Guillain-Barré Syndrome: Types, Causes, Risk Factors, Symptoms, Diagnosis & Treatment

Guillain-Barré syndrome (GBS) is a rare but serious autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nervous system. 

This condition is commonly triggered by infectious agents of Guillain-Barré syndrome, such as Campylobacter jejuni, Cytomegalovirus (CMV), Epstein-Barr virus (EBV), Zika virus, and COVID-19. It can also occur after vaccination or surgical procedures in rare cases. 

The immune system's attack damages the myelin sheath (the protective covering of nerves) or the axons (the nerve fibers themselves), leading to muscle weakness, numbness, and, in severe cases, paralysis. Guillain-Barré syndrome can affect people of all ages, but it is more common in adults and males. Early diagnosis and treatment are crucial to prevent complications and improve outcomes.

Guillain-Barré syndrome is considered a medical emergency because it can progress rapidly, leading to life-threatening complications such as respiratory failure. However, with prompt treatment, most patients recover fully or with minimal effects.

A neurologist is a specialist who treats Guillain-Barré syndrome (GBS). Guillain-Barré syndrome is a medical emergency that usually requires hospitalization.

Guillain-Barré syndrome meaning

The term "Guillain-Barré syndrome" is named after the French neurologists Georges Guillain and Jean Alexandre Barré, who, along with André Strohl, first described the condition in 1916. They identified the characteristic symptoms and the link between the syndrome and elevated protein levels in cerebrospinal fluid. Guillain-Barré syndrome is also known as acute inflammatory demyelinating polyneuropathy (AIDP), which refers to inflammation and damage to the myelin sheath of peripheral nerves.

Prevalence of Guillain-Barré syndrome

Incidence and prevalence of Guillain-Barré syndrome worldwide

Globally, the incidence of Guillain-Barré syndrome (GBS) is estimated to be between 1 and 2 cases per 100,000 people per year, which means it affects approximately 1-2 people out of 100,000 people each year.

Epidemiology of Guillain-Barré syndrome in Indian population

The reported incidence of Guillain-Barré syndrome in India varies, with estimates ranging from 1-2 cases per 100,000 people annually. A study conducted at major teaching hospitals found that approximately 138 cases are diagnosed each year. Another retrospective study from Rajasthan indicated a prevalence of 41.5% among cases of acute flaccid paralysis recorded between 2012 and 2015. There was a considerable male predominance (68.5%).

Types of Guillain-Barré syndrome

Guillain-Barré syndrome is classified into several subtypes based on the specific part of the nervous system affected and the progression of symptoms. The main types include:

• Acute inflammatory demyelinating polyneuropathy (AIDP): This is the most common form of Guillain-Barré syndrome, accounting for about 85–90% of cases in western countries. It is characterized by damage to the myelin sheath of peripheral nerves, leading to muscle weakness and sensory disturbances.

• Miller fisher syndrome (MFS): A rare variant of Guillain-Barré syndrome that primarily affects the cranial nerves, leading to symptoms like double vision, difficulty swallowing, and poor coordination. MFS is more common in Asia and is often associated with antibodies against a specific nerve component called GQ1b.

• Acute motor axonal neuropathy (AMAN): This type involves damage to the axons of motor nerves, leading to severe muscle weakness without significant sensory loss. AMAN is more common in children and young adults, particularly in rural areas of China and Mexico.

• Acute motor-sensory axonal neuropathy (AMSAN): A more severe form of Guillain-Barré syndrome that affects both motor and sensory nerves. AMSAN is less common but has a poorer prognosis due to extensive nerve damage.

• Acute panautonomic neuropathy: Acute panautonomic neuropathy, the rarest Guillain-Barré syndrome variant, involves sympathetic and parasympathetic nervous systems. Patients may have severe postural hypotension, bowel and bladder retention, decreased salivation and lacrimation, and pupillary abnormalities. Cardiovascular involvement is common, and dysrhythmias are a significant source of mortality. Recovery is gradual and often incomplete.

• Pure sensory GBS: It is characterized by a symmetrical and extensive pattern of rapid onset sensory loss, sensory ataxia, and areflexia. The results of EMG tests reveal distinctive indications of a demyelinating process in the peripheral nerves, and lumbar puncture examinations reveal albumin cytologic separation in the Cerebro spinal fluid (CSF).

• Pharyngeal-cervical-brachial variant: Weaknesses are limited to facial, oropharyngeal, cervical, and upper limb areas without lower limb involvement.

Symptoms of Guillain-Barré syndrome

Guillain-Barré syndrome symptoms often develop rapidly and can progress over days or weeks. The most common Guillain-Barré syndrome signs and symptoms include:

• Muscle weakness and Tingling: Often begins in the feet and legs, spreading to the arms and upper body.

