Guillain-Barre Syndrome

Guillain-Barré syndrome: description

In 1916, the three French doctors Guillain, Barré and Strohl first described Guillain-Barré syndrome (GBS). “Syndrome” means that it is a disease characterized by a certain combination of symptoms.

Guillain-Barré syndrome is characterized by ascending paralysis and sensory disturbances, most of which begin in the hands or feet. These failures occur because autoaggressive immune cells attack the insulating sheath of the nerve tracts (demyelination) and also damage the nerve tracts (axons) themselves. In particular, the peripheral nerves and nerve outlets from the spinal cord (spinal nerves) are affected.

The causes of Guillain-Barré syndrome are still largely unclear. However, the disease usually occurs after infection.

The Guillain-Barré syndrome can be divided into seven different subtypes, which differ by the severity of the symptoms and certain laboratory findings. In Europe, the so-called Acute Inflammatory Demyelinating Polyneuropathy (AIDP) the most common subtype. It is characterized by a degradation of the protective sheath, which isolated the nerve tracts (myelin sheath).

Guillain-Barré syndrome: frequency

About one in every hundred thousand people in Germany suffer from Guillain-Barré syndrome every year. The GBS can occur at any age, but it is more common in older age. Men are more likely to be affected by Guillain-Barré syndrome than women.

In about 70 percent of cases, the symptoms return completely within weeks or months. However, about eight percent of those infected die from complications of GBS, such as respiratory paralysis or pulmonary embolism. Guillain-Barré syndrome is more common in spring and autumn, possibly because infections are much more common during these seasons.

Guillain-Barré syndrome: symptoms

Guillain-Barré syndrome usually causes typical signs. However, the first indications of the onset of GBS are nonspecific and are similar to a mild infection. For example, back and body aches occur. In contrast to other diseases such as meningitis, Guillain-Barré syndrome usually causes no fever in the early stages.

In the further course, the actual Guillain-Barré syndrome develops with discomfort, pain and paralysis of the hands and feet. In most cases, these failures are approximately equally pronounced on both sides (symmetrical). Particularly typical are the paralyzes, which can develop within hours to days. These symptoms, which usually begin far from the body, progressively get closer to the body trunk and gradually increase in intensity. The back pain sometimes misleads to the misdiagnosis herniated disc. The pain in Guillain-Barré syndrome is probably caused by the inflammation of spinal cord nerves (spinal nerves).

In the second to third week of illness, the Guillain-Barre syndrome reaches its peak. Thereafter, the symptoms initially remain stable (plateau phase), before slowly regressing within eight to twelve weeks.

It is also possible that the Guillain-Barré syndrome lasts longer and the symptoms do not completely disappear. If the symptoms of GBS persist for more than two months, this chronic form of the disease is also called Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP) designated.

In many patients, so-called cranial nerves are affected by Guillain-Barré syndrome. These nerve pathways emerge directly from the brain and above all control sensory and motor functions in the head and face area. Cranial nerve involvement in Guillain-Barré syndrome is characterized by bilateral paralysis of the seventh cerebral nerve (facial nerve) leading to facial nerve paralysis (facial palsy).

In addition, Guillain-Barré syndrome may affect the autonomic nervous system. This can lead to dysregulation in the function of the circulation and glands (sweat, saliva, lacrimal glands). The normal functioning of the bladder and rectum may also be compromised, leading to incontinence.

Guillain-Barré syndrome: complications

The symptoms of Guillain-Barré syndrome often increase as the disease progresses. They can lead to almost complete paralysis of all muscles. For this reason, Guillain-Barré syndrome can cause serious complications. In the worst case, breathing and the cardiovascular system are severely disturbed. Breathing is impaired in up to 20 percent of the cases (“Landry paralysis”) and may require mechanical ventilation of the patient.

Since sufferers can move increasingly worse, the risk of blood clot formation in the vessels (thrombosis) increases. The clots can break loose, enter the lungs via the heart and block the vessels (pulmonary embolism).

If the signs of paralysis of the muscles last longer, muscle atrophy (muscular atrophy) can occur.

Guillain-Barré syndrome: special forms

The so-called Miller Fisher Syndrome is a special form of GBS disease that particularly affects the cranial nerves. The three main symptoms of this special form are paralysis of the eye muscles, reflex loss and gait disturbances. In contrast to the classic Guillain-Barré syndrome, Miller-Fisher syndrome causes paralysis of the extremities to be less pronounced.

