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

Treatment for Guillain-Barré Syndrome (GBS)

Patients with Guillain-Barré Syndrome who are unable to walk unaided should be treated with intravenous immunoglobulin (IVIG) at 0.4 g/kg/day for 5 consecutive days if within 2 weeks of symptom onset, or plasma exchange if IVIG is unavailable. 1

Diagnosis and Clinical Features

GBS is an immune-mediated inflammatory disease of the peripheral nervous system characterized by:

• Rapidly progressive bilateral weakness of legs and/or arms
• Decreased or absent reflexes
• Distal paresthesias or sensory loss
• Pain and autonomic dysfunction
• Maximum progression typically within 2 weeks 2, 1

GBS can be classified into several subtypes:

• Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) - most common in Western countries
• Acute motor axonal neuropathy (AMAN)
• Acute motor sensory axonal neuropathy (AMSAN)
• Miller Fisher syndrome (MFS) - characterized by ophthalmoplegia, ataxia, and areflexia 2

Treatment Algorithm

Step 1: Assess Severity and Timing

• If patient is unable to walk unaided and within 2-4 weeks of symptom onset, immunotherapy is indicated 1, 3
• Apply respiratory monitoring using the "20/30/40 rule":
  • Vital capacity < 20 ml/kg
  • Maximum inspiratory pressure < 30 cmH₂O
  • Maximum expiratory pressure < 40 cmH₂O
  • Single breath count ≤ 19 predicts need for mechanical ventilation 1

Step 2: First-Line Immunotherapy

• IVIG (preferred option): 0.4 g/kg/day for 5 consecutive days 1, 3

  • Advantages: Greater availability, lower complication rates
  • Most effective when started within 2 weeks of symptom onset

• Plasma Exchange: Alternative if IVIG unavailable

  • 12-15 L in four to five exchanges over 1-2 weeks
  • Effective when started within 4 weeks of symptom onset
  • Requires specialized equipment 1, 3

Step 3: Management of Complications

• Pain management: Use gabapentinoids (pregabalin, gabapentin), tricyclic antidepressants, or carbamazepine for neuropathic pain 1, 3
• Respiratory support: Consider ICU admission for patients with respiratory compromise
• Autonomic dysfunction: Monitor and treat cardiac arrhythmias and blood pressure instability 2
• Rehabilitation: Arrange comprehensive rehabilitation program before discharge 1

Important Considerations and Caveats

1. Do NOT use:

   • Corticosteroids (oral or IV) - no benefit and potential negative effects 1, 3
   • Combination therapy (plasma exchange followed by IVIG) - no additional benefit 1
   • Opioids for pain management when possible 1

2. Treatment-related fluctuations (TRFs):

   • Occur in 6-10% of patients within 2 months of treatment
   • May require repeated IVIG treatment 1, 4

3. Watch for A-CIDP:

   • About 5% of patients initially diagnosed with GBS develop chronic inflammatory demyelinating polyradiculoneuropathy with acute onset
   • Consider this diagnosis if progression continues after 8 weeks from onset 1, 3

4. Prognosis monitoring:

   • Use modified Erasmus GBS outcome score (mEGOS) to predict recovery of walking ability
   • Use Erasmus GBS Respiratory Insufficiency Score (EGRIS) to assess risk of requiring mechanical ventilation 1, 3

Despite optimal treatment, GBS remains a serious condition with 3-10% mortality rate. Approximately 80% of patients regain independent walking by 6 months, but many experience long-term residual symptoms including pain, weakness, and fatigue 2, 1.

Special Populations

• Children: IVIG is preferred over plasma exchange due to lower complication rates 1
• Pregnant women: IVIG is preferred, though neither treatment is contraindicated 1

Early recognition, prompt treatment, and careful monitoring are essential to improve outcomes and reduce mortality in patients with Guillain-Barré Syndrome.