Guillain-Barré Syndrome: Comprehensive Management Guide
Initial Treatment
Intravenous immunoglobulin (IVIg) at 0.4 g/kg body weight daily for 5 consecutive days is the recommended first-line treatment for Guillain-Barré syndrome in patients unable to walk unaided within 2 weeks of symptom onset. 1, 2, 3
Treatment Selection Algorithm
For adults unable to walk independently:
- First-line: IVIg 0.4 g/kg/day × 5 days (total 2 g/kg over 5 days) 1, 2, 3
- Alternative: Plasma exchange (PE) 200-250 mL plasma/kg over 4-5 sessions across 1-2 weeks if IVIg contraindicated or unavailable 3
- Timing: Initiate within 2 weeks of weakness onset (up to 4 weeks for PE) 3
For children:
- IVIg is strongly preferred over PE due to better tolerability, fewer complications, and easier administration 4, 5
- Use the standard 5-day regimen (0.4 g/kg/day × 5 days) rather than 2-day regimen, as treatment-related fluctuations occur more frequently with shorter courses (5/23 vs 0/23 children) 4
IVIg is preferred over PE because:
- Easier to administer and more widely available 1, 2
- Higher treatment completion rates 1, 6
- Comparable efficacy with no significant difference in disability improvement (mean difference 0.02 grade on 7-point scale, 95% CI -0.20 to 0.25) 6
- Lower complication rates, particularly in children 4, 7
What NOT to Use
- Do not use corticosteroids alone - randomized trials show no benefit and oral corticosteroids may worsen outcomes 2, 3
- Do not routinely combine PE followed immediately by IVIg - one trial with 249 participants showed only 0.2 grade improvement (95% CI -0.14 to 0.54), not clinically significant 4, 3
- Avoid medications that worsen neuromuscular function: β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides 1, 2
Critical Monitoring Requirements
Respiratory Assessment (Use the "20/30/40 Rule")
Patient is at risk of respiratory failure if ANY of the following:
- Vital capacity <20 mL/kg 4, 1, 3
- Maximum inspiratory pressure <30 cmH₂O 4, 1, 3
- Maximum expiratory pressure <40 cmH₂O 4, 1, 3
- Single breath count ≤19 (predicts need for mechanical ventilation) 4, 1
- Use of accessory respiratory muscles 4, 5
Key facts:
- Approximately 20-25% of patients require mechanical ventilation 1, 8
- Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to calculate individual ventilation probability 2, 3
Autonomic Dysfunction Monitoring
Monitor continuously via:
- Electrocardiography for arrhythmias 4, 5
- Heart rate and blood pressure for fluctuations 4, 5
- Bowel and bladder function 4, 5
Critical warning: Up to two-thirds of GBS deaths occur during the recovery phase due to cardiovascular and respiratory complications 4, 5. Maintain vigilance even after ICU discharge, especially in patients with cardiovascular risk factors 4.
Neurological Assessment
- Measure muscle strength using Medical Research Council grading scale in neck, arms, and legs 4
- Document functional disability using GBS disability scale 4
- Monitor for swallowing and coughing difficulties to prevent aspiration 4, 5
- Assess for bulbar palsy and facial palsy 4
Managing Treatment Response and Complications
Insufficient Initial Response (40% of patients)
About 40% of patients do not improve in the first 4 weeks following treatment - this does NOT necessarily mean treatment failed, as progression might have been worse without therapy 4, 1, 2. Currently, no evidence supports repeating treatment or switching therapies in this scenario 4.
Treatment-Related Fluctuations (TRFs)
Definition: Disease progression within 2 months after initial treatment-induced improvement or stabilization, occurring in 6-10% of patients 4, 1, 2
Management approach:
- Repeat the full course of IVIg (0.4 g/kg/day × 5 days) or PE 4, 1, 5
- This is common practice despite limited supporting evidence 4, 1
- TRFs indicate the inflammatory phase is ongoing while treatment effect has worn off 4
Acute-Onset CIDP
Consider diagnosis change to acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) if:
- Three or more TRFs occur 4
- Clinical deterioration continues ≥8 weeks after disease onset 4, 3
- This occurs in approximately 5% of patients initially diagnosed with GBS 4, 3, 8
Complication Management
GBS-Specific Complications
Bulbar dysfunction:
- Inability to swallow safely requires alternative feeding routes 4
- Monitor coughing ability to prevent aspiration 4, 5
Facial palsy:
- Watch for corneal ulceration and provide eye protection 4
Limb weakness complications:
- Prevent limb contractures, ossification, and pressure palsies 4
- Initiate early range-of-motion exercises 5
Universal Hospital Complications
Standard preventive measures for:
- Pressure ulcers 4, 5
- Hospital-acquired pneumonia and urinary tract infections 4, 5
- Deep vein thrombosis 4, 5
Pain and Psychological Symptoms
Pain is common and frequently underrecognized - actively ask patients about pain, especially those with limited communication abilities or in ICU 4
For neuropathic pain management:
- Weakly recommend gabapentinoids, tricyclic antidepressants, or carbamazepine 3
- Encourage mobilization as tolerated 5
Psychological support is crucial:
- Anxiety, depression, and hallucinations are frequent 4, 5
- Patients with complete paralysis usually have intact consciousness, vision, and hearing 4
- Be mindful of bedside conversations and explain all procedures to reduce anxiety 4
Multidisciplinary Care Requirements
Essential team members:
- Nurses for continuous monitoring 4
- Physiotherapists for mobility and strength training 4, 5
- Rehabilitation specialists for recovery planning 4, 5
- Occupational therapists for activities of daily living 4, 5
- Speech therapists for swallowing assessment 4
- Dietitians for nutritional support 4
Rehabilitation program should include:
- Range-of-motion exercises 5
- Stationary cycling and walking as tolerated 5
- Strength training 5
- Caution: Monitor exercise intensity closely as overwork can cause severe fatigue 5
Prognosis and Long-Term Outcomes
Recovery Timeline
- 80% of patients regain independent walking ability at 6 months 4, 1, 2
- Recovery can continue for more than 5 years after disease onset 4, 1, 5
- Use the modified Erasmus GBS Outcome Score (mEGOS) to predict individual walking ability 4, 3
Mortality and Severe Outcomes
- Mortality: 3-10% of cases, primarily from cardiovascular and respiratory complications 4, 1, 2
- Risk factors for death: advanced age and severe disease at onset 4, 2
- Approximately 25% require artificial ventilation for days to months 8
- About 20% remain unable to walk at 6 months 8
Long-Term Residual Symptoms
Common persistent complaints:
Recurrence:
Diagnostic Considerations
Clinical Features Increasing GBS Likelihood
- Recent diarrhea or respiratory infection 3
- Progressive flaccid paralysis reaching maximum severity within 4 weeks 3, 8
Supportive Testing
CSF examination:
- Valuable, particularly when diagnosis is uncertain 3
Electrodiagnostic testing:
- Advised to support diagnosis 3
- Helps distinguish AIDP (most common in Western countries) from AMAN (most common in Asia/Japan) 8
Anti-ganglioside antibody testing:
- Limited clinical value in typical motor-sensory GBS 3
- Test anti-GQ1b antibodies when Miller Fisher syndrome suspected 3
- Test nodal-paranodal antibodies when autoimmune nodopathy suspected 3
Imaging:
- Consider MRI or ultrasound in atypical cases 3
Special Populations
Pregnant Women
- Both IVIg and PE are not contraindicated 2
- IVIg generally preferred due to fewer monitoring requirements 2