there was a suspicion that there is more predisposition, as there was an increase in cases of Guillain-Barré syndrome after the patient had Zika ( disease transmitted by the bite of the Aedes mosquito).
But due to the few studies still developed, the greater relationship between diseases is just an assumption.
A study published in the medical journal Epidemiology and Health Services, in 3018, points out that in 2015 there were 3 times more cases of Guillain-Barré Syndrome than in 2014. Of the 44 patients analyzed in the study, 18 had had symptoms of Zika before the symptomatic manifestation of the Syndrome.
Guillain-Barré syndrome and influenza vaccine
Between 1976 and 1977, the flu vaccine was produced with swine influenza viruses. At the time, there was an increase in the rates of Guillain-Barré syndrome after vaccination, pointing to the relationship between them.
However, after the occurrence, the vaccine started to be produced with other strains of the virus and there is currently no proven relationship that the vaccine can increase the chances of the syndrome.
It is worth remembering that the incidence of the syndrome is quite small and that vaccination prevents diseases and infections.
Groups and risk factors
Among the main risk factors are infections that can cause the disease. As the mechanism of activation of the syndrome is not known, it is difficult to indicate exactly who is most likely to develop it.
Zika fever, diarrhea, mononucleosis, HIV and pneumonia can result in the disease, as well as surgery.
Symptoms
Guillain-Barré syndrome develops quickly, in some cases it can take a few weeks, but in others it can only take a few hours. Symptoms are usually:
Tingling and numbness
With reduced electrical impulses, the upper and lower limbs can begin to tingle and give a sensation of numbness. After starting, they have a progressive manifestation, in which they often start in the legs and advance to the middle of the body.
Progressive weakness
Like tingling, the weakness that increases with time tends to start in the legs, gradually advancing to the rest of the body, as electrical impulses cannot reach the regions furthest from the spinal cord.
The weakness, in general, worsens between 3 and 4 weeks after it starts to manifest and stabilizes or regresses to the normal state of the organism. When the symptom persists and worsens for more than 8 weeks, there is a condition called chronic inflammatory demyelinating polyneuropathy.
On average, between 3% and 10% of patients with Guillain-Barré syndrome progress to chronic inflammatory demyelinating polyneuropathy.
Paralysis
Weakness can develop until it becomes paralysis. When the myelin sheath of the nerves is destroyed enough that electrical impulses cannot get through, the limbs can become paralyzed.
The level and intensity of the paralysis can be quite variable. In some cases, it can be a very mild manifestation, but it can also affect the respiratory muscles requiring mechanical ventilation (MV).
Cardiac arrhythmias, which are changes in the heartbeat, can occur intensely, but tend to last a maximum of 2 weeks.
Drop in blood pressure
The drop in pressure is the result of weakness that affects the muscles, both in the limbs and in the heart itself, and is therefore a sign due to muscle changes.
Reduced sensitivity
With the damaged myelin sheaths, the electrical impulses that carry the pain and tenderness signals also find it difficult to reach the central nervous system. Heat, cold, touch and pain can be reduced.
Muscle pain
Although sensitivity may be reduced during Guillain-Barré syndrome, neuropathic pain may be present. Among the manifestations are the sensation of acute (intense) burning and pain similar to electric shock, which can be prolonged and stable or pulsatile.
Lack of coordination
When combined with other symptoms, the sudden difficulty in motor coordination is a sign of Guillain-Barré syndrome. Due to the difficulty that electrical signals have to reach the limbs, coordination is affected.
How is the diagnosis made?
The diagnosis of Guillain-Barré syndrome can be made by the general practitioner and the neurologist.
To confirm the diagnosis, the patient needs to present some criteria, such as:
- Presence of 2 essential criteria;
- At least 3 clinical criteria suggestive of the syndrome;
- Do not present more than 1 condition capable of excluding the syndrome (such as HIV infection, sarcoidosis or polyneuropathy);
- Having no condition capable of excluding the syndrome.
The essential criteria are: Progressive weakness of muscle groups or limbs; Hyporeflexia (decreased body reflexes) and areflexia (absence of reflexes) along with varying degrees of hyperreflexia (very exaggerated reflexes). Along with the two criteria above, the patient must have at least 3 conditions below:
- During the 4 weeks, the symptoms worsen and progress;
- Absence of fever at the beginning of the picture;
- Pain and feeling of numbness throughout the body;
- Weakness of facial muscles;
- Limb paralysis occurs relatively symmetrically (on both sides of the body);
- Specific tests should show cellular changes or speed in motor conduction.