In some cases of Guillain-Barré syndrome, only the autonomic nervous system be affected (acute pandy autonomy). This may result in the disturbances of circulatory function, sweat and saliva secretion as well as bladder and rectal function described above.

Guillain-Barré syndrome: causes and risk factors

So far, it is not clear what causes Guillain-Barré syndrome. Particularly striking, however, is that GBS disease usually occurs after infection. Three-quarters of all patients report having had respiratory or gastrointestinal infection before the onset of Guillain-Barré syndrome. In most cases, GBS begins seven to ten days after infection.

It is assumed that autoaggressive immune cells attack the insulating sheaths of the nerve tracts (myelin sheaths) and thus provoke inflammation of the nerves (polyneuritis). In addition, inflammation-related swelling (edema) of the nerves. In Guillain-Barré syndrome, peripheral nerves (peripheral nervous system) and spinal nerves (spinal nerves) emerge from the spinal cord. The so-called central nervous system, which includes the brain and spinal cord, is less frequently affected.

Guillain-Barré syndrome after infections

It has been proven that Guillain-Barré syndrome is particularly common in infections with certain pathogens. These include infections such as herpes zoster, mumps or even Lyme disease. Campylobacter jejuni, a bacterial agent of gastrointestinal infection, is believed to be the most common trigger of GBS:

During an infection, the body forms antibodies against surface structures of a pathogen. Campylobacter jejuni has structures on its surface that are similar to those of the nerve sheath. It is therefore assumed that the antibodies against the pathogen circulate after surviving the infection in the body and now attack the nerves due to the similar surface structures (“molecular mimicry”). However, only about 30 out of 100,000 people who are infected with this bacterium develop Guillain-Barré syndrome. This assumption of “molecular mimicry” applies equally to other bacteria and viruses.

Guillain-Barré syndrome after vaccinations

In the safety information of vaccinations, Guillain-Barré syndrome is mentioned as a potential side effect within six weeks of vaccination. A GBS as a result of vaccination, however, according to the current state of knowledge is extremely. The benefit of vaccination in any case exceeds the negligible risk of developing Guillain-Barré syndrome.

Guillain-Barré syndrome: examinations and diagnosis

Suspected Guillain-Barré syndrome should immediately a neurological clinic with intensive care be visited. The doctor can already receive important information (history) by describing your symptoms and possible pre-existing conditions. Typical questions the doctor might face if Guillain-Barré syndrome is suspected are:

• Have you been ill for the past four weeks (cold or gastrointestinal infection)?
• Have you been vaccinated in the last few weeks?
• Do you notice signs of paralysis or discomfort on hands, feet, or any other part of the body?
• Do you have back pain?
• Do you take any medicine?

Often, the above-mentioned association is with a gastrointestinal or respiratory disease about two weeks in advance. If muscle weakness or sensitivity disorders appear after such an infection, medical advice should be sought immediately. In a clinic then has to be checked whether in fact the Guillain-Barré syndrome behind it or the symptoms have another cause such as muscle diseases or spinal cord injuries.

Physical examination:

After the medical history follows the physical examination. The doctor tests the sensitivity and muscle strength at various parts of the body. Also, a review of the twelve cranial nerves and the reflexes is part of the physical examination.

Experts have defined criteria that can help diagnose Guillain-Barré syndrome. The three required main criteria are:

• Progressive weakness more than one limb over a maximum of four weeks
• Loss of certain reflexes
• Exclusion of another cause

Further investigations:

In the clinic, after a thorough physical examination, a sample of the Brain-spinal fluid (CSF)) and examined in the laboratory (CSF). This is absolutely necessary in order to confirm the suspicion of Guillain-Barré syndrome and to rule out other causes. To get the nerve water, a very fine needle at the level of the lumbar spine is advanced to the spinal canal and the nerve water is withdrawn with a syringe. The spinal cord already ends above the puncture site, so that it can not be injured. Most people feel that the procedure is not pleasant, but not particularly painful. In nerve water, Guillain-Barré syndrome typically has an increased concentration of protein, while the number of cells in the CSF is normal (cyto-albuminous dissociation). However, this characteristic finding can only be seen seven to ten days after the onset of the disease.

