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

Immediate Treatment for Guillain-Barré Syndrome

The immediate treatment for a patient diagnosed with Guillain-Barré Syndrome (GBS) is intravenous immunoglobulin (IVIg) at a dose of 0.4 g/kg body weight daily for 5 days, or alternatively plasma exchange (PE) with 200-250 ml plasma/kg body weight in five sessions. 1

Initial Assessment and Monitoring

Before initiating treatment, patients should be assessed for:

• Risk of respiratory failure using the Erasmus GBS Respiratory Insufficiency Score (EGRIS)
• Presence of bulbar weakness or facial weakness
• Autonomic dysfunction
• Swallowing difficulties
• Vital capacity and respiratory function

Indications for ICU Admission:

• Evolving respiratory distress or imminent respiratory insufficiency
• Severe autonomic cardiovascular dysfunction
• Severe swallowing dysfunction or diminished cough reflex
• Rapid progression of weakness 1

Signs of respiratory distress requiring immediate attention include:

• 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 measurements 1

Treatment Algorithm

For All Patients with GBS Unable to Walk Unaided:

1. First-line treatment:

   • IVIg 0.4 g/kg/day for 5 consecutive days (total 2 g/kg) 1
   • OR Plasma exchange (PE): 200-250 ml plasma/kg in five sessions 1

2. Treatment selection considerations:

   • IVIg is generally preferred due to:
     • Easier administration
     • Greater availability
     • Lower discontinuation rates
     • Similar efficacy to PE 1, 2

3. Special populations:

   • Pregnant women: Both IVIg and PE are not contraindicated, but IVIg is preferred 1
   • Children: IVIg is preferred over PE due to lower complication rates 1
   • Miller Fisher Syndrome (MFS): Treatment generally not recommended unless progression to limb weakness, bulbar/facial palsy, or respiratory failure occurs 1

Monitoring During Treatment

• Regular respiratory function assessment:

  • Use of accessory respiratory muscles
  • Single breath count (≤19 predicts need for mechanical ventilation)
  • Vital capacity
  • Maximum inspiratory and expiratory pressure
  • Consider the "20/30/40 rule" (risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O) 1

• Muscle strength assessment using Medical Research Council grading scale

• Swallowing and coughing ability

• Autonomic function (ECG, heart rate, blood pressure, bowel/bladder function) 1

Managing Complications

• Pain management: Consider gabapentinoids, tricyclic antidepressants, or carbamazepine 3
• Autonomic dysfunction: Monitor for arrhythmias and blood pressure fluctuations
• Deep vein thrombosis prophylaxis
• Prevention of pressure ulcers
• Management of bulbar palsy complications
• Psychological support for anxiety and depression 1

Important Considerations and Pitfalls

• Do not use corticosteroids alone: Eight randomized controlled trials showed no significant benefit, and oral corticosteroids may have negative effects 1
• Avoid combination therapy: Plasma exchange followed by IVIg is no more effective than either treatment alone 1
• Be alert for treatment-related fluctuations (TRFs): Occur in 6-10% of patients within 2 months following initial improvement; may consider repeating treatment 1
• Watch for progression to CIDP: About 5% of GBS patients develop acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP), characterized by ≥3 TRFs and/or clinical deterioration ≥8 weeks after onset 1
• Monitor for respiratory failure: Up to 22% of GBS patients require mechanical ventilation within the first week of admission 1

Early recognition and prompt treatment are critical for improving outcomes in GBS, with mortality rates of 3-10% despite optimal treatment 1, 4.