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 patient with GBS 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, has higher completion rates, and demonstrates better tolerability with fewer complications—particularly critical in children and pregnant women 1, 2
• Plasma exchange (200-250 ml plasma/kg body weight in five sessions) is an equally effective alternative if IVIg is unavailable or contraindicated 1, 3
• Treatment must be initiated as early as possible in the disease course to maximize effectiveness, ideally within 2 weeks of symptom onset 1, 2
• Do not use corticosteroids alone—randomized controlled trials demonstrate no significant benefit, and oral corticosteroids may worsen outcomes 1, 3

Critical Respiratory Monitoring and ICU Admission

• 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 3, 2
• Single breath count ≤19 predicts need for mechanical ventilation 3, 2
• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to calculate probability of requiring ventilation 1, 3
• Admit to ICU if any of the following are present: evolving respiratory distress with imminent respiratory insufficiency, severe autonomic cardiovascular dysfunction, severe swallowing dysfunction or diminished cough reflex, or rapid progression of weakness 3, 2
• Up to 30% of patients develop respiratory failure during hospitalization and require mechanical ventilation 4

Medications to Avoid

• Avoid β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides—these medications worsen neuromuscular function and can exacerbate the clinical condition 1, 3

Managing Treatment-Related Fluctuations

• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement, representing disease reactivation while the inflammatory phase continues 1, 2
• For TRFs, repeat the full course of IVIg (0.4 g/kg/day for 5 days) or switch to plasma exchange 1, 2
• Do not give a second IVIg course to patients with poor prognosis who have not experienced TRFs—evidence shows no benefit 5

Special Populations

• In children: use the same 5-day IVIg regimen (0.4 g/kg/day for 5 days) rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 1
• In pregnant women: IVIg is preferred over plasma exchange because it requires fewer monitoring considerations, though neither treatment is contraindicated during pregnancy 1
• For Miller-Fisher Syndrome: treatment is generally not recommended as most patients recover completely within 6 months without intervention, though close monitoring is essential 1

Multidisciplinary Supportive Care

• Perform continuous ECG monitoring for arrhythmias, blood pressure monitoring for hypertension/hypotension, and monitor bowel and bladder function 2
• Assess muscle strength using Medical Research Council grading scale and document functional disability using GBS disability scale 3, 2
• Manage neuropathic pain with gabapentin, pregabalin, or duloxetine—pain is common and significantly impacts quality of life 1, 2
• Provide prophylaxis for deep vein thrombosis and pressure ulcers 1
• Evaluate for dysphagia and provide nutritional support if necessary 1, 3

Rehabilitation

• Initiate early rehabilitation with physiotherapists, occupational therapists, speech therapists, and dietitians 2
• Include range-of-motion exercises, stationary cycling, walking, and strength training 2
• Monitor exercise intensity closely—overwork causes fatigue 2

Prognosis and Monitoring

• 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 include advanced age and severe disease at onset 1, 2
• About 40% of patients do not improve in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness 1

Critical Pitfalls to Avoid

• Respiratory failure can develop rapidly without obvious clinical signs of dyspnea—maintain vigilant respiratory monitoring even in patients who appear stable 3
• Verify serum IgA levels before the first IVIg infusion, as IgA deficiency increases the risk of anaphylaxis 1
• Consider changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS 3