Obeying at least 3 criteria above, the patient needs to fit in at most 1 criterion below:
- Asymmetric weakness;
- Bowel and bladder dysfunction or change at the onset of the condition;
- Permanence of bowel and bladder changes;
- Presence of more than 50 cells / mm3 in the examination of cerebrospinal fluid;
- Presence of polymorphonuclear cells in the CSF;
- Sensitive level well demarcated.
However, in order to guide the doctor to the diagnosis of Guillain-Barré syndrome, the patient cannot meet any of the criteria below:
- Have been exposed to hexacarbon, a substance present in solvents, heavy metals, paints and pesticides;
- Indications that the body does not carry out the proper metabolism of porphyrin;
- Recent diphtheria;
- Clinical suspicion of heavy metal poisoning, such as lead;
- Absence of motor signals, which characterize a pure sensory syndrome;
- Diagnosis of botulism, myasthenia gravis, polio, toxic neuropathy or conversion paralysis.
Exams
The tests that can be performed and help to establish the patient within the diagnostic criteria are:
Electroneuromyography
The electromyography is a test that seeks to identify lesions in the peripheral nervous system. It measures the electrical activity of nerves and the speed of conduction of signals, as well as muscle responses.
Electrodes are connected to the patient’s skin in the regions where the nerves pass, and small shocks – safe and of low intensity – are applied to measure the activity.
Through the electrode data, it is possible to identify problems in the electrical activity of the body, such as the lack or damage of the myelin sheath.
Before performing this test, patients should inform the doctor about the presence of a pacemaker or any other device.
Analysis of cerebrospinal fluid (CSF)
CSF is a fluid that protects the central nervous system and is located in the vicinity of the brain and spinal cord.
The analysis is relevant for the diagnosis of several neurological pathologies, in addition to vascular changes, infectious and inflammatory processes.
In general, the lumbar region is the most used for the collection of material (lumbar puncture), but the suboccipital regions and the ventricular route (both located in the skull region) also allow the examination.
The exam is quick and usually causes mild discomfort and pain, which can cause headache or tenderness at the puncture site, which usually subside in a few hours.
Electrocardiogram
Electrocardiograms seek to find effects of Guillain-Barré syndrome in the heart, since when the affected nerves are part of the autonomic nervous system, the heart rate can be affected.
To perform the exam, electrodes are placed over the patient’s body (similar to a patch), capable of recording the heart rate.
Lung tests
In the same way as the electrocardiogram, lung exams aim to analyze the way the respiratory system is being affected by the disease, evaluating the organism’s involvement due to the disease.
Is there a cure?
No . Guillain-Barré syndrome is characterized by an attack by the immune system to the nervous system. In other words, the disease is considered to be present during the weeks when the condition progresses and begins to regress (or stabilize).
It is mainly between the second and fourth week that most patients have their symptomatic conditions stabilized and regress. From that moment on, it is the consequences of the syndrome that must be treated.
What is the treatment?
Treatment is based on the alleviation of symptoms, while the disease is ongoing, and the adoption of preventive measures, considering the possible consequences.
At first, it is recommended that the patient be hospitalized or assisted in observation centers, for the strict monitoring of the condition.
Possible complications can be avoided through cardiac monitoring, respiratory monitoring, protection of the airways, care for intestinal and urinary functions, in addition to adequate nutrition.
Physiotherapy sessions, while myelin sheaths regenerate, can be recommended to speed up the recovery of motor functions.
Therapeutic referral depends on the situation of each patient. In general, the treatments below are indicated when the patient has a moderate to severe condition. That is, there must be a need to use walking sticks or supports to walk, an inability to get around and / or a need for assisted ventilation. The treatments are:
Plasmaférese
Plasmapheresis is a procedure very similar to hemodialysis, in which the patient’s blood is filtered so that the plasma is separated from the rest of the substances present in the blood.
The goal is to remove antibodies or autoimmune agents that are attacking the body’s organs or systems.
Although this procedure does not completely stop the destruction of the myelin sheaths, the concentration of disease agents is reduced, causing the damage to be considerably less. The patient receives serum to replace the fluid and the antibodies are replaced by the body itself over the next few hours.
Intravenous human immunoglobulins
Immunoglobulin is used more consistently than plasmapheresis, as it tends to be easier to administer, safer and have fewer side effects.
The procedure consists of the intravenous application of immunoglobulins, which are immune cells, in order to balance the immune system and reduce the destruction of the myelin sheaths.
Physiotherapy
Physiotherapy is geared to the needs of the patient, who may have very different degrees of involvement.
In more intense conditions, the ability to move around can be greatly affected, making the patient need to stay in bed constantly. Thus, physiotherapy assists in the recovery of movements by increasing strength, motor control and muscle stability.
Psychological monitoring
The impact on mental health that the patient suffers is usually quite high, so it is important that there is a therapeutic work aimed at psychological assistance.
