Guillain-Barré Syndrome (GBS) and CIDP • dangerous consequences after infection

The most common cause of generalized sudden paralysis is Guillain-Barré syndrome (GBS). Children can get it as well as adults. GBS is particularly common in young adults and people between the ages of 50 and 70. Around one to two in 100,000 people develop it every year.

The Guillain-Barré syndrome was named after the French doctors Georges Charles Guillain and Jean-Alexandre Barré, who first described the disease in 1916. Another name for GBS is acute inflammatory demyelinating polyneuropathy (AIDP – "suddenly occurring inflammatory nerve membrane damaging nerve disease"). If the symptoms of the disease do not subside after a few weeks and also develop more slowly overall, doctors speak of chronic inflammatory demyelinating polyneuropathy (CIDP).

At a glance:

Corona: long-term and long-term consequences of COVID-19

Cause: Guillain-Barré syndrome after infections

Why some people get Guillain-Barré syndrome is unclear. In around two thirds of those affected, GBS occurs after an infection of the respiratory tract or the digestive tract. This has usually already subsided when the typical symptoms of paralysis of GBS are noticed around one to four weeks later.

Triggers for Guillain-Barré Syndrome:

In very rare cases, Guillain-Barré syndrome can also occur after an influenza vaccination – but the positive effects of the vaccination outweigh this. When infected with SARS-CoV-2, some sufferers complain of paralysis of their limbs after five to ten days. If an undersupply of oxygen is noticed at the same time, doctors must carefully check whether this is due to a lung attacked by COVID-19 or a muscle weakness as a result of the nerve disease.

In Guillain-Barré syndrome, immune cells attack the covering of the body's own nerve pathways in a misdirected autoimmune reaction. This causes them to ignite and no longer transmit stimuli well.

Symptoms: GBS has difficulty climbing stairs

Those affected often notice GBS for the first time when climbing stairs or when getting up from a chair. Her feet no longer support her and the walk becomes unsteady. The strength in both legs is missing. Fever, however, usually does not occur.

Other typical symptoms of Guillain-Barré syndrome are:

• Tingling and sensory disturbances in the legs, less often in the arms
• Back pain
• Pain in legs and arms, paresthesia
• Motion control issues
• Swallowing disorders
• Disruption of sweat production
• Paralysis of the facial muscles
• Breathing problems
• Indigestion
• Dizziness, circulatory problems

Reflexes are absent or only weakly developed

The condition of those affected usually worsens within a few days. The paralysis becomes more and more severe and spreads towards the middle of the body. It becomes dangerous when organs such as the heart or lungs are affected by paralysis. It is therefore recommended that patients with severe Guillain-Barré syndrome be closely monitored in an intensive care unit. However, there are also those affected who can walk for the entire duration of the illness and take care of themselves.

Usually the symptoms get worse and worse for three to six weeks until the condition stabilizes ("plateau phase"). Usually an improvement begins afterwards. The organs start working again and the muscles gradually develop strength again.

Guillain-Barré syndrome: diagnosis by spinal tap

Anyone suffering from significant signs of paralysis in the legs should quickly visit a neurological clinic with an intensive care unit. In the anamnesis interview, the doctor asks about the exact symptoms and how they have changed. He also asks whether the person affected has had an infection in the past few weeks or has been vaccinated. This is followed by physical examinations to rule out other diseases.

With a spinal puncture (lumbar puncture), some fluid (liquor) is removed from the spinal cord with a long needle on the lumbar spine. In the laboratory, this is mainly examined for its protein values. These are significantly increased in Guillain-Barré syndrome, but the protein level only increases in the course of the disease. With an early examination it may still be normal.

Electrophysiological examination in GBS

The doctor uses electromyography to test the conductivity of the nerves. To do this, he inserts a fine needle into a muscle to record the electrical activity of the muscle. Each time the muscle contracts, an electrical impulse is generated. The doctor can use the pattern recorded from the muscle during electromyography to identify the problem. In addition, targeted small electrical stimuli on the surface of the skin cause the muscles to contract. A device measures the time it takes for this stimulus to be transmitted to the target muscle (nerve conduction speed). It is typically reduced in GBS.

