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

Management of Guillain-Barré Syndrome (GBS)

Intravenous immunoglobulin (IVIg) is the first-line treatment for Guillain-Barré Syndrome, administered at 0.4 g/kg daily for 5 days, with plasma exchange as an equally effective alternative in patients who cannot walk independently within 4 weeks of symptom onset. 1

Initial Assessment and Diagnosis

• Assess for typical presentation:

  • Progressive symmetric weakness (usually ascending)
  • Reduced or absent deep tendon reflexes
  • History of recent respiratory or gastrointestinal infection (1-4 weeks prior)
  • Facial and/or bulbar weakness
  • Autonomic dysfunction

• Diagnostic workup:

  • Lumbar puncture (typically shows elevated protein with normal cell count)
  • Electrodiagnostic studies to confirm polyneuropathy
  • MRI spine with contrast to rule out compressive lesions
  • Consider anti-ganglioside antibody testing (especially anti-GQ1b for Miller Fisher variant)

Risk Assessment for Respiratory Failure

• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict need for ventilation:

  • Days between onset and hospital admission (≤3 days: 2 points; 4-7 days: 1 point)
  • Facial/bulbar weakness (present: 1 point)
  • MRC sum score (≤20: 4 points; 21-30: 3 points; 31-40: 2 points; 41-50: 1 point)
  • Total score 0-7; higher scores indicate greater risk of respiratory failure 1

• Monitor respiratory function regularly:

  • Vital capacity (VC < 20 ml/kg indicates risk)
  • Maximum inspiratory pressure (< 30 cmH₂O indicates risk)
  • Maximum expiratory pressure (< 40 cmH₂O indicates risk)
  • Single breath count (≤19 predicts need for ventilation)
  • Use of accessory respiratory muscles

Treatment Algorithm

1. Disease-Modifying Treatment

• First-line treatment options:

  • IVIg: 0.4 g/kg daily for 5 days (total 2 g/kg)
  • Plasma exchange: 200-250 ml plasma/kg in 5 sessions over 1-2 weeks

• Treatment decision factors:

  • Both IVIg and plasma exchange are equally effective
  • IVIg is preferred due to:
    • Greater ease of administration
    • Better completion rates
    • Wider availability
    • Fewer complications 1

• Treatment timing:

  • Start within 2 weeks of symptom onset for IVIg
  • Start within 4 weeks of symptom onset for plasma exchange
  • Treatment indicated for patients unable to walk independently

• Important treatment considerations:

  • Corticosteroids are NOT recommended (shown to be ineffective or harmful) 1
  • Combined treatment (plasma exchange followed by IVIg) is NOT recommended (no additional benefit over single therapy) 1
  • For treatment-related fluctuations (6-10% of patients), repeating the full course of IVIg or plasma exchange is common practice

2. ICU Admission Criteria

• Indications for ICU admission:

  • Evolving respiratory distress or imminent respiratory failure
  • Severe autonomic dysfunction (arrhythmias, blood pressure fluctuations)
  • Severe swallowing dysfunction or diminished cough reflex
  • Rapid progression of weakness 1

• Consider early tracheostomy if:

  • Unable to lift arms from bed at 1 week after intubation
  • Axonal subtype or unexcitable nerves on electrophysiological studies 1

3. Special Patient Populations

• Miller Fisher Syndrome (MFS):

  • Generally has mild course with complete recovery
  • Treatment typically not recommended
  • Monitor closely for development of limb weakness, bulbar/facial palsy, or respiratory failure 1

• Bickerstaff brainstem encephalitis (BBE):

  • Treatment with IVIg or plasma exchange recommended due to severity 1

• Pregnant women:

  • Both IVIg and plasma exchange are safe
  • IVIg generally preferred due to simpler monitoring requirements 1

• Children:

  • IVIg preferred over plasma exchange (fewer complications)
  • Use standard 5-day regimen rather than accelerated 2-day regimen (fewer fluctuations) 1

• Immune checkpoint inhibitor-related GBS:

  • Permanently discontinue the checkpoint inhibitor
  • Administer IVIg or plasma exchange
  • Consider adding corticosteroids (unlike idiopathic GBS) 1

Management of Complications

• Respiratory complications:

  • Regular monitoring of respiratory function
  • Early intubation if respiratory parameters deteriorate
  • Consider "20/30/40 rule" for respiratory failure risk assessment

• Autonomic dysfunction:

  • Continuous cardiac monitoring
  • Regular blood pressure checks
  • Management of arrhythmias and blood pressure fluctuations

• Pain management:

  • Consider gabapentin, pregabalin, tricyclic antidepressants, or carbamazepine 1

• Other supportive care:

  • DVT prophylaxis
  • Pressure ulcer prevention
  • Nutritional support
  • Early rehabilitation
  • Eye care for patients with facial weakness
  • Psychological support

Monitoring Disease Progression

• Regular assessment of:

  • Muscle strength using Medical Research Council grading
  • Functional disability using GBS disability scale
  • Respiratory parameters
  • Swallowing and coughing ability
  • Autonomic function (heart rate, blood pressure, bowel/bladder function) 1

• Be vigilant during recovery phase as up to two-thirds of deaths occur during this period, primarily from cardiovascular and respiratory complications 1

Prognosis

• Approximately 80% of patients regain independent walking by 6 months
• Mortality rate is 3-10%, most commonly due to cardiovascular and respiratory complications
• Long-term residual symptoms may include neuropathic pain, weakness, and fatigue
• Recovery may continue for more than 5 years after disease onset 1