Brief overview
- Symptoms: Initially tingling and numbness in the arms and legs, with progression of the disease muscle weakness and paralysis in the legs as well as respiratory disorders
- Treatment: As early as possible by infusion with immunoglobulins (special antibodies) or plasma exchange procedures (plasmapheresis); cortisone helps with acute GBS, other possible medications are heparins for thrombosis prophylaxis or antibiotic therapy, physiotherapy and psychotherapy are used in the course of rehabilitation
- Causes and risk factors: Largely unexplained, usually occurs in connection with previous viral infections such as COVID-19 or the Epstein-Barr virus as well as after bacterial infections with Campylobacter jejuni, for example
- Course of the disease and prognosis: Rapidly increasing symptoms within the first four weeks, in particular muscle weakness and neuronal disorders, which then stabilize; prognosis usually favourable, but with a slow, sometimes incomplete healing process
- Diagnosis: Physical examinations, laboratory tests of blood samples and cerebrospinal fluid (cerebrospinal fluid puncture), electroneurography, magnetic resonance imaging (MRI)
- Prevention: As the exact causes and triggers of Guillain-Barré syndrome are still largely unknown, there are no recommendations for prevention.
The first signs of the onset of GBS are non-specific and resemble those of a mild infection. For example, back and limb pain may occur. In contrast to other diseases such as meningitis, Guillain-Barré syndrome usually does not cause fever in the early stages.
As the disease progresses, the actual Guillain-Barré syndrome develops with paresthesia, pain and paralysis in the hands and feet. These deficits are often more or less equally pronounced on both sides (symmetrical). The paralysis, which develops within hours to days, is particularly typical. These symptoms, which usually begin in the legs, progressively move towards the trunk of the body and gradually increase in intensity.
The back pain sometimes leads to the misdiagnosis of a slipped disc. It is probably the inflammation of the nerve pairs emerging from the spinal cord (spinal nerves) that causes the pain in Guillain-Barré syndrome.
Guillain-Barré syndrome reaches its peak in the second to third week of illness. After this, the symptoms initially remain stable (plateau phase) before slowly subsiding over a period of eight to twelve weeks.
In many patients, the so-called cranial nerves are affected by Guillain-Barré syndrome. These nerve tracts emerge directly from the brain and primarily control sensitivity and motor function in the head and facial area.
Typical of cranial nerve involvement in Guillain-Barré syndrome is bilateral paralysis of the seventh cranial nerve (facial nerve), which leads to facial nerve paralysis (facial nerve palsy). This manifests itself in sensory and movement disorders in the face, especially in the mouth and eye area. In those affected, this can be recognized by a lack of or impaired facial expressions, among other things.
In Guillain-Barré syndrome, it is also possible that the autonomic nervous system is affected. This leads to dysfunction of the circulatory system and glands (sweat, salivary and lacrimal glands). The normal function of the bladder and rectum is also sometimes impaired, resulting in incontinence.
Special forms of Guillain-Barré syndrome
Miller-Fisher syndrome is a special form of GBS that particularly affects the cranial nerves. The three main symptoms of this special form are paralysis of the eye muscles, loss of reflexes and gait disorders. In contrast to classic Guillain-Barré syndrome, the paralysis of the extremities is only mild in Miller-Fisher syndrome.
How is Guillain-Barré syndrome treated?
Depending on the severity of the symptoms, Guillain-Barré syndrome is treated in an intensive care unit. This is not necessary in mild cases, but monitoring in a normal hospital ward is usually essential. In some cases, Guillain-Barré syndrome leads to life-threatening paralysis. The patient must be closely monitored at regular intervals, particularly in the event of respiratory, cardiovascular or swallowing reflex disorders.
Life-threatening situations sometimes occur suddenly and require rapid treatment. In the case of severe Guillain-Barré syndrome, for example, doctors and nursing staff must be constantly prepared for the occurrence of serious cardiac arrhythmia or the need for artificial respiration. Such artificial respiration is necessary at times in around 20 percent of cases.
There is no known causal therapy for GBS. In more severe cases, immunomodulating therapy with so-called immunoglobulins, which the patient receives via an infusion, is useful. These are a mixture of antibodies that interact with the autoaggressive antibodies and thus normalize the immune response.
