What is the treatment for Guillain-Barre Syndrome?

Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days is the first-line treatment for Guillain-Barré syndrome and should be initiated immediately in patients with moderate to severe weakness, rapid progression, or any signs of respiratory compromise, dysphagia, facial weakness, or bulbar symptoms. 1, 2

Why IVIg is Preferred Over Plasma Exchange

IVIg is the preferred first-line therapy because it is easier to administer, more widely available, has higher completion rates, and requires fewer monitoring considerations compared to plasma exchange, despite both treatments being equally effective. 1

• Plasma exchange (200-250 mL/kg for 5 sessions) remains an equally effective alternative and should be used if IVIg is unavailable or contraindicated 1, 2
• In children, IVIg is strongly preferred over plasma exchange due to better tolerability, fewer complications, and reduced need for pediatric intensive care unit admission 1, 3
• In pregnant women, IVIg is preferred because it requires fewer monitoring considerations, though neither treatment is contraindicated during pregnancy 1

Critical Initial Assessment and Monitoring

Immediately assess respiratory function and autonomic stability upon presentation, as these determine mortality risk and need for ICU-level care. 4

Respiratory Monitoring

• Apply the "20/30/40 rule" at presentation and serially: patient is at risk of respiratory failure if vital capacity <20 mL/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1, 4
• Single breath count ≤19 predicts need for mechanical ventilation 4
• Admit patients to a unit with rapid transfer capability to ICU, as respiratory compromise can occur even during treatment 1, 4

Neurological Assessment

• Grade muscle strength using Medical Research Council scale in neck, arms, and legs 4
• Assess functional disability using GBS disability scale 4
• Test swallowing and coughing ability to identify aspiration risk 4
• Check for facial weakness, ophthalmoplegia, and corneal reflex in patients with facial palsy to prevent corneal ulceration 4

Autonomic Monitoring

• Perform electrocardiography and continuously monitor heart rate and blood pressure for arrhythmias and blood pressure instability 4

Medications to Avoid During Treatment

Avoid medications that worsen neuromuscular function: β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides, as they can exacerbate the clinical condition. 1, 2

Treatment Monitoring and Adverse Reactions

• Monitor patients rigorously during and after each IVIg infusion for neurological function (motor strength, reflexes, bulbar symptoms) and potential adverse reactions 1
• Verify serum IgA levels before the first infusion, as IgA deficiency increases anaphylaxis risk; use preparations with reduced IgA levels if deficiency is confirmed 1

Management of Treatment Failure and Fluctuations

About 40% of patients do not improve in the first 4 weeks following treatment, which does not necessarily indicate treatment ineffectiveness. 1, 2

• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1
• For TRFs, repeat the full course of IVIg or plasma exchange 1
• In refractory cases where IVIg fails, consider plasma exchange early, particularly in patients with axonal involvement, as some severe cases may benefit from plasma exchange after failed IVIg treatment 5

Special Considerations

Miller-Fisher Syndrome Variant

• Treatment is generally not recommended for Miller-Fisher syndrome (ophthalmoplegia, ataxia, areflexia), as most patients recover completely within 6 months without intervention, though close monitoring is essential 1

Pediatric Dosing

• Use the same 5-day regimen (0.4 g/kg/day for 5 days) in children rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 1

Immune Checkpoint Inhibitor-Related GBS

• Discontinue the causative agent permanently and consider concurrent corticosteroids with IVIg or plasma exchange 1

What NOT to Do

Do not use corticosteroids alone for GBS treatment, as randomized controlled trials have shown no significant benefit and oral corticosteroids may have negative effects on outcomes. 1

• Do not wait for antibody test results before starting treatment if GBS is suspected 4
• Do not dismiss GBS based on normal CSF protein in the first week, as albumino-cytological dissociation may develop later 4

Supportive Care

• Manage neuropathic pain with gabapentin, pregabalin, or duloxetine; avoid opioids 1
• Provide prophylaxis for deep vein thrombosis and pressure ulcers 1
• Evaluate for dysphagia and provide nutritional support if necessary 1
• Address constipation/ileus, which is common in GBS patients 1
• Screen for anxiety, depression, and hallucinations, which are frequent complications 4

Prognosis

• About 80% of patients regain walking ability at 6 months after disease onset 1, 2
• Recovery can continue for more than 3 years, with full recovery expected in approximately 90% of cases 4
• Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications; risk factors include advanced age and severe disease at onset 1, 2