What is Guillain-Barre Syndrome (GBS)?

Guillain-Barré syndrome (pronounced ghee-yan bar-ray) is a rare neurological and autoimmune disease where your bodies immune system mistakenly attacks your peripheral nervous system.  This can affect your feelings of sensation, movement and even breathing and your heartbeat.  GBS affects roughly 2 in 100,000 people. It is acute in nature, meaning it has a quick onset and requires urgent hospital treatment. 

• 🦠 Acute Onset: Guillain-Barré Syndrome presents with rapid, symmetrical weakness beginning in the feet
• 🧬 Immune Response: The syndrome is linked to molecular mimicry, where antibodies target nerve cells
• 🏥 Symptoms: Patients experience reduced reflexes, sensory loss, and potential cranial nerve involvement
• 📈 Progression: Symptoms typically appear within four weeks post-infection and peak within two to four weeks
• 🧪 Diagnosis: Diagnosis is clinical, supported by nerve conduction studies and lumbar puncture findings
• 💉 Management: Treatment includes IV immunoglobulins, plasma exchange, and supportive care to prevent complications
• ⚖️ Prognosis: Approximately 80% recover fully, while 15% may have some lasting neurological issues

What triggers Guillain-Barre Syndrome (GBS)?

Guillain-Barré Syndrome (GBS) is typically triggered by an infection and is particularly associated with gastroenteritis caused by campylobacter jejuni or infection with cytomegalovirus or epstein-barr virus.

Bacterial Infections: 

Campylobacter jejuni, a bacterium linked to food poisoning, is a frequent trigger.

Viral Infections: 

Influenza, Epstein-Barr virus, Zika virus, and even cytomegalovirus can precede GBS.

Respiratory and Digestive Infections:

The syndrome often develops after common infections that impact the respiratory or digestive tract.

Vaccinations or Surgeries: 

Rarely, GBS may be associated with recent vaccinations such as COVID vaccinations or surgical procedures.

Signs of Guillain-Barré Syndrome

Guillain-Barre syndrome presents with a symmetrical ascending weakness which starts from the feet and moves up the body. There will be reduced reflexes and because it’s a peripheral neuropathy, there may be peripheral loss of sensation or neuropathic pain and it may progress to the cranial nerves and cause a facial nerve weakness as well. 

Symptoms usually start within four weeks of the preceding infection.  symptoms typically start in the feet and progress upwards and the symptoms peak within two to four weeks and then there’s a slow recovery period that can last months to years.

The common signs of Guillain-Barré syndrome include:

• Tingling, pins & needles and numbness in your feet and ascending to your hands
• Symmetrical muscle weakness affecting both sides of the body equally difficulty moving your joints 
• Possible shooting pains in your legs or back
• Experiencing problems with your breathing
• Noticing facial changes such as drooping face muscles or trouble with swallowing or speaking

In severe cases, you may not able to move your legs, arms and face.  This is known as paralysis.

What is the pathophysiology of Guillain-Barré Syndrome?

Guillain-Barre syndrome is thought to occur due to a process called molecular mimicry, following a preceding infection.  This is where the B cells which are a type of white blood cell create infection-fighting proteins called antibodies against the antigens on the pathogen (bacteria or virus) that’s caused the infection.

So if you’ve got campylobacter gastroenteritis, the B cells of your immune system create antibodies to fight the campylobacter. These antibodies also match proteins on the nerve cells.  In GBS, the antibodies your body creates may attack proteins on the myelin sheath (the sheath that covers your nerves) of the motor nerve (the nerve that responsible for movement) or on the nerve axon.  This results in damage to your nerve cells causing the neuropathy and the symptoms of GBS.

How is Guillain-Barre syndrome diagnosed?

Diagnosis of Guillain-Barre syndrome is made clinically. There is no specific test that confirms the diagnosis but there is some criteria called the brighton criteria that can be used to make a diagnosis.  It can be supported by investigations such as nerve conduction studies which show reduced signal throughout the nerves and lumbar puncture to test Cerebrospinal fluid. Somebody with Guillain-Barre may have raised protein with normal cell count and normal glucose.

How is Guillain-Barre syndrome managed?

The management of Guillain-Barre focuses on immunotherapy.  This can help help stop your immune system from attacking your nerves.

The following treatment management options include:

• IV immunoglobulins administered over 5 days
• Plasma exchange to try and remove some antibodies
• Supportive care to make sure the patient is still well despite having the associated weakness 
• Venous thromboembolism prophylaxis such as heparin to prevent developing of blood clots because pulmonary embolism is a leading cause of death in Guillain-Barre syndrome 
• In very severe cases where there’s respiratory failure then the patients made it intubation ventilation and admission to the intensive care unit 

Recovery & Prognosis

80% of people will fully recover. 15% will be left with some sort of neurological disability such as slight weakness or some neuropathic pain 5% of people will actually die from the condition

Sources

• Guillain Barré syndrome – WHO
• Guillain Barré syndrome  – NHS