Experts do not currently recommend combining the administration of immunoglobulins and plasma exchange.
Cortisone is another treatment option for patients with chronic GBS. However, the drug is not effective in acute Guillain-Barré syndrome.
If many muscles are affected by the paralysis and the patient is no longer able to move sufficiently, so-called heparins are used to prevent the formation of blood clots (thrombosis prophylaxis). For this purpose, an injection is usually administered under the skin (subcutaneously) once a day. It is also important to start accompanying physiotherapy as early as possible in order to support the body in maintaining its ability to move and to promote rapid regeneration.
Some patients with Guillain-Barré syndrome are very frightened by their illness, particularly because of the paralysis. However, in the majority of cases, these unpleasant symptoms generally disappear completely.
If the unpredictable course of GBS leads to severe psychological stress, intensive support for the patient (e.g. psychotherapy) is advisable. If the anxiety becomes particularly severe, medication is sometimes used to reduce the anxiety.
Causes of GBS – complication after COVID-19 vaccination?
For example, doctors have investigated a link between GBS and vaccinations against SARS-CoV-2 (COVID-19) and found that by the end of May 2021, symptoms of GBS had appeared in over 150 cases in Germany within four to a maximum of six weeks after the first dose of vaccine. They mostly manifested as bilateral facial paralysis and sensory disturbances (paraesthesia).
Infection with the COVID-19 virus or other infections were not present in any of the cases. The experts have not yet established a clear link between the COVID-19 vaccination and GBS and have not observed any significant increase in GBS cases over the vaccination period. The European Medicines Agency (EMA) therefore assumes that GBS is very unlikely to be caused by vaccination against SARS-CoV-2.
Experts from the Paul Ehrlich Institute (PEI), which is responsible for German vaccine approvals, have already investigated a similar connection for the swine flu vaccination. According to the study, vaccinated people had no to a slightly increased risk of developing Guillain-Barré syndrome in the six weeks following vaccination. During this period, around six people in every million vaccinated would also develop GBS.
Other causes of Guillain-Barré syndrome: Infections
GBS often begins seven to ten days after an infection. In addition to SARS-CoV-2, possible triggers include the Epstein-Barr virus, the Zika virus or the cytomegalovirus.
It is suspected that autoaggressive immune cells, which are directed against the body and attack the insulating sheaths of the nerve tracts (myelin sheaths), provoke inflammation of the nerves (polyneuritis). This is accompanied by inflammation-related swelling (edema) of the nerves.
Campylobacter jejuni, a bacterial pathogen that causes gastrointestinal infections, is probably the most common trigger of GBS. During an infection, the body forms antibodies against the surface structures of a pathogen. Campylobacter jejuni has structures on its surface that resemble those of the nerve sheath. Experts therefore assume that the antibodies against the pathogen continue to circulate in the body after the infection has been overcome and now attack the nerves due to the similar surface structures (“molecular mimicry”). However, only around 30 out of 100,000 people infected with this bacterium develop Guillain-Barré syndrome. This assumption of “molecular mimicry” also applies to other bacteria and viruses.
Complications and consequential damage
For the majority of those affected, the disease means a restriction or change in their previous life. Severe long-term complications due to respiratory and cardiovascular problems are possible. As those affected are increasingly less able to move, the risk of blood clots forming in the blood vessels (thrombosis) increases. Lying down for long periods of time often leads to blood clots that block the blood vessels (leg vein thrombosis, pulmonary embolism).
If the symptoms of muscle paralysis persist for a long time, muscle atrophy is more common.
Course of the disease and prognosis
During the plateau phase of GBS, movement restrictions and other symptoms are usually severe. However, the further course of the disease is favorable for the vast majority of patients: the symptoms disappear completely in around 70 percent of those affected. However, complete recovery can take many months. In some cases, the regression of symptoms is also incomplete.
One year after the disease, a third of patients still complain of pain. Around 15 percent of those affected are permanently ill and continue to suffer from muscle weakness and neuronal disorders. For example, they need walking aids to get around.
Children and adolescents rarely suffer long-term damage, although it is possible that mild disorders may persist in them. For this reason, the course of the disease is usually more favorable in children.
What is Guillain-Barré syndrome?