Treatment: GBS often requires artificial ventilation

If an affected person shows only slight signs of paralysis, he is mainly observed in the hospital and receives additional physical therapy. During this time, a GBS patient must be examined closely. There is a great risk that the heart and lungs will be affected by muscle weakness and will no longer be able to perform their function adequately. Lung volume and muscle strength are checked every eight hours at the latest. As soon as the muscle strength decreases and the lungs or the muscles of the larynx and throat are affected, treatment in the intensive care unit is necessary. Around 20 to 30 percent of those affected have to be artificially ventilated at some point. Specialists recommend intubation when the oxygen saturation in the blood drops and the carbon dioxide increases at the same time.

At the same time, the heart's activity must be monitored. If doctors fear arrhythmias, they put on a pacemaker as a preventive measure. In most cases, artificial or intravenous feeding is also necessary. In order to avoid bedsores (bedsores), sick people must be repositioned regularly.

In view of the frightening symptoms and intensive medical treatment, it is important that the patient is given good psychological care. His courage to face life and his conviction that he can conquer the disease are decisive for the success of healing. Accompanying physiotherapy also helps in the long term because the joints and muscles are trained. Painkillers are given if necessary.

GBS: blood purification or antibodies if the course is severe

In the moderate to severe course of Guillain-Barré syndrome, there are two treatment methods that usually bring relief: plasma exchange or the administration of antibodies (immunoglobulins). Both methods are considered to be approximately equally effective, but may not be combined with one another. Glucocorticosteroids do not help with GBS.

During plasma exchange (plasmapheresis), the patient's blood plasma is cleaned of antibodies and immune complexes that attack the nerves. The blood is then returned to the patient's body.

During treatment with immunoglobulins, the sick person is given antibodies that inhibit the body's own immune response. This is to stop the body's own antibodies from attacking the nerves.

Special forms of CIDP, Miller-Fisher syndrome and AMAN

While the symptoms of acute Guillain-Barré syndrome noticeably worsen within a few days, development of chronic inflammatory demyelinating polyneuropathy (CIDP) is much slower. An unsteady and wide-legged walk is often the only symptom for a long time. Later, the symptoms are similar to GBS, but are often accompanied by great tiredness or tremors. In contrast to GBS, there is no improvement after a few weeks. Instead, the affected person's condition worsens gradually or in attacks.

The administration of immunoglobulins in conjunction with corticosteroids has proven effective in the treatment of CIDP. An exchange of the blood plasma is only recommended if the other two therapies do not work. Immunosuppressants can also be given or an autologous stem cell transplant can be used.

In the rare Miller-Fisher syndrome, Guillain-Barré syndrome primarily affects the cranial nerves. As a result, the eye muscles in particular are paralyzed, the reflexes are missing and the movement coordination is poor. However, legs and feet feel largely normal.

Acute motor axonal neuropathy (AMAN) is a variant of GBS that damages motor nerves. Therefore, a rapidly progressing muscle weakness with a lack of reflexes is noticeable.

How long does Guillain-Barré Syndrome last?

Anyone suffering from Guillain-Barré syndrome needs patience. On average, it takes between 40 and 70 days for the symptoms to subside. It is true that only about 15 percent of those affected get all physical abilities back completely; but for around two-thirds of those affected, the long-term consequences hardly affect their everyday life.

Those who suffer from CIDP have a significantly worse prognosis: only every third to seventh CIDP patient fully recovers from their limitations. Between two and six percent of those affected die. Long bed rest increases the risk of thrombosis or pulmonary embolism.

The faster the Guillain-Barré syndrome progresses and the older the person affected, the greater the risk of permanent damage. Around ten to fifteen percent of those affected cannot walk freely again after a year. Typical long-term consequences of GBS are excessive exhaustion (fatigue), cardiovascular problems, disturbance of sweat production or digestive problems. Some recovered people find it difficult to lift their feet even later. It is important to train the muscles and joints in the later stages of the Guillain-Barré syndrome in order to maintain their functionality.

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