Guillain-Barré Syndrome (GBS)

Definition

Guillain-Barré Syndrome (GBS) is an acute, rapidly progressing, and potentially life-threatening autoimmune disorder in which the body's immune system attacks the peripheral nerves. It often leads to muscle weakness, numbness, and, in severe cases, paralysis.

Aetiology

The exact cause of GBS is unknown, but it is often preceded by an infectious illness such as a respiratory infection or gastrointestinal infection. Other potential triggers include:

• Campylobacter jejuni infection
• Influenza virus
• Epstein-Barr virus (EBV)
• Cytomegalovirus (CMV)
• Mycoplasma pneumoniae
• Zika virus
• Vaccinations (rarely)
• Surgery

Pathophysiology

GBS occurs when the immune system mistakenly attacks the peripheral nerves, causing inflammation and damage to the myelin sheath or the nerve axon itself. This disruption impairs the transmission of nerve signals, leading to muscle weakness and sensory disturbances. The exact mechanism involves both humoral and cell-mediated immune responses.

Risk Factors

• Recent infection (e.g., respiratory or gastrointestinal)
• Age (more common in older adults)
• Sex (slightly more common in males)
• History of autoimmune diseases
• Genetic predisposition

Signs and Symptoms

• Symmetrical muscle weakness, typically starting in the legs and spreading to the upper body
• Numbness, tingling, or pain, especially in the extremities
• Difficulty walking or climbing stairs
• Severe cases may lead to paralysis
• Difficulty with facial movements, including speaking, chewing, or swallowing
• Severe respiratory muscle weakness, requiring ventilatory support
• Autonomic dysfunction (e.g., heart rate and blood pressure fluctuations)

Investigations

• Clinical history and physical examination
• Nerve conduction studies and electromyography (EMG) to assess nerve function
• Lumbar puncture to test cerebrospinal fluid (CSF) for elevated protein levels with normal white blood cell count
• Blood tests to rule out other causes and assess overall health

Management

Primary Care Management

• Immediate referral to hospital if GBS is suspected
• Initial supportive care, including monitoring respiratory function and autonomic stability

Specialist Management

• Hospitalisation for close monitoring and supportive care
• Intravenous immunoglobulin (IVIG) therapy or plasma exchange (plasmapheresis) to reduce immune system activity
• Pain management with analgesics or neuropathic pain medications
• Physical therapy to maintain muscle strength and prevent contractures
• Occupational therapy to assist with daily activities
• Respiratory support if needed, including mechanical ventilation in severe cases

Example Management for Guillain-Barré Syndrome

A patient presenting with rapidly progressing muscle weakness and suspected GBS should be referred to the hospital immediately. Initial management includes close monitoring of respiratory function and autonomic stability. Treatment may involve IVIG therapy or plasma exchange to reduce the immune response. Supportive care includes pain management, physical and occupational therapy, and respiratory support if necessary. Regular follow-up is essential to monitor recovery and address any long-term complications.

References

1. NICE. (2024). Guillain-Barré Syndrome: Diagnosis and Management. Retrieved from NICE
2. NHS. (2023). Guillain-Barré Syndrome. Retrieved from NHS
3. Yuki, N., & Hartung, H. P. (2012). Guillain-Barré Syndrome. New England Journal of Medicine.
4. Van Doorn, P. A. (2013). Guillain-Barré Syndrome. The Lancet.
5. Hughes, R. A., & Cornblath, D. R. (2005). Guillain-Barré Syndrome. The Lancet.