Also important in suspecting a Guillain-Barré syndrome is to prevent the disruption of nerve conduction electrophysiological examinations to examine more closely. For this purpose, for example, the conductivity of nerves can be checked with short electrical impulses (electroneurography). Nerve conduction velocity is typically reduced in Guillain-Barré syndrome because the isolating myelin sheaths are fragmented by the immune cells. But that can only be measured after several days of illness. Therefore, electrophysiological examinations should be repeated regularly during Guillain-Barré syndrome.

in the blood In about 30 percent of cases of Guillain-Barré syndrome, certain antibodies to components of the nerve sheath (for example anti-GQ1b-AK, anti-GM1-AK) can be found. Only rarely is it still possible to determine the causative agent of an infection preceding Guillain-Barré syndrome. In children, this succeeds a little more often than in adults. The pathogen determination usually has no influence on the therapy. If Borrelia or Mycoplasma are detected, antibiotics can be used to eliminate these bacteria.

If the examinations mentioned so far do not give clear results, one can additionally Magnetic Resonance Imaging (MRI) be performed. With the help of this technique very accurate images of the spinal cord and the exiting nerves can be made. The patient is injected with a contrast agent into the vein. It accumulates in the Guillain-Barré syndrome especially in the nerve roots (entry and exit points of the nerve fibers on the spinal cord). In addition, MRI can also rule out that the symptoms are caused by a herniated disc.

At four to eight-hour intervals in patients with Guillain-Barré syndrome muscle strength and general parameters of the Cardiac and respiratory function be checked. Especially in the elderly or a rapid progression of the symptoms must be closely monitored. Doctors pay particular attention to possible complications such as respiratory paralysis (Landry paralysis) and pulmonary embolism.

Guillain-Barré syndrome: treatment

Depending on the severity of the symptoms, Guillain-Barré syndrome treatment takes place in an intensive care unit. While this is not necessary in lighter cases, monitoring at a normal hospital ward is almost always essential. Indeed, Guillain-Barré syndrome can lead to life-threatening paralysis. Particularly in the case of respiratory disorders, the cardiovascular system or the swallowing reflex, the patient must be examined and observed at regular intervals. Life-threatening situations can occur quite suddenly and require rapid treatment. For example, in severe Guillain-Barré syndrome, physicians must be constantly prepared for serious cardiac arrhythmias or artificial respiration. Such ventilation is needed in about 20 percent of cases temporarily.

A causal therapy of GBS is unknown. In more severe cases, immunomodulatory therapy is useful. The patients become so-called immunoglobulins administered. It is a mixture of antibodies that interact with the autoaggressive antibodies to normalize the immune response.

An equivalent therapeutic alternative in Guillain-Barré syndrome is the so-called Plasma exchange (plasmapheresis), The blood of the patient is passed through a machine in which membranes are located, as in dialysis. These membranes filter out the harmful autoaggressive antibodies. This prevents further damage to the nerve structures. However, since the antibodies can also be formed again, the plasmapheresis is often repeated several times.

A combination of the administration of immunoglobulins and the plasma exchange is not recommended at the moment.

Patients with chronic GBS disease may cortisone be administered. In acute Guillain-Barré syndrome, however, the drug is not effective.

If many muscles are affected by the paralysis and therefore the patient can not move sufficiently, in any case with the help of so-called heparin the formation of blood clots must be prevented (thromboprophylaxis). For this purpose, a syringe is usually administered once a day subcutaneously (under the skin). It is also very important to have an accompanying one as soon as possible physical therapy to assist the body in maintaining mobility and promote rapid recovery.

Some patients with Guillain-Barré syndrome are particularly frightened by their disease, especially because of paralysis. Basically, however, for the absolute majority of cases, these unpleasant symptoms completely regress. However, if the GBS disease leads to a severe mental stress due to the unpredictable course, intensive care of the patient (for example by psychotherapy) makes sense. If the anxiety is particularly great, it can be reduced with the help of drugs.

Guillain-Barré syndrome: disease course and prognosis

During the plateau phase of GBS, the movement restrictions and other symptoms are usually severe. However, the further course of the disease turns out to be favorable in the vast majority of patients: The symptoms are completely reversed in about 70 percent of those affected. However, full recovery can take many months. In some cases, the regression of the complaints may also be incomplete. One year after the illness, one third of the patients still complain of pain. Around 20 percent of those affected are permanently disabled. In total, around eight percent of those affected die.

For a majority of those affected the disease means a restriction or conversion of their previous life. Severe long-term complications due to respiratory and cardiovascular problems are possible. The long lying can also cause blood clots, which close the vessels (leg vein thrombosis, pulmonary embolism). Children and adolescents rarely have long-term damage, although minor disorders may persist. For this reason, the course in children is usually cheaper.

The Guillain-Barre Syndrome can occur repeatedly and transitions into a chronic course form are still possible years later.