What is the treatment for Guillain Barré syndrome?

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 patient with GBS who cannot walk unaided, starting as early as possible within 2 weeks of symptom onset. 1

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, and has better tolerability with fewer complications—particularly important in children and pregnant women. 1

• For patients unable to walk unaided within 2 weeks of symptom onset: Administer IVIg 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg). 1, 2

• Alternative option: Plasma exchange (PE) at 200-250 ml plasma/kg body weight in five sessions over 1-2 weeks is equally effective for patients within 4 weeks of symptom onset who cannot walk unaided. 3, 2

• Do not combine PE followed immediately by IVIg—this approach is not recommended as it provides no additional benefit. 2

Critical Respiratory Monitoring

Respiratory failure develops in up to 30% of GBS patients and can occur rapidly without obvious dyspnea. 4, 5

• Apply the "20/30/40 rule" immediately: Patient is at imminent risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O. 1, 3, 4

• Single breath count ≤19 predicts need for mechanical ventilation—perform this simple bedside test regularly. 1, 3

• Monitor for use of accessory respiratory muscles and obtain arterial blood gas measurements if respiratory compromise is suspected. 3

ICU Admission Criteria

Admit to ICU if any of the following are present: 1, 3

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

What NOT to Use

• Corticosteroids alone are not recommended for GBS treatment as they have shown no significant benefit and may have negative effects on outcomes. 3, 4, 2

• Do not give a second IVIg course to patients with poor prognosis—this is not recommended based on current evidence. 2

• Avoid medications that worsen neuromuscular function: β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides. 3

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, 6

• For TRFs: Repeat the full course of IVIg or switch to PE, though evidence supporting this approach is limited. 1

• Consider changing diagnosis to acute-onset CIDP (A-CIDP) if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. 2, 6

Multidisciplinary Supportive Care

• Continuous cardiovascular monitoring: ECG for arrhythmias, blood pressure monitoring for hypertension/hypotension, as cardiovascular and respiratory dysfunction cause up to two-thirds of deaths in GBS. 1, 3, 4

• Monitor bowel and bladder function for autonomic dysfunction. 1, 3

• Assess muscle strength using Medical Research Council grading scale and document functional disability using GBS disability scale. 1, 3, 4

• Monitor for swallowing and coughing difficulties to prevent aspiration. 3, 4

Pain Management

Pain is common in GBS and significantly impacts quality of life—recognize and treat early. 1

• Weakly recommend gabapentinoids, tricyclic antidepressants, or carbamazepine for treatment of neuropathic pain. 2

Early Rehabilitation

• Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 1, 4

• Include range-of-motion exercises, stationary cycling, walking, and strength training. 1

• Monitor exercise intensity closely—overwork causes fatigue and should be avoided. 1

Prognosis

• 80% of patients regain independent walking ability at 6 months. 1

• Mortality is 3-10%, primarily from cardiovascular and respiratory complications. 1, 6, 7

• Risk factors for mortality include advanced age and severe disease at onset. 1

• About 40% of patients do not show improvement in the first 4 weeks following treatment. 4