In that case, work with psychologists may be necessary during and after symptom regression.
Medicines
The medication used during the treatment of Guillain-Barré syndrome is intended to fight a possible infection that may still be active. The medical literature includes the use of Doxycycline , an antibiotic medication, with good results in Guillain-Barré Syndrome.
In addition, treatment with intravenous immunoglobulins is done with injections of the drug. Among the brands sold are: Hizentra , Sandoglobulina and Privigen .
Attention!
NEVER self-medicate or stop using a medication without first consulting a doctor. Only he will be able to tell which medication, dosage and duration of treatment is the most suitable for his specific case. The information contained in this website is only intended to inform, not in any way intended to replace the guidance of a specialist or serve as a recommendation for any type of treatment. Always follow the instructions on the package insert and, if symptoms persist, seek medical or pharmaceutical advice.
Living together
Living with the disease can be a challenge during the phase in which it is active (period with symptoms progressing) and in the following months.
Weakness and paralysis can be frightening, and the prospect of loss of movement can also be frightening. However, recovery is possible and even extremely likely.
It is necessary to seek treatment as soon as possible, especially because of the risk that presents itself in the form of respiratory paralysis. After the acute phase of the disease has passed, physiotherapy can help the body regain muscle strength while the myelin sheaths recover.
In this sense, the routine needs to be adapted and facilitate recovery. In addition to properly maintaining physiotherapy, when recommended by the doctor, it may be indicated to adopt walkers or walking sticks to facilitate mobility.
Often manual activities, such as eating or brushing your teeth, can become quite difficult. For this reason, relying on specialized assistance (such as occupational therapists) and from the family itself is essential.
Prognosis
Although there is an optimistic prognosis in relation to Guillain-Barré syndrome, recovery is generally slow and not always complete, and after 2 years, only 15% of patients do not have any remnants or damage caused by the syndrome.
On average, 10% of patients remain with severe and disabling sequelae and up to 7% can result in death due to severe lung or cardiac damage.
In the recovery of most cases, the time is between 3 and 18 months for the sheaths to recover.
Complications
The main complications of Guillain-Barré syndrome are as follows:
Permanent loss or reduction of movements
In the vast majority of cases, recovery is complete or almost complete. When full regeneration in the myelin sheaths is not possible, there may be a loss or reduced ability to move certain parts of the body.
This type of loss can be small to the point of not being noticeable or large, leading to severe muscle weakness or even paralysis in extreme cases. However, these cases are rare.
Cardiac weakness
In severe conditions of the disease, the cardiac muscles may be weakened or even immobile. In such cases, there is a serious risk of cardiac arrest. It is one of the lethal consequences of Guillain-Barré syndrome, but it is also extremely rare.
Respiratory failure
Like heart problems, it is possible that the disease paralyzes or weakens lung function, impairing breathing. It may be necessary to resort to mechanical breathing therapy.
Death
Only 2% to 5% of patients die as a result of Guillain-Barré syndrome, and the evolution is related to cardiac or respiratory arrest.
How to prevent Guillain-Barré syndrome?
Preventing Guillain-Barré syndrome is a complex task, since the disease is very rare and it is not known exactly what causes it. What you can do is to avoid certain infections, reducing the risks.
Wash your hands thoroughly
Washing your hands whenever possible and necessary considerably reduces the chances of contamination. The skin can protect the body from most infectious agents, but the hands often come into contact with the mouth, nose or eyes, which are areas that can allow pathogens to enter the body.
Filter and boil water
Boiling water before using it for food eliminates bacteria that may be alive in them. Filtering also removes a variety of infectious agents that can cause disease that can eventually lead to Guillain-Barré syndrome, so it is an important step in preventing the disease.
Wash the food well
Washing food can prevent several pathologies caused by microorganisms living in the food. It is recommended to carry out this washing with water that has been filtered or boiled to prevent pathogens from coming into contact with food through the water.
Common questions
How common is the disease?
Guillain-Barré syndrome is extremely rare, affecting around one person per 100,000 per year.
Will I have the disease forever?
If you have contracted Guillain-Barré syndrome, there is a possibility that it will return, but recurrence is very rare and there is probably no need to fear it.
Can I die from Guillain-Barré Syndrome?
It is possible that the disease leads to death when it affects the Autonomic Nervous System, but only 2% to 5% of patients who contract the disease will die, that is, in most cases, death is not a risk when there is treatment.
Guillain-Barré syndrome is a rare autoimmune disease that can lead to muscle weakness and temporary paralysis. On rare occasions it can lead to death, but in most cases, the patient will recover completely or almost completely within three months. Share this text with your friends so they can learn about the disease!
