According to abbreviationfinder.org, Guillain-Barré syndrome is an acute inflammation of the peripheral nerves and spinal ganglia (nerve nodes in the spinal canal) with an etiology (cause) that is still unclear. With an incidence of 1 to 2 new cases in 100,000 people per year, Guillain-Barré syndrome is a rare disease that affects men slightly more often than women.
What is Guillain-Barré Syndrome?
Guillain-Barré syndrome is an acute idiopathic (etiology of unclear) polyneuritis with multifocal (occurring at several sites) inflammation in the peripheral nervous system.
Inflammatory changes, especially in the roots of the peripheral nerves (polyradiculitis) and the proximal spinal ganglia, cause sensory disturbances, motor paralysis and vegetative dysfunction. Paraesthesia (tingling or “pins and needles”) as well as paralysis rising from the legs, which can be life-threatening in combination with respiratory paralysis and/or cardiac arrhythmia, are characteristic.
In addition, in some cases, involvement of the cranial nerves with dysphagia and bilateral facial paralysis can be observed. Depending on the course, Guillain-Barré syndrome is differentiated into different forms, with the most common variant also being referred to as acute inflammatory (inflammatory) demyelinating (damaging the marrow sheath) polyneuropathy.
The underlying causes of Guillain-Barré syndrome have not yet been conclusively clarified. Above all, immunological processes are suspected, since Guillain-Barré syndrome occurs in more than half of those affected (about 60 to 70 percent) following pulmonary or gastrointestinal infections.
Guillain-Barré syndrome is associated in particular with cytomegalovirus, varicella-zoster, measles, Epstein-Barr, mumps, hepatitis and HI viruses and certain bacteria such as salmonella, brucella, spirochetes, mycoplasma pneumoniae or campylobacter jejuni.
In very rare cases, Guillain-Barré syndrome manifests itself after influenza or rabies vaccinations. It is assumed that the antibodies formed by the body as a result of the infection are directed against the body’s own structures, in particular against the gangliosides that are increasingly found in the nervous system, and in combination with other still unknown factors cause the development of Guillain-Barré syndrome.
Symptoms, Ailments & Signs
The symptoms of Guillain-Barré syndrome are largely dependent on the corresponding course. A basic distinction is made between acute and chronic forms. In general, Guillain-Barré syndrome is characterized by increasing general weakness due to destruction of peripheral nerves and spinal nerve roots.
The acute form (acute inflammatory demyelinating polyneuropathy or AIDP) begins with back and joint pain, tingling and numbness in the fingers, toes, nose, ears or chin (acren) and signs of paralysis in the legs. Furthermore, the musculature of the pelvic, torso and respiratory musculature becomes paralyzed, resulting in the loss of all reflexes. In some cases, certain cranial nerves also fail.
This leads to disturbances in respiratory regulation, regulation of heart rate and bladder emptying as well as temperature regulation. Furthermore, circulatory disorders occur due to blood pressure fluctuations. The chronic form of Guillain-Barré syndrome, also known as chronic inflammatory demyelinating polyneuropathy (CIDP), begins insidiously and is characterized by alternating symptoms.
In chronic inflammatory demyelinating polyneuropathy, paralysis of the legs and paresthesia of the acral muscles are the main features. Involvement of cranial nerves is rarely observed here. The paralysis increases much more slowly in the chronic form. They can even be suppressed by administering glucocorticoids. Overall, a third of patients can be cured. Ten percent of those infected die. Some of those affected require lifelong care.
Diagnosis & History
Guillain-Barré syndrome is usually diagnosed on the basis of a CSF analysis (examination of the cerebrospinal fluid). If an increased protein concentration (cytoalbumin dissociation) can be detected with a normal cell count, Guillain-Barré syndrome can be assumed.
In addition, in Guillain-Barré syndrome, the nerve conduction speed, which is measured as part of an electroneurography, is reduced. An electromyography enables statements to be made about possible disturbances in the nerve tracts supplying the muscle fibers. However, this diagnostic method is not suitable for the early diagnosis of Guillain-Barré syndrome, since the corresponding changes can only be determined after about two weeks.
Existing cardiac arrhythmias can be detected with the help of an electrocardiogram, while the respiratory function can be checked with a lung function and blood gas analysis. In addition, in many cases antibodies against gangliosides can be detected in the serum. A Guillain-Barré syndrome usually has a favorable course and those affected are largely or completely healed within 1 to 6 months.
Guillain-Barré syndrome causes those affected to suffer from inflammation of the nerves. In most cases, this inflammation leads to sensory disturbances and paralysis. These do not have to occur all over the body, the affected region usually depends on the nerve that is disturbed. The patient suffers from the typical tingling and numbness.
Most patients also experience back pain and muscle pain. Furthermore, coordination and gait disorders also occur. The patient’s movement is restricted by Guillain-Barré syndrome. In the worst case, this leads to paraplegia, in which case the patient is then dependent on a wheelchair.
Not infrequently, the help of other people is then necessary in everyday life in order to be able to continue to master it. The pain can also occur at night, leading to difficulty sleeping. In many cases, the patient’s immune system is also weakened, making it easier for inflammation and infections to occur.
Guillain-Barré syndrome can be treated with medication. The sooner treatment begins, the greater the likelihood that the patient will be fully cured. Late treatment can lead to consequential damage, which is usually irreversible and can no longer be treated.
When should you go to the doctor?
