What is the treatment for Guillain-Barré Syndrome (GBS)?

Treatment of Guillain-Barré Syndrome

First-line treatment for Guillain-Barré Syndrome (GBS) patients who are unable to walk independently is either intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days or plasma exchange (PE) with 4-5 exchanges over 1-2 weeks. 1, 2

Immunotherapy Options

First-line Treatment

• IVIg therapy: 0.4 g/kg/day for 5 consecutive days

  • Preferred in most clinical settings due to practical considerations
  • Should be initiated within 2 weeks of symptom onset (can still be considered within 2-4 weeks)
  • For patients unable to walk independently (GBS disability score ≥3) 1, 2

• Plasma exchange: 12-15L in 4-5 exchanges over 1-2 weeks

  • Equally effective as IVIg but has higher complication rates
  • Requires specialized equipment
  • Should be initiated within 4 weeks of symptom onset
  • For patients unable to walk independently 1, 2

Treatments NOT Recommended

• Combination therapy (PE followed by IVIg) - no additional benefit 1
• Corticosteroids - no benefit and potential negative effects 1, 2
• Second IVIg course in patients with poor prognosis - not recommended based on current evidence 2

Respiratory Management

Respiratory failure occurs in approximately 30% of GBS patients, requiring close monitoring:

• Apply the "20/30/40 rule" for respiratory monitoring:

  Parameter	Critical Value
  Vital Capacity	<20 ml/kg
  Maximum Inspiratory Pressure	<30 cmH₂O
  Maximum Expiratory Pressure	<40 cmH₂O

• Single breath count ≤19 predicts need for mechanical ventilation

• Consider early intubation for patients showing signs of respiratory compromise

Management of Complications

Pain Management

• First-line: Gabapentinoids (pregabalin, gabapentin) 1
• Second-line: Tricyclic antidepressants or carbamazepine
• Avoid opioids when possible 1

Autonomic Dysfunction

• Monitor closely for blood pressure fluctuations, heart rate abnormalities, pupillary dysfunction, and temperature dysregulation 1
• Treat symptomatic dysautonomia as needed

Preventive Measures

• Implement standard preventive measures for:
  • Pressure ulcers
  • Hospital-acquired infections (pneumonia, UTIs)
  • Deep vein thrombosis 1

Managing Treatment-Related Fluctuations (TRFs)

• TRFs occur in 6-10% of patients within 2 months of initial treatment 3, 1
• Consider repeating the full course of IVIg or PE for patients with TRFs, although evidence is limited 3
• Be alert for development of acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) in approximately 5% of patients initially diagnosed with GBS 3, 1

Rehabilitation and Long-term Care

• Arrange comprehensive rehabilitation before discharge 3, 1

• Address potential long-term issues:

  • Fatigue (affects 60-80% of patients)
  • Chronic pain (affects at least one-third of patients)
  • Physical function restoration 3, 1

• Exercise programs should include:

  • Range-of-motion exercises
  • Stationary cycling
  • Walking and strength training 3
  • Monitor intensity to prevent overexertion

Prognosis

• Approximately 80% of patients regain independent walking by 6 months 3, 1
• Death occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 3
• Risk factors for mortality include advanced age and severe disease at onset 3
• Long-term residual complaints are common (neuropathic pain, weakness, fatigue) 3, 1
• Recovery may continue for more than 5 years after disease onset 3

Clinical Pitfalls to Avoid

1. Delayed treatment initiation: Start immunotherapy promptly in patients who cannot walk independently
2. Inadequate respiratory monitoring: Use the 20/30/40 rule to identify patients at risk for respiratory failure
3. Missing treatment-related fluctuations: Be vigilant for clinical deterioration after initial improvement
4. Overlooking A-CIDP: Consider this diagnosis in patients with ≥3 TRFs or clinical deterioration ≥8 weeks after onset
5. Neglecting pain management: Chronic pain is common and significantly impacts quality of life
6. Insufficient rehabilitation planning: Comprehensive rehabilitation is crucial for optimal recovery