In 1916, the three French doctors Guillain, Barré and Strohl described Guillain-Barré syndrome (GBS) for the first time. “Syndrome” means that it is a disease characterized by a specific combination of symptoms.
Guillain-Barré syndrome is a rare disease of the nervous system and is characterized by ascending paralysis (paresis) and sensory disturbances, which usually begin on both sides of the hands or feet. These deficits occur because immune cells attack the insulating sheathing of the body’s own nerve tracts (demyelination) and also damage the nerve tracts (axons) themselves.
These immune cells are autoaggressive, which is why Guillain-Barré syndrome is an autoimmune disease. In GBS, it is primarily peripheral nerve tracts (peripheral nervous system) and the nerve pairs emerging from the spinal cord (spinal nerves) that are damaged. The so-called central nervous system, which includes the brain and spinal cord, is less frequently affected.
The causes of Guillain-Barré syndrome are still largely unclear. However, the disease usually occurs after an infection.
Frequency
Around one in every hundred thousand people in Germany is diagnosed with Guillain-Barré syndrome each year. GBS is more common in older people, although in some cases middle-aged people, children and adolescents are also affected. Men also develop Guillain-Barré syndrome slightly more frequently than women.
In around 70 percent of cases, the symptoms disappear completely within weeks or months. However, around eight percent of sufferers die from complications of GBS, such as respiratory paralysis or pulmonary embolism. Guillain-Barré syndrome occurs more frequently in spring and fall, possibly because infections are much more common at these times of year.
How is Guillain-Barré syndrome diagnosed?
If Guillain-Barré syndrome is suspected, doctors recommend visiting a neurological clinic with an intensive care unit immediately. The doctor will obtain important information (medical history) from the description of your symptoms and any previous illnesses. Typical questions that the doctor will ask if Guillain-Barré syndrome is suspected are
- Have you been ill in the last four weeks (cold or gastrointestinal infection)?
- Have you been vaccinated in the last few weeks?
- Have you noticed any signs of paralysis or paresthesia in your hands, feet or other parts of your body?
- Do you have back pain?
- Are you taking any medication?
Physical examination
The medical history is followed by a physical examination. The doctor tests sensitivity and muscle strength in various parts of the body. A check of the twelve cranial nerves and reflexes is also part of the physical examination.
Experts have defined criteria that the doctor uses to diagnose Guillain-Barré syndrome. The three main criteria required are
- Progressive weakness of more than one limb over a maximum of four weeks
- Loss of certain reflexes
- Exclusion of other causes
Further examinations
After a thorough physical examination, a sample of cerebrospinal fluid (CSF) is taken in the clinic and examined in the laboratory (CSF puncture). This is absolutely necessary to confirm the suspicion of Guillain-Barré syndrome and to rule out other causes. To obtain the cerebrospinal fluid, the doctor inserts a very fine needle at the level of the lumbar spine up to the spinal canal and draws off the cerebrospinal fluid with a syringe. The spinal cord ends above the puncture site so that it is not injured.
If Guillain-Barré syndrome is suspected, it is also important to examine the nerve conduction disorders more closely using electrophysiological examinations. For example, the conductivity of nerves is tested using short electrical impulses (electroneurography).
Nerve conduction velocity is typically reduced in Guillain-Barré syndrome as the insulating myelin sheaths are destroyed segment by segment by the immune cells. However, this can only be measured after a few days of illness. For this reason, electrophysiological examinations should be repeated regularly during the course of Guillain-Barré syndrome.
In around 30 percent of cases of Guillain-Barré syndrome, certain antibodies against components of the nerve sheath (e.g. anti-GQ1b-AK, anti-GM1-AK) can be found in the blood. Only rarely is it still possible to determine the causative agent of an infection preceding Guillain-Barré syndrome. This is somewhat more common in children than in adults. The determination of the pathogen usually has no influence on the therapy.
Due to the severe effects of GBS, doctors check the muscle strength and general parameters of heart and respiratory function of patients with Guillain-Barré syndrome every four to eight hours. Close monitoring is particularly necessary in older people or if the symptoms progress rapidly. Doctors pay particular attention to possible complications such as respiratory paralysis (Landry’s paralysis) and pulmonary embolism.
Prevention
As the exact causes and triggers of Guillain-Barré syndrome are still largely unknown, there are no recommendations for prevention.
