Guillain-Barré syndrome: what it is, symptoms and treatment of GBS, causes

Introduction

The first description of Guillain-Barré syndrome was carried out by Guillain, Barré and Strohl in 1916, while much was done at that time to understand the pathophysiology and therapy of this very dangerous disease.

Meanwhile, early detection and treatment of this rare disease (1-2 cases per 100,000 inhabitants) reduced mortality from 50% to less than 8%. Therefore, it is a very treatable disease if it is detected and treated in a timely manner.

The disease can occur at any age, but the incidence rate increases with age, i.e. mostly adults are sick. Men suffer more than women. The course of the disease is influenced by many factors, some of which have regional (Europe, Asia, USA) characteristics.

What is Guillain-Barré Syndrome?

Guillain-Barré Syndrome (GBS, acute polyradiculoneuritis) is a rare disease of the nervous system in which a person's immune system attacks its own peripheral nerves.

The disease affects from 1 to 2 out of 100,000 people every year. Symptoms include muscle weakness or paralysis of the limbs, face, and respiratory system. The condition can be fatal, but most people with the syndrome will make a full recovery.

Acute polyradiculoneuritis can occur in anyone, but men are more likely to get sick than women. The incidence increases with age, and most common between the ages of 50 and 74.

Causes Guillain-Barré syndrome

Guillain-Barré Syndrome is an autoimmune disorder in which the body's immune system attacks its own tissues.

With Guillain-Barré syndrome immune cells attack the myelin sheath - a fatty substance that covers nerve fibers. The myelin sheath insulates and protects nerve fibers and facilitates the transmission of electrical impulses throughout the nervous system. If the myelin sheath is damaged, the transmission of impulses from the brain can be slow or completely blocked.

What causes the autoimmune reaction that leads to Guillain-Barré syndrome is unknown. but in about 60% of cases the blockage is preceded by a viral respiratory infection or gastrointestinal upset. The disease can be triggered by Campylobacter infection, which causes diarrhea, Epstein-Barr virus, which causes glandular fever, HIV, which is the virus that causes AIDS, and cytomegalovirus (CMV).

It is believed that these viruses can alter the nature of nerve fibers so that they appear foreign to the immune system. In rare cases, Guillain-Barré syndrome can be caused by surgery or immunization.

Guillain-Barré syndrome is described as polyradiculoneuritis, which means inflammation of many nerves. In particular, she affects peripheral nerves - those that connect the brain and spinal cord to the muscles and skin. There are a number of different subtypes of Guillain-Barré syndrome, each of which affects the body in slightly different ways.

Subtypes of GBS

In recent years, subtypes of the classic Guillain-Barré syndrome (GBS) have been identified, which differ in clinical presentation and history. The SGB is currently divided into the following options:

• The most common form in Europe is acute inflammatory demyelinating polyneuropathy (AIDP) (60-90% of cases).
• In addition, there are acute primary axonal motor sensory neuropathy (OPAMN) and acute axonal motor neuropathy (OMAN). Both of the latter are found only in 5-10% of cases.
• An even rarer variant is Miller-Fisher syndrome, which is GBS with predominantly cranial nerve involvement. In some cases, the acute clinical picture of GBS turns into a chronic form, which is called chronic inflammatory demyelinating polyneuropathy (CIDP)

Signs and symptoms

Guillain-Barré syndrome is an acute illness and has a rapid onset compared to other neurological conditions. Signs and symptoms may develop within hours and days or after 3-4 weeks.

The first symptoms are usually tingling (as if with pins and needles) or numbness, starting first with the legs and passing into the hands, and sometimes into the face. This is usually accompanied by varying degrees of muscle weakness in the legs and then arms and face. A person may have difficulty holding or gripping objects, and a heaviness can be felt in his limbs. These symptoms may be vague at first, but tend to progress rapidly.

As the condition progresses, the following signs and symptoms may appear:

• progressive and extreme muscle weakness in the limbs;
• decrease or absence of tendon reflexes;
• labored breathing;
• muscle and joint pain;
• paralysis of the eye muscles.

The severity of symptoms can vary greatly from person to person. Symptoms can range from mild muscle weakness that resolves quickly to complete muscle paralysis.

