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ID: 126
Category: Autoimmune Disease
CreatedBy: 1
UpdatedBy: 1
createdon: 02 Feb 2023
updatedon: 04 Apr 2023

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Author: Khoa Tran
Published Feb 02, 2023
Updated Apr 04, 2023

Table of contents

Guillain-Barré Syndrome (GBS)

Etymology and Pronunciation

Guillain-Barré syndrome is named after the two physicians who first described it, Jean-Baptiste Octave Landry and Georges Charles Guillain, and Jean-Alexandre Barré, who independently published cases of the same condition.

History of Guillain-Barré Syndrome (GBS)

The history of Guillain-Barré syndrome (GBS) can be traced back to the mid-19th century when Landry, a French physician, observed a case of rapidly progressive paralysis in a young woman. However, it was not until 1916 that Landry's work was published, describing the characteristic features of the syndrome, including muscle weakness and loss of reflexes.

In 1917, during World War I, Guillain, a French army physician, encountered similar cases of acute flaccid paralysis among soldiers. He published a series of cases in 1916 and 1917, which caught the attention of the medical community. Guillain emphasized the association of the syndrome with a preceding infection, which later became one of the hallmarks of GBS.

In 1918, Barré, a French neurologist, independently reported cases of acute flaccid paralysis with similar features, and he also recognized the association with preceding infections. Barré's contribution was significant in identifying the involvement of the peripheral nervous system in the disorder.

Over the years, other physicians and researchers furthered the understanding of GBS. In the 1930s, the use of spinal fluid examination became a valuable diagnostic tool, and in the 1950s, the use of electrophysiological studies helped confirm the involvement of peripheral nerves in GBS. In the 1980s, the discovery of elevated levels of certain antibodies in the blood of GBS patients provided further insights into the underlying immune-mediated mechanism of the disorder.

Today, GBS is recognized as an autoimmune condition where the immune system attacks the peripheral nerves, leading to inflammation and damage.

Modern Understanding of Guillain-Barré Syndrome (GBS)

Guillain-Barré syndrome (GBS) is a rare autoimmune disorder that affects the peripheral nerves, leading to numbness, weakness, tingling, and in severe cases, muscle paralysis. This condition can cause respiratory failure and requires prompt medical attention. The cause of GBS is not completely understood, but it is thought to occur when the body's immune system mistakenly attacks the peripheral nerves, leading to inflammation and damage to the nerve fibers.

GBS is a rapid onset condition that can occur after an infection, such as Campylobacter jejuni infection, influenza, Epstein-Barr virus (EBV), Cytomegalovirus (CMV), Herpes simplex virus (HSV), or Mycoplasma pneumoniae. It can also occur after vaccinations for diseases such as tetanus, diphtheria, polio, and Haemophilus influenzae type b (Hib).

There are several types of GBS, including acute inflammatory demyelinating polyneuropathy (AIDP), Miller Fisher syndrome (MFS), acute motor axonal neuropathy (AMAN), and acute motor-sensory axonal neuropathy (AMSAN). In some cases, anti-ganglioside antibodies are present in the blood, indicating an autoimmune cause of the disorder.

GBS can cause a range of symptoms, including muscle weakness and tingling or numbness in the extremities, which can progress to complete paralysis. The symptoms can appear suddenly and can get worse over a period of days or weeks. Other symptoms can include difficulty swallowing, breathing problems, and changes in heart rate or blood pressure.

Diagnosis of GBS can be challenging, as the symptoms can resemble other neurological conditions. However diagnostics is done through a combination of medical history, physical examination, electrodiagnostic tests, and nerve conduction studies. Blood tests and nerve biopsy may also be performed.

Causes of Guillain-Barré Syndrome (GBS)

The exact cause of GBS is not fully understood, but it is thought to involve a combination of genetic, environmental, and immune factors.
- Infection: In many cases, GBS is triggered by an infection, most commonly by certain types of bacteria or viruses. The immune response triggered by the infection can sometimes go awry and attack the peripheral nerves, leading to inflammation and damage. It is thought that the immune system's response to the infection may cross-react with the nervous system, leading to an autoimmune reaction. Some of the common infections that have been associated with GBS include respiratory infections, such as the flu or pneumonia, gastrointestinal infections, such as Campylobacter jejuni, and viral infections, such as cytomegalovirus (CMV), Epstein-Barr virus (EBV), and Zika virus.
- Genetic predisposition: There may be a genetic predisposition to developing GBS, as some studies have suggested that certain genetic factors may increase the risk of developing the condition. However, more research is needed to fully understand the genetic factors involved in GBS.
- Other triggers: Other potential triggers for GBS include recent vaccination (although this is rare), surgery, trauma, and other factors that may trigger an immune response. However, these triggers are not always present in all cases of GBS, and their relationship with GBS is still not fully understood.
- Immune system dysfunction: Dysfunction of the immune system, such as an overactive immune response or an inability to properly regulate the immune response, may play a role in the development of GBS. This can lead to the immune system attacking the peripheral nerves, resulting in inflammation and damage.
- Molecular mimicry: Molecular mimicry is a theory that suggests that certain infectious agents may have proteins that closely resemble proteins in the nervous system. The immune response triggered by the infection may then mistakenly target the nervous system, leading to an autoimmune reaction in GBS.

Treatments for Guillain-Barré Syndrome (GBS)

Treatment for GBS typically involves supportive care, such as physical therapy, occupational therapy, speech therapy, and artificial respiration. In severe cases, mechanical ventilation or tracheostomy may be necessary. Plasmapheresis and intravenous immunoglobulin (IVIG) can help reduce inflammation and improve nerve function. Corticosteroids may also be used to reduce inflammation.

Prevention of GBS includes avoiding infections that can trigger the autoimmune response, such as Campylobacter jejuni, and getting vaccinated against diseases such as tetanus, diphtheria, polio, and Hib. Pneumonia vaccination may also help reduce the risk of GBS. In some cases, GBS post-exposure prophylaxis may be recommended.

GBS is a serious condition that can have long-lasting effects on a person's health and mobility. Early diagnosis and prompt medical attention can greatly improve the chances of a full recovery.

Symptoms

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Muscle Weakness
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Difficulty swallowing
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Difficulty breathing
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Numbness in fingers and toes
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Pain in lower back, legs, or arms
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Back pain

Confirmation Tests

- Spinal fluid analysis
- Electromyography (EMG)
- Nerve conduction test

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