What is the primary treatment for a patient diagnosed with Guillain-Barre Syndrome (GBS)?

Guillain-Barré Syndrome Treatment

Primary Treatment Recommendation

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) is the first-line treatment for patients with Guillain-Barré syndrome who cannot walk independently. 1, 2, 3

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When to Initiate Treatment

• Start IVIg immediately in patients with moderate to severe weakness (GBS disability score ≥3), rapid progression, or any signs of respiratory compromise, dysphagia, facial weakness, or bulbar weakness 1, 2
• Treatment is most effective when initiated within 2 weeks of symptom onset, though benefit may extend to 2-4 weeks 2, 3
• All grades warrant immediate workup and intervention given the potential for rapid progression to respiratory compromise 4

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Alternative First-Line Treatment

Plasma exchange (PE) is equally effective to IVIg and should be used when IVIg is contraindicated or unavailable 1, 2, 3:

• Dose: 200-250 mL/kg total plasma volume divided into 5 sessions over 1-2 weeks 2
• Timing: Most effective within 4 weeks of symptom onset 3
• Cost consideration: PE costs approximately $4,500-5,000 versus $12,000-16,000 for IVIg, which may be relevant in resource-limited settings 2

Why IVIg is Preferred Over PE

IVIg is generally chosen as first-line therapy because it is:

• Easier to administer 1, 2
• More widely available 2
• Has higher completion rates 1, 2
• Better tolerated with fewer complications, particularly in children 1, 2

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Critical Monitoring Requirements

Respiratory Assessment: The "20/30/40 Rule"

A patient is at high risk for respiratory failure requiring mechanical ventilation if: 2

• Vital capacity <20 mL/kg, OR
• Maximum inspiratory pressure <30 cmH₂O, OR
• Maximum expiratory pressure <40 cmH₂O

Approximately 20% of GBS patients will require mechanical ventilation 2

Admission and Monitoring

• Admit all patients to an inpatient unit with rapid transfer capability to ICU 4, 1, 5
• Perform frequent neurological assessments monitoring motor strength, reflexes, and bulbar symptoms 1
• Monitor for autonomic dysfunction including cardiovascular complications 2, 5
• Assess respiratory function regularly with vital capacity and maximum inspiratory/expiratory pressures 4, 5

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Medications to AVOID

The following medications can worsen neuromuscular function and must be avoided: 4, 1, 2

• β-blockers
• IV magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolide antibiotics

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What NOT to Use

Corticosteroids Are Not Recommended

• Do not use oral corticosteroids as they have shown no benefit and may have negative effects 1, 3
• IV corticosteroids are weakly recommended against in typical idiopathic GBS 3
• Exception: In immune checkpoint inhibitor-related GBS, corticosteroids may be considered alongside IVIg or PE (methylprednisolone 2-4 mg/kg/day or pulse dosing 1 g daily for 5 days) 4

Sequential Therapy Not Recommended

• Do not use PE followed immediately by IVIg, as plasma exchange will remove the immunoglobulin 4, 3

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Management of Treatment Non-Response

Understanding Expected Response

• 40% of patients do not improve in the first 4 weeks following treatment—this does NOT necessarily indicate treatment failure 2, 5
• Recovery can continue for more than 5 years after disease onset 4, 2

Treatment-Related Fluctuations (TRFs)

• TRFs occur in 6-10% of patients within 2 months of initial improvement 4, 2, 5
• Repeating the full course of IVIg or switching to PE is common practice for TRFs, though evidence is limited 4, 2, 5
• Do not give a second IVIg course in patients with poor prognosis who have not shown initial response 3

When to Consider Plasma Exchange After IVIg Failure

In severe, refractory cases with progressive clinical deterioration despite IVIg, plasma exchange should be considered early, particularly in cases with axonal involvement 6

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Special Populations

Children

• IVIg is strongly preferred over PE in pediatric patients (0.4 g/kg daily for 5 days) due to better tolerability and fewer complications 1, 2
• Use the standard 5-day regimen rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter courses 1

Pregnant Women

• Both IVIg and PE are not contraindicated in pregnancy 1
• IVIg is generally preferred due to fewer monitoring requirements and considerations 1

Miller Fisher Syndrome

• Treatment is generally not recommended as most patients recover completely within 6 months without intervention 1
• Close monitoring is essential 1

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Essential Supportive Care

Pain Management

• Use non-opioid neuropathic pain medications: gabapentin, pregabalin, or duloxetine 4, 2, 3
• Severe pain occurs in at least one-third of patients at 1 year and can persist for >10 years 4

Preventive Measures

• DVT prophylaxis is essential due to immobility 2, 5
• Pressure ulcer prevention through regular repositioning 2, 5
• Prevention of hospital-acquired infections (pneumonia, UTIs) 2
• Treatment of constipation/ileus, which is common in GBS patients 4

Nutritional Support

• Evaluate for dysphagia and provide nutritional support if necessary 1

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Diagnostic Workup

Before or concurrent with treatment initiation 4, 5:

• Neurology consultation (immediate)
• MRI spine with and without contrast to rule out compressive lesions and evaluate for nerve root enhancement
• Lumbar puncture: CSF analysis typically shows elevated protein with normal or mildly elevated WBC (albuminocytologic dissociation)
• Serum antiganglioside antibody tests for GBS subtypes (e.g., anti-GQ1b for Miller Fisher variant)
• Electrodiagnostic studies (nerve conduction studies and EMG) to evaluate polyneuropathy
• Pulmonary function testing (negative inspiratory force or vital capacity)

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Prognosis

• 80% of patients regain walking ability at 6 months after disease onset 4, 2, 5
• Mortality is 3-10%, most commonly from cardiovascular and respiratory complications 4, 2, 5
• Risk factors for mortality include advanced age, severe disease at onset, and lack of ICU support when needed 2
• Recurrence is rare (2-5%) but higher than general population lifetime risk (0.1%) 4, 2
• Long-term residual complaints include neuropathic pain, weakness, and fatigue 4, 2, 5

Prognostic Tools

• Use the modified Erasmus GBS Outcome Score (mEGOS) to predict walking ability at 6 months 4, 5
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to calculate probability of requiring ventilation 1, 5

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Rehabilitation Planning

• Arrange rehabilitation with a specialist, physiotherapist, and occupational therapist before discharge 4
• Exercise programs including range-of-motion exercises, stationary cycling, walking, and strength training improve physical fitness and independence 4
• Monitor exercise intensity closely as overwork can cause fatigue 4
• Graded, supervised exercise programs are useful for reducing fatigue, which affects 60-80% of patients 4