In recent years, more attention has been paid to muscle diseases. A rare muscle disease is Guillain-Barré syndrome. This causes paralysis and loss of sensation, among other things. Anyone can be affected by this disease. Guillain-Barré syndrome is a rare muscle disease. In the Netherlands, the disease is diagnosed in 1 in 100,000 people every year. Guillain-Barré syndrome is also sometimes called Landry-Guillain-Barré-Strohl syndrome, which better explains the abbreviation GBS (by which the disease is also known).
The disease is named after George Charlais Guillain and Jean-Alexandre Barré, two doctors who worked in the neurological center of the French army during the First World War. There they are confronted with soldiers who are suddenly paralyzed and have reduced reflexes. To the great surprise of the doctors, the patients recover from their paralysis and can leave the hospital on their own after a while.
Who gets this disease?
Anyone can be confronted with this muscle disease: both young and old, both men and women. The disease is not hereditary and not congenital. The severity of the symptoms can vary greatly: one patient has only a minor loss of muscle strength, while another is almost completely paralyzed. Fortunately, almost everyone does recover, even though this can sometimes take a few years. Unlike other muscle diseases such as ALS, Guillain-Barré syndrome is not fatal.
What causes Guillain-Barré?
Guillain-Barré syndrome is caused by an autoimmune reaction, that is, a reaction of the patient’s immune system. The immune system sees its own nerve pathways as foreign material and tries to fight it. This ‘battle’ damages the insulating material of the nerve (myelin) and, in more serious cases, the enclosed conductive part (axon). This makes the nerve cells less able to transmit signals: signals arrive delayed or changed.
How does the immune system come to attack its own nerve pathways? This is usually caused by a bacterial infection, such as Campylobacter jejuni. This bacterium is found in, among other things, undercooked chicken meat. Viral infections can also cause GBS. In rare cases, Guillain-Barré occurs after surgery or a vaccination.
Complaints and symptoms
The signs and symptoms of Guillain-Barré develop quite suddenly and can become intense from that moment on. Possible symptoms are:
- Numbness;
- Tingling;
- Shocks and/or vibrations in feet or hands;
- A sleeping feeling;
- Nerve pain
If the motor nerves are affected:
- Muscle weakness;
- Cramps;
- Paralysis.
The paralysis almost always starts in the legs, but can also spread to other parts of the body, such as the arms, facial muscles and even the respiratory muscles and/or the heart muscle. This sometimes makes patients unable to speak or breathe freely. In severe cases, respiratory support may be required. Patients diagnosed with Guillain-Barré are admitted to hospital and usually placed in Intensive Care at a fairly early stage, because it is impossible to predict in advance how (severely) the disease will progress. In the ICU department, all the patient’s bodily functions can be continuously monitored.
Therapy
During admission, protective proteins, or immunoglobulins, are administered by infusion. In severe cases, even the liquid portion of the patient’s blood can be replaced. This treatment is called plasmapheresis. The oxygen level in the blood is also closely monitored.
After about 4 weeks the disease has usually passed its peak. Patients can then start a rehabilitation program so that they learn to use their muscles optimally again. 80% of patients eventually recover completely within three to six months after the onset of Guillain-Barré. Unfortunately, the remaining 20% continue to suffer from minor residual symptoms, such as loss of muscle strength or loss of sensation. The disease also sometimes comes back: this affects approximately 3% of patients.