• Paresthesia: Sensations such as "pins and needles," tingling, or numbness in hands and feet.

• Pain: Sharp, shooting pain often occurs in the legs or back, which may worsen at night.

• Coordination problems: Unsteadiness or difficulty walking due to muscle weakness.

• Facial symptoms: Drooping face muscles, trouble swallowing (dysphagia), speaking (dysarthria), or chewing; double vision; difficulty moving eyes.

• Respiratory issues: Difficulty breathing if chest muscles are affected; may require intensive care support.

• Autonomic dysfunction: Abnormal heart rate or blood pressure fluctuations; bladder control issues may occur in severe cases.

The progression of symptoms typically peaks within 2–4 weeks, after which patients enter a plateau phase before recovery begins. Recovery can take weeks to months, and some patients may experience long-term residual effects.

Guillain-Barré syndrome in children symptoms

In children, Guillain-Barré syndrome often begins with weakness in the lower limbs, which can spread to the upper body. A common sign is the loss of reflexes, making it harder for health care experts to detect them. Many children experience neuropathic pain, which can be quite distressing. Younger kids might refuse to walk due to pain or weakness in their legs. 

Some may also have trouble with facial movements or swallowing because of cranial nerve involvement. Additionally, children with GBS might experience fluctuations in blood pressure and other autonomic issues. Sensory disturbances like numbness or tingling are also common. These symptoms can vary widely among children, and some might show unusual signs like uneven weakness or predominant sensory problems.

Early symptoms of Guillain-Barré syndrome

The initial symptoms of Guillain-Barré syndrome (GBS) include weakness in the limbs and tingling. These symptoms may spread to the arms and upper body.

Guillain-Barré syndrome causes

Guillain-Barré Syndrome (GBS) is not inherited, but it is linked to various infections, illnesses, and traumas. The exact cause remains unknown. It's believed that certain illnesses might alter nerve cells, causing the immune system to mistakenly view them as threats. In some cases, the immune system seems to lose its ability to distinguish between harmful invaders and the body's own cells.

When GBS occurs, the immune system starts attacking the nerves. In the most common form of GBS, known as Acute inflammatory demyelinating polyneuropathy (AIDP), the protective covering of the nerves (myelin sheath) is damaged. Sometimes, the nerve's core (axon) is also affected. This damage disrupts the nerve signals between the brain and muscles, leading to muscle weakness and abnormal sensations. As a result, muscles cannot respond properly to brain commands, and the brain receives distorted sensory information from the affected areas.

Risk Factors for Guillain-Barré syndrome

Guillain-Barré syndrome is a complex condition where the immune system mistakenly attacks nerves. Identifying its triggers and risk factors is crucial for developing effective treatment strategies and improving patient outcomes in this challenging neurological disorder.

Certain factors may increase the risk of developing Guillain-Barré syndrome, including:

• Age
• Gender
• Recent infection
• Chronic illness
• Surgery or Trauma
• Vaccinations
• Other factors

Age: Older adults above the age of 50 years old have a higher risk of Guillain-Barré syndrome due to age-related immune system changes, making them more susceptible to autoimmune responses that attack peripheral nerves.

Gender: Men are slightly more likely to develop Guillain-Barré syndrome than women, possibly due to hormonal or genetic differences that influence immune system behavior and nerve vulnerability.

Recent infection: Bacterial (e.g., Campylobacter jejuni) or viral (e.g., flu, Zika) infections can trigger Guillain-Barré syndrome by causing the immune system to mistakenly attack nerve cells.

Chronic illnesses: Conditions like lupus or HIV weaken the immune system or cause chronic inflammation, increasing the likelihood of autoimmune reactions that lead to Guillain-Barré syndrome.

Surgery or Trauma: Recent surgeries or injuries can stress the immune system, potentially triggering an abnormal immune response that damages peripheral nerves and leads to Guillain-Barré syndrome.

Vaccinations: While rare, certain vaccines, such as the influenza vaccine and some COVID-19 vaccines have been associated with a slight increase in GBS risk.

Other factors: Trauma, certain medical conditions like Hodgkin's lymphoma, and exposure to other pathogens like Mycoplasma pneumoniae and hepatitis viruses have also been linked to GBS.

Guillain-Barré syndrome complications

While many people are fortunate enough to make a full recovery from Guillain-Barré syndrome, others face a more challenging journey. For them, GBS can lead to serious complications that profoundly affect their quality of life and even survival. These complications often stem from extensive damage to the nerves, which can disrupt the body's ability to perform its most basic yet vital functions.

