What is it ?
Guillain-Barré syndrome (GBS), or acute inflammatory polyradiculoneuropathy, is an autoimmune disease that causes peripheral nerve damage and paralysis. This paralysis is called extensive because it usually begins with the legs and arms and extends to the rest of the body. Its causes are many, but the syndrome most often occurs after an infection, hence its name of post-infectious acute polyradiculoneuropathy. Every year in France, 1 to 2 people out of 10,000 are affected by the syndrome. The majority of sufferers recover completely in a few months, but the syndrome can leave significant sequelae and, in rare cases, lead to death, most often by paralysis of the respiratory muscles.
Symptoms of Guillain-Barré syndrome
Tingling and foreign sensations appear in the hands and feet, often symmetrically, and spread through the legs, arms and the rest of the body. The severity and evolution of the syndrome are very variable, from simple muscle weakness to paralysis of certain muscles and, in severe cases, to almost complete paralysis. 90% of patients experience maximal systemic involvement during the third week following the first symptoms. In severe forms, the vital prognosis is engaged because of the involvement of the muscles of the oropharynx and the respiratory muscles, posing the risk of insufficiency and respiratory arrest. The symptoms are quite similar to those of other conditions such as botulism (link) or Lyme disease.
The origins of the disease
Following an infection, the immune system produces autoantibodies that will attack and damage the myelin sheath surrounding the nerve fibers (axons) of the peripheral nerves, preventing them from transmitting electrical signals from the brain to the muscles.
The cause of Guillain-Barré syndrome is not always identified, but in two-thirds of cases it occurs a few days or weeks after diarrhea, lung disease, influenza … the bacterium Campylobacter (responsible for intestinal infections) is one of the main risk factors. Much more rarely, the cause may be vaccination, surgery or trauma.
Risk factors for Guillain-Barré syndrome
The syndrome affects men more frequently than women and adults than children (the risk increases with age). Guillain-Barré syndrome is neither contagious nor hereditary. However, there may be genetic predispositions. After long controversy, researchers have confirmed that Guillain-Barré syndrome can be caused by Zika virus infection.
Prevention and treatment of Guillain-Barré syndrome
Two immunotherapy treatments have been shown to be effective in stopping nerve damage:
- Plasmapheresis, which consists of replacing the blood plasma containing the autoantibodies that attack the nerves with healthy plasma.
- Intravenous injection of antibodies (intravenous immunoglobulins) that will neutralize autoantibodies.
They require hospitalization and will be more effective if they have been implemented early enough to limit the damage to the nerves. Because when the nerve fibers protected by the myelin sheath are themselves affected, the sequelae become irreversible.
Irregularities in breathing, heart rate and blood pressure should be given special attention and the patient should be ventilated if paralysis reaches the respiratory system. Rehabilitation sessions may be necessary to recover full motor skills.
The prognosis is generally good and all the better as the patient is young. Healing is complete after about six to twelve months in about 85% of cases, but about 10% of sufferers will have significant sequelae. The syndrome causes death in 3% to 5% of cases according to WHO, but up to 10% according to other sources. Death occurs from cardiac arrest, or due to complications of long-term resuscitation, such as nosocomial infection or pulmonary embolism .