What is the immediate treatment for a patient diagnosed with Guillain-Barré Syndrome (GBS)?

Immediate Treatment for Guillain-Barré Syndrome (GBS)

The immediate treatment for Guillain-Barré Syndrome is intravenous immunoglobulin (IVIg) at 0.4 g/kg body weight daily for 5 days (total dose 2 g/kg) or plasma exchange (200-250 ml plasma/kg body weight in five sessions), with IVIg generally preferred due to easier administration and wider availability. 1

Initial Assessment and Monitoring

Respiratory Function Evaluation

• Monitor for signs of respiratory distress:
  • Breathlessness at rest or during talking
  • Inability to count to 15 in a single breath
  • Use of accessory respiratory muscles
  • Increased respiratory or heart rate
  • Vital capacity <15-20 ml/kg or <1 L
  • Abnormal arterial blood gas or pulse oximetry 1

Risk Assessment for Respiratory Failure

• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict the probability of requiring ventilation within 1 week 1
• Consider the "20/30/40 rule": patient at risk if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1

Treatment Algorithm

For Patients Unable to Walk Unaided (Moderate to Severe GBS)

1. First-line treatment:

   • IVIg 0.4 g/kg/day for 5 consecutive days (total 2 g/kg) if within 2-4 weeks of symptom onset 1, 2
   • OR Plasma exchange 200-250 ml plasma/kg in five sessions over 1-2 weeks if within 4 weeks of symptom onset 1

2. Treatment selection considerations:

   • IVIg is generally preferred due to:
     • Easier administration
     • Wider availability
     • Lower discontinuation rate
     • Fewer complications 1
   • Plasma exchange may be considered if:
     • IVIg is contraindicated or unavailable
     • Resource constraints exist (though small-volume plasma exchange requires further validation) 1

For Severe Cases with Respiratory Compromise

1. Immediate actions:

   • Admit to inpatient unit with capability for rapid transfer to ICU-level monitoring 1
   • Start IVIg or plasma exchange as above 1
   • Consider corticosteroids (methylprednisolone 2-4 mg/kg/day) in immune checkpoint inhibitor-related GBS forms only 1
   • Implement frequent neurological checks and pulmonary function monitoring 1

2. Ventilatory support:

   • Consider early intubation for patients with signs of respiratory distress
   • Consider early tracheostomy in patients with risk factors for prolonged mechanical ventilation:
     • Inability to lift arms from bed at 1 week after intubation
     • Axonal subtype or unexcitable nerves on electrophysiological studies 1

Special Patient Populations

Pregnant Women

• Both IVIg and plasma exchange are not contraindicated
• IVIg is generally preferred as plasma exchange requires additional monitoring 1

Children

• IVIg is usually the first-line therapy due to:
  • Limited availability of plasma exchange in pediatric centers
  • Greater discomfort and higher complication rates with plasma exchange in children
  • Standard adult regimen of 2 g/kg over 5 days is preferred over 2-day regimen (which has higher treatment-related fluctuation rates) 1

GBS Variants

• Miller Fisher Syndrome (MFS): Treatment generally not recommended for pure MFS, but close monitoring is essential 1
• Bickerstaff's Brainstem Encephalitis (BBE): IVIg or plasma exchange is justified due to severity 1

Important Caveats and Pitfalls

1. Avoid ineffective treatments:

   • Corticosteroids alone are not effective for idiopathic GBS and may have negative effects 1, 3
   • Combined plasma exchange followed by IVIg is no more effective than either treatment alone 1
   • Second IVIg course is not recommended for patients with poor prognosis 2

2. Monitor for complications:

   • Autonomic dysfunction (monitor heart rate, blood pressure, bowel and bladder function)
   • Swallowing difficulties and diminished cough reflex
   • Pain (consider gabapentinoids, tricyclic antidepressants, or carbamazepine) 1, 2
   • Treatment-related fluctuations (TRFs) occur in about 10% of patients within 8 weeks of IVIg initiation 3

3. Watch for disease progression:

   • If progression continues beyond 8 weeks from onset, consider changing diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP), which occurs in about 5% of patients initially diagnosed with GBS 2

By following this treatment approach, you can optimize outcomes for patients with Guillain-Barré Syndrome, reducing morbidity, mortality, and improving quality of life.