What is the treatment for Guillain-Barré Syndrome?

Treatment of Guillain-Barré Syndrome

The first-line treatment for Guillain-Barré Syndrome (GBS) is intravenous immunoglobulin (IVIG) at 0.4 g/kg/day for 5 consecutive days or plasma exchange (4-5 exchanges over 1-2 weeks) for patients who cannot walk independently or have rapidly progressing symptoms. 1

Diagnostic Criteria and Assessment

• Progressive, typically symmetrical muscle weakness with reduced or absent deep tendon reflexes
• Often preceded by sensory symptoms or neuropathic pain in lower back and thighs
• May involve extremities, facial, respiratory, bulbar, and oculomotor nerves
• Autonomic nervous system dysfunction may be present

Essential Diagnostic Workup

1. Neurologic consultation
2. MRI of spine with/without contrast to rule out compressive lesions
3. Lumbar puncture (CSF typically shows elevated protein with normal WBC count)
4. Serum antiganglioside antibody tests (e.g., anti-GQ1b for Miller Fisher variant)
5. Electrodiagnostic studies to evaluate polyneuropathy
6. Pulmonary function testing
7. Frequent neurological assessments 2

Treatment Algorithm Based on Severity

Moderate GBS (Grade 2)

• Patient can walk but has symptoms interfering with activities of daily living
• Management:
  • Discontinue immune checkpoint inhibitors if applicable
  • Initiate IVIG (0.4 g/kg/day for 5 days) OR plasma exchange (4-5 exchanges over 1-2 weeks)
  • Monitor closely for respiratory compromise
  • Consider hospitalization for observation 2, 1

Severe GBS (Grade 3-4)

• Patient cannot walk independently, has respiratory compromise, or rapidly progressive symptoms
• Management:
  • Immediate admission to inpatient unit with capability for ICU transfer
  • Start IVIG (0.4 g/kg/day for 5 days) OR plasma exchange (4-5 exchanges over 1-2 weeks)
  • For immune checkpoint inhibitor-related GBS: Consider methylprednisolone (2-4 mg/kg/day)
  • Implement respiratory monitoring using the "20/30/40 rule":
    • Vital Capacity < 20 ml/kg
    • Maximum Inspiratory Pressure < 30 cmH₂O
    • Maximum Expiratory Pressure < 40 cmH₂O
  • Consider early intubation if respiratory parameters deteriorate
  • Frequent neurological assessments and pulmonary function monitoring 2, 1

Important Treatment Considerations

Immunotherapy Selection

• IVIG and plasma exchange are equally effective but IVIG is often preferred due to:
  • Greater availability
  • Easier administration
  • Fewer complications compared to plasma exchange 1, 3
• Combination therapy (plasma exchange followed by IVIG) shows no additional benefit 1
• Corticosteroids alone are not recommended as they show no benefit and may have negative effects 1, 3

Treatment Timing

• Immunotherapy should be initiated within 2 weeks of symptom onset for IVIG
• Plasma exchange can be effective if started within 4 weeks of symptom onset 3

Special Situations

• Treatment-related fluctuations (TRFs) occur in approximately 10% of patients within 8 weeks of initial treatment
  • Consider repeating the full course of IVIG or plasma exchange 4
• Approximately 5% of patients initially diagnosed with GBS develop acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP)
  • Consider this diagnosis if progression continues after 8 weeks from onset 4, 3

Supportive Care and Complication Management

Respiratory Support

• Approximately 30% of GBS patients develop respiratory failure requiring mechanical ventilation
• Monitor closely for respiratory compromise with regular pulmonary function tests 1, 5

Pain Management

• Pain affects at least one-third of patients and can be severe
• First-line treatment: Gabapentinoids (pregabalin, gabapentin)
• Alternative options: Tricyclic antidepressants, carbamazepine
• Avoid opioids when possible 1, 3

Autonomic Dysfunction

• Monitor for blood pressure fluctuations, heart rate abnormalities, and temperature dysregulation
• Treat dysautonomia symptomatically 1

Preventive Measures

• Implement standard preventive measures for:
  • Pressure ulcers
  • Hospital-acquired infections
  • Deep vein thrombosis 1

Rehabilitation

• Comprehensive rehabilitation program addressing:
  • Physical function restoration
  • Fatigue management
  • Chronic pain
  • Psychological support 1

Prognosis

• Approximately 80% of patients regain independent walking by 6 months
• Mortality rate: 3-10% due to cardiovascular and respiratory complications
• Long-term residual complaints (neuropathic pain, weakness, fatigue) affect 60-80% of patients 1, 6

Monitoring for Recovery

• Recovery may continue for more than 5 years after disease onset
• Regular follow-up to assess for residual symptoms and functional status
• Be alert for treatment-related fluctuations or development of A-CIDP 1, 4