About 60% of people will be unable to walk once the disease progresses. Respiratory function is impaired in about 50% of cases. In 30% of cases, the respiratory muscles are paralyzed to such an extent that artificial respiration (artificial lung ventilation) is needed to maintain breathing. In addition, the fluid balance in the body may be unstable, blood pressure can fluctuate, a heart rate cuts may become irregular.

Symptoms appear in the worst case two to three weeks after the onset of the disease. During this stage, there is usually a period of time when symptoms remain stable. This phase is called "plateau" and can last from several days to several weeks.

Diagnostics

Diagnosis can be difficult in the early stages of the condition due to the uncertainty of the initial symptoms. Diagnosis may be easier if the onset is rapid and the same on both sides of the body. To diagnose Guillain-Barré syndrome, your doctor will do the following:

• complete medical history;
• questioning current symptoms;
• physical examination, for example: sensation, reflexes, and muscle strength;
• blood tests.

Nerve conduction studies may also be recommended. They measure the speed at which electrical impulses travel through the nervous system. In Guillain-Barré syndrome, impulses travel more slowly than usual.

A lumbar puncture may also be recommended. It involves inserting a needle into your back and taking a sample of the fluid that surrounds the brain and spine (cerebrospinal fluid). In Guillain-Barré syndrome, the concentration of proteins and immune cells in this fluid is likely to be increased.

Guillain-Barré Syndrome Treatment

There is no cure for Guillain-Barré syndrome. Treatment is largely supportive and focused on minimizing symptoms and preventing complications.

Hospitalization is usually necessary at the initial stages, since the course of the disease can be unpredictable. A medical team from a rheumatologist, neurologist, doctors of many other specialties, nurses, physiotherapists will participate in the treatment.

At an easy stage, it is important recreationyou may need medicines to reduce pain. When the condition becomes more severe, treatment goals are to:

• maintaining breathing;
• reduction of pain;
• return of muscle tone;
• preventing complications of paralysis such as contractures (tightening and thickening of tendons).

Blood pressure, fluid balance, heart rate and rhythm will also be closely monitored.

Plasma exchange (plasmapheresis)

This procedure involves drawing blood, which is then separated into plasma and red blood cells. Plasma is discarded, and red blood cells are returned to the body with donor or "clean" plasma. This process can be repeated several times a day for 2-3 days.

Plasmapheresis is thought to remove antibodies from the body's plasma, which may contribute to an autoimmune attack on the nervous system.

Immunoglobulin (gamma globulin) therapy

It is a special protein that is naturally used by the immune system. When given in high doses intravenously (through an IV drip into the bloodstream), it is believed to reduce the autoimmune attack on the nervous system.

Forecast and recovery

The time it takes to recover from Guillain-Barré syndrome varies greatly. Recovery from illness can take weeks or months, with an average recovery time of three to six months.

The prognosis for most people will be full recovery, while 20-30% of people will have weakness and discomfort. In some cases, small improvements can last for one to two years after the initial recovery period.

Approximately 5% of cases of Guillain-Barré syndrome are fatal. This usually occurs due to heart rhythm problems, but it can also be due to respiratory failure, pulmonary embolism (blood clots in the lungs), or infection.

Mortality

A 2008 epidemiological study found that the mortality rate is 2-12%despite being treated in an intensive care unit, although in tertiary care centers this rate may be less 5% with a team of healthcare professionals familiar with GBS management.

Causes of deathassociated with Guillain-Barré syndrome include acute respiratory distress syndrome (ARDS), sepsis, pneumonia, venous thromboembolic disease and cardiac arrest. In most cases, mortality is due to severe autonomic instability or complications of prolonged intubation and paralysis. The leading cause of death in elderly patients with GBS is arrhythmia.

Mortality from GBS increases markedly with age. In Europe, the case fatality rate ranges from 0.7% in those under 15 to 8.6% in those over 65. Survey data showed that patients aged 60 years and older have a 6 times higher risk of death than people aged 40–59 years, and 157 times higher than patients younger than 15 years old. Although mortality increases with age in men and women, after 40 years, mortality in men is 1.3 times higher than in women.

Mortality associated with GBS usually occurs in patients with mechanical ventilation (ALV) due to complications such as pneumonia, sepsis, acute respiratory distress syndrome and, less commonly, autonomic dysfunction.

Major lung diseases and the need for mechanical ventilation increase the risk of death, especially in elderly patients.