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

Treatment of Guillain-Barré Syndrome

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg body weight daily for 5 consecutive days as first-line treatment for any GBS patient who cannot walk unaided, starting as early as possible within 2 weeks of symptom onset. 1, 2

First-Line Immunotherapy

IVIg is the preferred first-line treatment over plasma exchange because it is easier to administer, more widely available, achieves higher completion rates, and has better tolerability with fewer complications—particularly critical in children and pregnant women. 1

Treatment Options:

• IVIg: 0.4 g/kg/day for 5 consecutive days for patients unable to walk unaided within 2 weeks of onset 1, 2, 3
• Plasma Exchange (PE): 200-250 mL/kg in 4-5 sessions over 1-2 weeks, effective alternative within 4 weeks of onset if IVIg is contraindicated or unavailable 2, 3
• Corticosteroids alone are NOT recommended as they show no significant benefit and may have negative effects 2, 3
• Sequential PE followed immediately by IVIg is NOT recommended 3

Critical Respiratory Monitoring

Apply the "20/30/40 rule" to identify imminent respiratory failure risk: 1

• 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 Monitor for use of accessory respiratory muscles and inability to count to 20 in one breath. 2

ICU Admission Criteria

Admit to ICU if ANY of the following are present: 1

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

Managing Treatment Failures and Fluctuations

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, 4

For TRFs, repeat the full course of IVIg or switch to PE, though evidence supporting this is limited. 1, 4 Note that approximately 40% of patients do not show improvement in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness. 4

Consider changing the diagnosis to acute-onset CIDP (A-CIDP) if progression continues after 8 weeks from onset or if repeated relapses occur, which happens in approximately 5% of cases initially diagnosed as GBS. 4, 3

Multidisciplinary Supportive Care

Autonomic Monitoring:

• Continuous ECG monitoring for arrhythmias 1
• Blood pressure monitoring for hypertension/hypotension 1
• Monitor bowel and bladder function 1
• Up to two-thirds of deaths occur during recovery phase due to cardiovascular and respiratory dysfunction 4

Functional Assessment:

• Assess muscle strength using Medical Research Council grading scale 1
• Document functional disability using GBS disability scale 1

Complication Prevention:

• Prevent pressure ulcers, deep vein thrombosis, and hospital-acquired infections 2, 4
• Monitor swallowing to prevent aspiration 2, 4

Pain Management:

Pain is common and significantly impacts quality of life—recognize and treat early. 1 Severe pain is reported in at least one-third of patients 1 year after onset and can persist for >10 years. 5

Weakly recommend gabapentinoids, tricyclic antidepressants, or carbamazepine for neuropathic pain. 3 Management strategies include encouraging mobilization and administering drugs for neuropathic or nociceptive pain. 5

Psychological Support:

Address anxiety, depression, and hallucinations which are frequent in GBS patients. 2, 4 Providing accurate information on the relatively good chance of recovery and low recurrence risk (2-5%) can help reduce fear. 5

Rehabilitation

Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 1, 2

Exercise Program Components:

• Range-of-motion exercises 5, 2
• Stationary cycling 5, 2
• Walking and strength training 5, 2

Monitor exercise intensity closely—overwork causes fatigue. 5, 2 Fatigue unrelated to residual motor deficits is found in 60-80% of patients and is often one of the most disabling complaints. 5

Prognosis

• 80% of patients regain independent walking ability at 6 months 1, 2
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 2
• Risk factors for mortality: advanced age and severe disease at onset 1, 2
• Recovery can continue for more than 5 years after disease onset 2, 4
• Use the modified Erasmus GBS outcome score (mEGOS) to predict walking ability 2, 3
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess risk of requiring artificial ventilation 3