Key complications of Guillain barre syndrome include:

• Breathing difficulty
• Paralysis
• Dysautonomia
• Blood pressure fluctuations
• Arrhythmias
• Contractures and Deformities
• Pressure sores
• Infections
• Thromboembolic events
• Long-term effects
• Mortality risks

Breathing difficulty:  Approximately 22% of patients may require mechanical ventilation due to respiratory muscle weakness, particularly in the early stages of disease. Severe cases can lead to respiratory failure, which is life-threatening.

Paralysis:  Many patients experience varying degrees of paralysis, which can be temporary or, in rare cases, permanent.

Dysautonomia:  This condition affects autonomic functions like heart rate and blood pressure, leading to instability and potentially life-threatening complications.

Blood pressure fluctuations:  Patients often experience low or unstable blood pressure, which can complicate their clinical management.

Arrhythmias:  Cardiac irregularities are common and can increase mortality risk, particularly in older patients.

Contractures and Deformities:  Prolonged immobility can lead to shortening of tissues in the joints (contractures), causing deformities over time.

Pressure sores:  Patients are at risk of developing bedsores due to extended periods of inactivity.

Infections:  Patients with GBS are more susceptible to infections such as pneumonia and urinary tract infections due to reduced mobility and respiratory function.

Thromboembolic events:  GBS increases the risk of deep vein thrombosis (DVT), necessitating preventive measures like blood thinners and compression stockings.

Long-term effects:  While many individuals recover fully, about 15-30% may experience persistent weakness or other neurological deficits long after the initial episode.

Mortality risks:  The overall mortality rate ranges from 2% to 12%, with higher rates in older populations due to complications such as sepsis or acute respiratory distress syndrome (ARDS).

Guillain-Barré syndrome diagnosis

Diagnosing Guillain-Barré syndrome (GBS) can be a deeply concerning experience for patients, as symptoms like weakness or tingling often appear suddenly and progress quickly. Health care experts carefully listen to the patient’s history and perform detailed exams to provide clarity and guide the next steps toward care.

Guillain-Barré syndrome diagnosis criteria include:

Medical history and Physical examination

• Assessing symptoms, reflexes, and muscle strength.

Lumbar puncture (Spinal tap)

• To analyze cerebrospinal fluid for elevated protein levels.

Electromyography (EMG)

• To evaluate nerve and muscle function.

Nerve conduction studies (NCS)

• To measure the speed of nerve signals.

Blood tests

• To rule out other conditions or infections.

Imaging tests

• Magnetic resonance imaging (MRI) of the spine may show nerve root enhancement, supporting the diagnosis of Guillain-Barré syndrome.
  

Guillain-Barré syndrome treatment

While there is no cure for Guillain-Barré syndrome, early treatment can significantly improve outcomes. Guillain-Barré syndrome is usually treated in a hospital, generally in the intensive care unit, where the patient can be constantly watched 24 hours a day, seven days a week, and intensive nursing, respiratory support, and other required services are readily available.

The recommendations for treating and taking care for patients with Guillain-Barré syndrome are as follows:

• Intravenous immunoglobulin (IVIG)
• A therapy that uses antibodies to reduce the immune system's attack on nerves.

• Plasma exchange (Plasmapheresis)
• A procedure to remove harmful antibodies from the blood.

• Supportive care
• Physical therapy to maintain muscle strength and mobility.
• Mechanical ventilation for patients with respiratory failure.
• Pain management with medications.
• Medications to prevent blood clots.

• Long-term rehabilitation
• Occupational therapy and counseling help patients regain independence and cope with emotional challenges.
  

Prevention of Guillain-Barré syndrome

Preventing Guillain-Barré syndrome (GBS) is challenging due to its complex autoimmune nature. Since Guillain barre syndrome is often triggered by infections, preventive measures include:

• Practicing good hygiene: To reduce the risk of bacterial and viral infections.

• Staying updated on vaccinations: Staying up to date on vaccines (e.g., flu vaccine) to prevent infections that may trigger GBS.

• Early treatment of infections: Promptly treating infections to reduce the risk of complications.

• Avoiding contaminated food and water: To prevent infections like Campylobacter jejuni.



• Prompt treatment of infections: Early medical intervention helps prevent complications from infections that might trigger GBS.

Difference between Guillain-Barré syndrome and Transverse myelitis

Guillain-Barré syndrome vs transverse myelitis

Guillain-Barré syndrome (GBS) and Transverse myelitis (TM) are both neurological disorders that can cause weakness and paralysis, although they have different causes, symptoms, and areas of involvement. The six significant differences between these two circumstances are listed below.