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

Immediate Treatment for Guillain-Barré Syndrome (GBS)

Intravenous immunoglobulin (IVIg) at a dose of 0.4 g/kg body weight daily for 5 days is the recommended first-line treatment for Guillain-Barré Syndrome and should be initiated as early as possible in the disease course. 1

First-Line Treatment Options

• IVIg (0.4 g/kg daily for 5 days) is preferred over plasma exchange due to easier administration, wider availability, and higher completion rates 1, 2
• The 5-day IVIg regimen is preferred over a 2-day regimen, as treatment-related fluctuations were more frequent with the 2-day regimen 3
• Plasma exchange (200-250 ml plasma/kg body weight in five sessions over 2 weeks) is an effective alternative when IVIg is contraindicated, not tolerated, or unavailable 1, 4
• Treatment should be initiated as early as possible to maximize effectiveness, without waiting for diagnostic confirmation if GBS is strongly suspected 1, 2
• Corticosteroids alone are not recommended for GBS treatment as they have shown no significant benefit and may even have negative effects on outcomes 1, 3

Patient Assessment and Monitoring

• Regular respiratory function assessment is essential using the "20/30/40 rule": 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, 3
• The Erasmus GBS Respiratory Insufficiency Score (EGRIS) should be used to calculate probability of requiring ventilation 1
• Monitor for single breath count, use of accessory respiratory muscles, and ability to cough 3
• Swallowing and coughing difficulties should be assessed to prevent aspiration 2, 3
• Autonomic dysfunction should be monitored via electrocardiography, heart rate, blood pressure, and bowel/bladder function 2, 3

Management of Disease Progression

• Approximately 40% of patients do not show improvement in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness 1, 2
• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement 1, 3
• For patients with TRFs, repeating the full course of IVIg or switching to plasma exchange is common practice 2
• In approximately 5% of cases initially diagnosed as GBS, the diagnosis may change to acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) if repeated relapses occur 2, 5

Special Patient Populations

• In children, IVIg is preferred over plasma exchange due to better tolerability and fewer complications 1, 2
• Young children (<6 years) may present with nonspecific or atypical clinical features such as poorly localized pain, refusal to bear weight, irritability, meningism, or unsteady gait, which can delay diagnosis 3
• In pregnant women, both IVIg and plasma exchange are not contraindicated, but IVIg is generally preferred due to fewer monitoring requirements 1

Supportive Care and Complication Management

• Multidisciplinary supportive care is crucial and should include pain management, as pain is common in GBS patients 2, 3
• Prevention of pressure ulcers, hospital-acquired infections (pneumonia, urinary tract infections), and deep vein thrombosis is essential 2, 3
• Psychological support for anxiety, depression, and hallucinations which are frequent in GBS patients is crucial 2, 3
• Patients should be monitored for cardiovascular complications, as up to two-thirds of deaths in GBS occur during the recovery phase due to cardiovascular and respiratory dysfunction 2, 3

Prognosis

• About 80% of patients regain walking ability at 6 months after disease onset 1, 3
• Recovery can continue for more than 3 years after onset 3
• Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 1
• Risk factors for mortality include advanced age and severe disease at onset 1
• Recurrence of GBS is rare (2-5%) 3, 5

Common Pitfalls and Caveats

• Do not delay treatment while waiting for diagnostic confirmation if GBS is strongly suspected 1, 2
• Do not rely on corticosteroids alone for treatment 1, 3
• Do not underestimate the importance of respiratory monitoring - up to 30% of patients develop respiratory failure requiring mechanical ventilation 6
• The risk of treatment discontinuation is significantly lower with IVIg than with plasma exchange, which may influence treatment choice 4
• Even though IVIg is generally preferred, plasma exchange remains effective and should be considered when IVIg is unavailable or contraindicated 4, 7