Guillain-Barré syndrome always needs to be evaluated by a doctor. Without treatment, it can lead to serious symptoms and complications, which are usually irreversible. As a rule, the doctor should be consulted if there is severe back pain or paralysis that does not go away on its own.
Numbness or sensory disturbances can also indicate Guillain-Barré syndrome. Those affected often suffer from tingling in the affected regions. Furthermore, the doctor should be consulted if there is severe pain in the muscles.
Pain can occur even without movement. Likewise, disturbances in coordination or gait disturbances often indicate Guillain-Barré syndrome. If the syndrome is not treated, the worst case scenario can lead to complete paraplegia in the affected person. This is irreversible and can no longer be treated.
If these symptoms occur, a general practitioner should be consulted. This can determine the Guillain-Barré syndrome. However, further treatment depends on the exact symptoms and causes of the syndrome and is then treated by a specialist.
Treatment & Therapy
The therapeutic measures in Guillain-Barré syndrome correlate with the specific course of the disease. Therapy for mild forms aims to reduce existing paresis (paralysis of the muscles) and minimize the risk of infectious diseases, pneumonia, thrombosis and contractures (restricted mobility of the joints) and decubitus ( bed sores ) through physiotherapeutic measures.
Ergotherapeutic measures (e.g. exercises with the hedgehog ball) are used to increase surface sensitivity. In the case of severe or acute disease progression with pronounced impairments such as walking, breathing and/or swallowing disorders, the immune system of the affected person is therapeutically intervened (immunotherapy). Plasmapheresis or intravenously infused immunoglobulins are usually used for this purpose.
In plasmapheresis therapy, the body’s own plasma is replaced with a substitution solution enriched with albumin in order to exchange the immunoglobulins or antibodies responsible for the neurological impairments. As part of an immune adsorption, which is a newer therapy method, only the pathologically active antibodies are removed from the plasma and substituted.
A gentler therapeutic measure are intravenously infused immunoglobulins, which neutralize the responsible endogenous as well as viral and bacterial antibodies and inhibit their synthesis. In addition, immunoglobulins reduce the activity of certain cells of the immune system, the so-called macrophages.
In many cases, intubation or ventilation of those affected is necessary, which can result in respiratory therapy measures. If Guillain-Barré syndrome is life-threatening, a temporary pacemaker may be necessary if bradycardia (slow heartbeat) is present.
Outlook & Forecast
Guillain-Barré Syndrome cannot be completely cured because it is a genetic disease. Only symptomatic treatment can therefore be carried out, which can limit and reduce the symptoms.
In most cases, those affected by the syndrome very often develop inflammation of the lungs, thrombosis and other infectious diseases, so that the life expectancy of the patients is often reduced. This can also lead to breathing disorders or swallowing disorders, so that the patient is dependent on the help of other people throughout his life.
The immune system can be strengthened by injections or by taking medication, which also requires lifelong therapy. Many of those affected are also dependent on a pacemaker to extend life expectancy. With the help of physiotherapeutic exercises, the patient’s everyday life can be made easier in some cases.
Guillain-Barré syndrome often leads to depression or other psychological upsets, so that most patients are dependent on psychological treatment. If the Guillain-Barré syndrome is not treated, the life expectancy of the patient is drastically reduced and there are significant limitations in everyday life.
Since the etiology of Guillain-Barré syndrome is not clear, there are no preventive measures for this disease.
Since Guillain-Barré syndrome is a hereditary disease, the options for aftercare are very limited. Therefore, the early detection and treatment of this disease is paramount. If the person affected by Guillain-Barré syndrome wishes to have children, genetic counseling can also be carried out so that the syndrome is not passed on.
This syndrome may also limit and reduce the life expectancy of the patient. The treatment of the syndrome always depends on the exact severity and type of symptoms. As a rule, the patient is dependent on physiotherapy measures, with many exercises from this therapy being carried out at home.
This usually speeds up healing. The patient’s body must also be protected from various infections and other diseases in order not to unnecessarily burden the immune system. Since the internal organs and the heart are also affected by Guillain-Barré syndrome, regular examinations should be carried out in order to detect damage at an early stage.
Heart surgery may also be necessary. In many cases, contact with other patients with Guillain-Barré syndrome can also be useful, as this leads to an exchange of information.
You can do that yourself
The aim of all self-help measures is to enable those affected to be as independent as possible in everyday life. Outpatient physical and occupational therapy is indicated to prevent loss of mobility. Medical training therapy (MTT) is particularly suitable here.
As part of this training, a training plan is developed that is adapted to the performance of the patient. After a few accompanied therapy units, those affected can carry out these exercises independently. This can be done in special training centers as well as at home using an exercise bike or gymnastics.
Ergotherapeutic treatment makes sense in relation to the sensory disturbances of the upper and lower extremities. Functional disorders of the extremities are also improved as part of this therapy through exercises that can also be adapted to everyday life after instruction. Irrespective of medical training therapy, water aerobics are recommended.
Due to the buoyancy in the water, this is particularly muscle-strengthening. In addition, gait training can be carried out during everyday activities. Changing surfaces, climbing stairs and varying speeds train the sense of balance and the sensitivity of the feet.
Advice on home care and advice on aids is indicated in severe cases. The sudden loss of skills leads to reactive depression in many sufferers. Psychological support in dealing with the illness or self-help groups increase the understanding of the illness and help with the adaptation to everyday life.