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

Treatment of Guillain-Barré Syndrome

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days as first-line treatment for any GBS patient who cannot walk unaided or shows signs of respiratory compromise, dysphagia, facial weakness, or bulbar weakness. 1, 2, 3

First-Line Immunotherapy

• IVIg is the preferred first-line treatment over plasma exchange because it is easier to administer, more widely available, has higher completion rates (patients are significantly more likely to complete IVIg than PE), and has better tolerability with fewer complications—particularly critical in children and pregnant women. 1, 2, 4

• Start treatment as early as possible within 2 weeks of symptom onset to maximize effectiveness. 2

• The standard regimen is 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg). 1, 2, 3

• In children, use the same 5-day regimen rather than accelerated 2-day protocols, as treatment-related fluctuations occurred more frequently with shorter regimens. 1

• Corticosteroids alone are NOT recommended for GBS treatment—randomized controlled trials show no significant benefit and oral corticosteroids may even worsen outcomes. 1, 5

Critical Respiratory Monitoring

Apply the "20/30/40 Rule" to identify imminent respiratory failure risk: 2, 3

• Vital capacity <20 ml/kg

• Maximum inspiratory pressure <30 cmH₂O

• Maximum expiratory pressure <40 cmH₂O

• Single breath count ≤19 predicts need for mechanical ventilation. 2

• Approximately 20% of GBS patients require mechanical ventilation. 3

• Admit patients to a unit with rapid ICU transfer capability for close monitoring. 1, 2

ICU Admission Criteria

Admit to ICU if ANY of the following are present: 2

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

Medications to AVOID

Do NOT use the following medications in GBS patients, as they worsen neuromuscular function: 1, 3

• β-blockers
• IV magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolides

Managing Treatment Failures and Fluctuations

• About 40% of patients do not improve in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness. 1

• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement and represent disease reactivation while the inflammatory phase continues. 1, 2

• For TRFs, repeat the full 5-day course of IVIg (0.4 g/kg/day) or switch to plasma exchange. 1, 2

• Patients with only minor increases in serum IgG levels after standard IVIg treatment recover significantly slower and may benefit from a second IVIg dose. 6

• For severe axonal GBS variants with no improvement after initial IVIg, consider a second cycle using the same dose and regimen after 6 weeks. 7

Essential Supportive Care

Pain management (neuropathic pain is common and significantly impacts quality of life): 2, 3

• Use gabapentinoids (gabapentin, pregabalin)
• Tricyclic antidepressants
• Duloxetine
• Carbamazepine
• Avoid opioids for neuropathic pain 1, 3

Continuous monitoring requirements: 2

• ECG monitoring for arrhythmias
• Blood pressure monitoring for hypertension/hypotension
• Bowel and bladder function monitoring
• Address constipation/ileus (common in GBS) 1

Assess and document: 2

• Muscle strength using Medical Research Council grading scale
• Functional disability using GBS disability scale

Prophylaxis: 1

• Deep vein thrombosis prophylaxis
• Pressure ulcer prevention
• Evaluate for dysphagia and provide nutritional support if necessary

Rehabilitation

• Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 2, 3

• Include range-of-motion exercises, stationary cycling, walking, and strength training. 2

• Monitor exercise intensity closely—overwork causes fatigue. 2

Special Populations

Children: 1

• IVIg is preferred over plasma exchange due to better tolerability and fewer complications
• Use the standard 5-day regimen (0.4 g/kg/day for 5 days)

Pregnant women: 1

• Both IVIg and plasma exchange are not contraindicated
• IVIg is generally preferred due to fewer monitoring requirements

Miller-Fisher Syndrome: 1

• Treatment is generally not recommended as most recover completely within 6 months without intervention
• Close monitoring is essential

Immune checkpoint inhibitor-related GBS: 1

• Discontinue the causative agent permanently
• Consider concurrent corticosteroids with IVIg or plasma exchange

Prognosis

• 80% of patients regain independent walking ability at 6 months after disease onset. 2, 3

• Mortality is 3-10%, primarily from cardiovascular and respiratory complications. 1, 2, 3

• Risk factors for mortality: advanced age and severe disease at onset. 1, 2

• Recovery can continue for more than 5 years after disease onset. 3

• Recurrence is rare (2-5%). 3