Guillain-Barré Syndrome: Causes, Symptoms & Treatment

Guillain-Barré syndrome is an acute inflammation of the peripheral nerves and spinal ganglia (nerve nodes in the spinal canal) with an as yet unexplained etiology (cause). With an incidence of 1 to 2 new cases per 100,000 persons 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 (unclear etiology) polyneuritis with multifocal (occurring in multiple locations) inflammation in the peripheral nervous system. Inflammatory changes, especially in the roots of the peripheral nerves (polyradiculitis) and in the proximal spinal ganglia, cause sensory disturbances, motor paralysis, and autonomic dysfunction. Characteristic are mainly paresthesias (tingling or “formication”) as well as paralyses ascending from the legs, which in combination with respiratory paralysis and/or cardiac arrhythmias can become life-threatening. In addition, in some cases, cranial nerve involvement with paralysis of swallowing and bilateral facial paralysis may be observed. Depending on its course, Guillain-Barré syndrome is differentiated according to different forms, with the most common variant also referred to as acute inflammatory (inflammatory) demyelinating (damaging the medullary sheaths) polyneuropathy.

Causes

The underlying causes of Guillain-Barré syndrome have not been conclusively determined. Primarily immunologic processes are suspected, since Guillain-Barré syndrome occurs in more than half of affected individuals (approximately 60 to 70 percent) following pulmonary or gastrointestinal infectious diseases. Guillain-Barré syndrome is particularly associated with cytomegalovirus, varicella-zoster, measles, Epstein-Barr, mumps, hepatitis, and HIV viruses, as well as certain bacteria such as Salmonella, Brucella, spirochetes, Mycoplasma pneumoniae, or Campylobacter jejuni. In very rare cases, Guillain-Barré syndrome manifests after influenza or rabies vaccinations. It is suspected that antibodies produced by the body as a result of infection are directed against endogenous structures, particularly gangliosides, which are increasingly found in the nervous system, and, in combination with other as yet unknown factors, cause the development of Guillain-Barré syndrome.

Symptoms, complaints, and signs

The symptomatology of Guillain-Barré syndrome depends largely on the appropriate course. Basically, acute and chronic forms of progression are distinguished. In general, Guillain-Barré syndrome is characterized by increasing general weakness due to the destruction of peripheral nerves and spinal nerve roots. The acute form (acute inflammatory demyelinating polyneuropathy or AIDP) begins with back and limb pain, tingling and numbness in the fingers, toes, nose, ear or chin (acras), and paralysis in the legs. In addition, the muscles of the pelvis, trunk and respiratory system become weak, and all reflexes fail. In some cases, certain cranial nerves also fail. This leads to disturbances in respiratory regulation, regulation of heart rate and bladder emptying, and temperature regulation. Furthermore, circulatory disorders occur due to fluctuations in blood pressure. 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 paresthesias of the acras are prominent. Less frequently, the involvement of cranial nerves is observed here. The paralyses increase much more slowly in the chronic form of the disease. They can even be repressed by administration of glucocorticoids. Overall, one third of patients can be cured. Ten percent of patients die. Some of those affected require lifelong care.

Diagnosis and course

Guillain-Barré syndrome is usually diagnosed on the basis of a cerebrospinal fluid analysis (examination of the cerebrospinal fluid). If an increased protein concentration (cytoalbuminous dissociation) is detectable in the presence of a normal cell count, Guillain-Barré syndrome can be assumed. In addition, in Guillain-Barré syndrome, the nerve conduction velocity, which is measured in an electroneurography, is reduced.Electromyography enables statements to be made about possible disturbances in the nerve pathways supplying the muscle fibers. However, this diagnostic procedure is not suitable for early diagnosis of Guillain-Barré syndrome, since the corresponding changes can only be detected after about two weeks. Existing cardiac arrhythmias can be detected by an electrocardiogram, while pulmonary function and blood gas analysis can be used to check respiratory function. In addition, in many cases, antibodies to gangliosides can be detected in the serum. Guillain-Barré syndrome usually has a favorable course, and affected individuals are largely or completely cured within 1 to 6 months.

Complications

As a result of Guillain-Barré syndrome, affected individuals suffer from inflammation of the nerves. In most cases, this inflammation leads to disturbances in sensitivity and paralysis. These do not have to occur over the entire body; the region affected usually depends on the particular nerve that is disturbed. The patient suffers from typical tingling and numbness. Furthermore, most patients experience back pain and pain in the muscles. Furthermore, disturbances in coordination and gait disorders also occur. The patient’s movement is restricted by Guillain-Barré syndrome. In the worst case, paraplegia occurs, in which case the patient is 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 sleep problems. In many cases, the patient’s immune system is also weakened, making it easier for inflammations and infections to occur. Guillain-Barré syndrome can be treated with medication. The earlier treatment occurs, the greater the likelihood of the patient’s complete recovery. Late treatment may result in secondary damage, if necessary, which is usually irreversible and cannot be treated.

When should you see a doctor?

