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Most often it begins with the fact that after a usually mild infection of the respiratory or digestive system, sensory disturbances in the fingers or toes appear. However, it may end up under a respirator, and in every twentieth case – death. Fortunately, early diagnosis and appropriate treatment allow for complete recovery in 80% of cases. What disease are we talking about?
Guillain-Barré syndrome – causes
Guillain-Barré syndrome is a rare condition (incidence 1-4 / 100 / year) that leads to progressive weakness in the muscles due to damage to the peripheral nerves. It turns out that the antibodies are to blame. Antibodies are molecules produced by white blood cells that are designed to recognize and destroy foreign particles. Their main function is to fight viruses and bacteria that enter our body. In Guillain-Barré syndrome, the antibodies react abnormally and instead of defending the body, they begin to damage the nerve sheaths, leading to the innervation disorders that underlie the symptoms.
The causes of this abnormal reaction remain unclear, but the theory is that it is because some viral and bacterial infections cause antibodies that cross-react with molecules that make up the nerve sheath. They not only destroy viruses and bacteria against which they are directed, but also “by mistake” damage the body’s own tissues. Indeed, almost 2/3 of Guillain-Barre syndrome cases are preceded by an infection. These are usually mild viral, sometimes also bacterial, respiratory or digestive infections.
In recent years, attempts have been made to identify germs causing infections that may be complicated by Guillain-Barre syndrome. The blacklist includes: Campylobacter jejuni – a bacterium that causes diarrheal gastrointestinal infections, cytomegalovirus or Epstein-Barr virus. Less compelling scientific evidence links Guillain-Barré syndrome to mycoplasma, HIV, herpes, hepatitis A, B, C, influenza, and bacteria: Haemophilus influenzae or Escherichia coli. The list of suspected microorganisms is long, but in 1/3 of the cases it is often impossible to find any specific factor causing the disease. It is believed that the disease may be related to certain vaccinations (there are some indications of a link between the disease and the swine flu and meningococcal vaccine), trauma, and bone marrow transplants. It is also – and it is worth remembering – the first symptom of a neoplastic disease.
Guillain-Barré syndrome – symptoms
Most often, about 2-4 weeks after a usually mild respiratory or digestive system infection, sensory disturbances in the fingers or toes appear (they are the result of involvement of the sensory nerve fibers). This is accompanied by a gradually increasing, symmetrical weakness in the muscles of the legs, leading to difficulties in walking and, over time, to complete paralysis. Over the following hours, and sometimes days, the muscles of the torso (including the respiratory muscles), hands and face (including the muscles that move the eye) are involved. This phenomenon is sometimes called “ascending muscle paralysis”, resulting from the involvement of motor nerve fibers. During the physical examination, the doctor finds that deep reflexes (e.g. the knee reflex) have disappeared or weakened. Severe pain may appear in the lumbar or interscapular region. It also happens that the nerve fibers responsible for regulating the work of internal organs are seized. Then symptoms such as arrhythmias (potentially fatal), rapid increases and drops in blood pressure, increased drops in blood pressure when rising, or urinary retention appear. Due to the paralysis of the respiratory muscles, preventing proper breathing and leading to life-threatening respiratory failure, about 1/3 of patients require connection to a ventilator and mechanical ventilation.
In addition to the typical course of the disease, the diagnosis also requires the examination of the cerebrospinal fluid, which is collected during the lumbar puncture. Treatment is carried out depending on the severity of the disease in neurological or intensive care units. It consists in administering immunoglobulins or “filtering” the plasma from antibodies circulating in it during plasmapheresis procedures.
Guillain-Barré syndrome – treatment
With successful treatment, about 80 percent of patients are completely cured. Unobtrusive gait or balance disturbance may remain. However, according to statistics, about 3 percent of patients end up in a wheelchair. Some scientists believe that this is what happened in the case of US President Franklin D. Roosevelt. In recent years, a large body of evidence has accumulated showing that the cause of the president’s paralysis, which was initially thought to be poliomyelitis, was the Guillain-Barry syndrome. Unfortunately, every twentieth patient with Guillain-Barry syndrome dies despite intensive care. The prognosis is particularly poor for patients who require mechanical ventilation. Of them, even one in five dies. The main causes of death are acute respiratory failure, infectious complications including sepsis, pulmonary embolism, and arrhythmias and cardiac arrest.
Text: lek. Paulina Jurek