Guillain-Barré syndrome must always be evaluated by a doctor. It can lead to serious symptoms and complications without treatment, 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 may 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. The pain can occur even without movement. Likewise, coordination disorders or gait disturbances often indicate Guillain-Barré syndrome. If the syndrome is not treated, in the worst case it can lead to complete paraplegia of the affected person. This is irreversible and can no longer be treated. When these symptoms occur, a general practitioner should be consulted. He or she can diagnose Guillain-Barré syndrome. However, further treatment depends on the exact symptoms and causes of the syndrome and is then treated by a respective specialist.

Treatment and therapy

In Guillain-Barré syndrome, therapeutic measures correlate with the specific course of the disease. For example, in mild courses, therapy aims to reduce existing paresis (paralysis of the muscles) and minimize the risk for infectious diseases, pneumonia, thrombosis, and contractures (limited mobility of the joints) and decubiti (bedsores) through physiotherapy measures. Occupational therapy measures (e.g. exercises with the hedgehog ball) are used to increase surface sensitivity. In severe or acute courses of the disease with pronounced impairments such as walking, breathing and/or swallowing disorders, therapeutic intervention in the immune system of the affected person (immunotherapy) is performed. Plasmapheresis or intravenously infused immunoglobulins are usually used for this purpose. In plasmapheresis therapy, the body’s own plasma is replaced by a substitution solution enriched with albumin in order to exchange the immunoglobulins or antibodies responsible for the neurological impairments.In the course of immunoadsorption, which is a newer therapeutic procedure, only the pathologically active antibodies are removed from the plasma and substituted. A gentler therapeutic measure is 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 the affected person is necessary, which may entail respiratory gymnastic therapy measures. If Guillain-Barré syndrome has a life-threatening course, a passive pacemaker may be necessary if bradycardia (slowed heartbeat) is present.

Outlook and prognosis

Guillain-Barré syndrome cannot be completely cured because it is a genetic disorder. Therefore, only symptomatic treatment can be given to limit and reduce symptoms. In most cases, the syndrome causes frequent inflammation of the lungs, thrombosis and other infectious diseases, so that the life expectancy of patients is often reduced. Respiratory disorders or swallowing difficulties may also occur, so that the patient is dependent on the help of other people for the rest of his or her life. The immune system can be strengthened by injections or by taking medication, although lifelong therapy is also necessary. Many sufferers are also dependent on a pacemaker to prolong their life expectancy. With the help of physiotherapeutic exercises, the patient’s daily life can be made easier in some cases. Guillain-Barré syndrome not infrequently also leads to depression or other psychological upsets, so that most patients are dependent on psychological treatment. If Guillain-Barré syndrome is not treated, the patient’s life expectancy is drastically reduced and significant limitations in daily life occur.

Prevention

Because the etiology of Guillain-Barré syndrome is not clear, no preventive measures exist for this disease.

Follow-up

Because Guillain-Barré syndrome is a hereditary disease, the options for follow-up care are very limited. Therefore, the focus is also on early detection and treatment of this disease. If the person affected by Guillain-Barré syndrome wishes to have children, genetic counseling can also be performed so that the syndrome is not passed on. Possibly the life expectancy of the patient is also limited and reduced by this syndrome. The treatment of the syndrome always depends on the exact manifestation and the nature of the symptoms. As a rule, the patient is dependent on physiotherapy measures, although many exercises from this therapy can also be performed at home. As a rule, this accelerates the healing process. Likewise, the patient’s body must be protected from various infections and other diseases, so as not to put unnecessary strain on the immune system. Since the internal organs and the heart are also affected by Guillain-Barré syndrome, regular examinations should be performed to detect damage at an early stage. Surgical interventions on the heart may also be necessary. In many cases, contact with other Guillain-Barré syndrome patients may also be useful, as this allows for an exchange of information.

Here’s what you can do yourself

The goal of all self-help measures is to enable affected individuals to achieve the greatest possible independence in everyday life. Outpatient physical and occupational therapy is indicated to prevent loss of mobility. Medical training therapy (MTT) is particularly suitable here. In the course of this training, a training plan is worked out that is adapted to the patient’s ability to perform. After a few supervised therapy sessions, those affected can perform these exercises independently. This can be done in special training centers as well as at home using an exercise bike or gymnastics. Occupational therapy treatment is useful in relation to sensitivity disorders of the upper and lower extremities. Functional disorders of the extremities are also improved within the scope of this therapy by means of exercises that can also be adapted to everyday life after instruction. Independently of medical training therapy, water gymnastics is recommended.Due to the buoyancy in the water, this is particularly muscle-strengthening. This can be supplemented by gait training during everyday activities. Changing surfaces, climbing stairs and varying the speed train the sense of balance and the sensitivity of the feet. Home care counseling and assistive device counseling are indicated for severe progressions. The sudden loss of abilities leads to reactive depression in many affected persons. Psychological support in coping with the disease or self-help groups increase understanding of the disease and help with adaptation to everyday life.