Treatment of Acute Demyelinating Polyradiculoneuropathy (Guillain-Barré Syndrome)
Intravenous immunoglobulin (IVIg) 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) is the first-line treatment for patients with GBS who cannot walk independently within 2 weeks of symptom onset. 1, 2
First-Line Immunotherapy Options
IVIg and plasma exchange are equally effective treatments, but IVIg is generally preferred due to better tolerability and higher completion rates. 3, 1
IVIg Protocol
- Dosing: 0.4 g/kg/day for 5 consecutive days (total 2 g/kg) 1, 2
- Timing: Initiate within 2 weeks of weakness onset for patients unable to walk unaided 1, 2
- Advantages: Higher completion rates, better tolerability, fewer complications, particularly important in children and pregnant women 1
Plasma Exchange Protocol
- Dosing: 4-6 sessions of 12-15 L exchanges over 1-2 weeks 1, 4, 2
- Timing: Initiate within 4 weeks of weakness onset for patients unable to walk unaided 2
- Evidence: 4 sessions are effective; 6 sessions provide no additional benefit over 4 4
What NOT to Do
Corticosteroids alone are NOT recommended for idiopathic GBS and do not alter disease outcome. 1, 2, 5
- Oral corticosteroids: Not recommended 2
- IV corticosteroids: Weakly recommended against 2
- Exception: Corticosteroids (methylprednisolone 2-4 mg/kg/day) may be considered specifically for immune checkpoint inhibitor-related GBS 3, 1
Do NOT combine plasma exchange followed immediately by IVIg—this combination is not recommended. 2
Critical Monitoring Requirements
Respiratory Monitoring
All patients require frequent respiratory function monitoring as respiratory failure can occur without dyspnea symptoms. 3, 1
- Use the "20/30/40 rule" to assess respiratory failure risk: 1
- Vital capacity <20 mL/kg
- Maximum inspiratory pressure <30 cmH₂O
- Maximum expiratory pressure <40 cmH₂O
- Approximately 20% of patients develop respiratory failure requiring mechanical ventilation 3
Autonomic Monitoring
Monitor for autonomic dysfunction including cardiac arrhythmias, blood pressure instability, and bowel/bladder function. 1
- Autonomic involvement contributes significantly to the 3-10% mortality rate 3, 1
- Two-thirds of deaths occur during the recovery phase, requiring continued vigilance even after apparent improvement 1
Management of Treatment Failure and Fluctuations
Treatment-Related Fluctuations (TRFs)
TRFs occur in 6-10% of patients within 2 months of initial improvement, indicating the treatment effect has worn off while inflammation continues. 1, 4, 6
- Management: Consider repeating the full course of IVIg or plasma exchange 1, 4
- Important distinction: 40% of patients show no improvement in the first 4 weeks—this does NOT indicate treatment failure, as progression might have been worse without therapy 1, 4
Poor Initial Response
Do NOT give a second IVIg course to GBS patients with a poor prognosis who have not shown initial response—evidence does not support this practice. 2
Diagnostic Revision
If progression continues beyond 8 weeks from onset, or if three or more TRFs occur, consider changing the diagnosis to acute-onset CIDP (A-CIDP), which occurs in approximately 5% of patients initially diagnosed with GBS. 4, 2, 6
Supportive Care and Complication Prevention
Pain Management
For neuropathic pain, use gabapentinoids, tricyclic antidepressants, or carbamazepine. 2
- Pregabalin, gabapentin, or duloxetine are recommended 1
- Avoid opioids for neuropathic pain management 1
Preventive Measures
Implement protocols for pressure ulcer prevention, hospital-acquired infection prevention, and DVT prophylaxis. 1
Medications to Avoid
Avoid medications that worsen neuromuscular function: 1
- β-blockers
- IV magnesium
- Fluoroquinolones
- Aminoglycosides
- Macrolides
Specific Complications
Address bulbar palsy (inability to swallow), facial palsy (corneal ulceration risk), and limb contractures. 1
IVIg Safety Considerations
Common Infusion Reactions
IVIg causes self-limited infusion reactions including chills, tachycardia, hypertension, muscle pains, fever, nausea, and headache—manage by slowing the infusion rate. 7
Life-Threatening Complications
Monitor for two FDA-warned complications: 7
- Thrombosis: Myocardial infarction, stroke, pulmonary embolism, deep vein thrombosis
- Renal failure: Particularly in patients with pre-existing renal insufficiency, diabetes, volume depletion, sepsis, or those receiving nephrotoxic drugs
High-Risk Patients
Patients at higher risk for IVIg complications include those with: 7
- Pre-existing renal disease
- Advanced age
- Diabetes mellitus
- Volume depletion
- Concurrent nephrotoxic medications
Religious Considerations
IVIg may not be acceptable to Jehovah's Witnesses and other patients who refuse blood products, as it is derived from pooled human plasma. 7
Prognosis and Recovery Timeline
Most patients reach maximum disability within 2 weeks, defining the critical treatment window. 3, 4
- 60-80% of patients walk independently at 6 months after disease onset 3, 4
- Approximately 80% regain walking ability at 6 months 1
- Clinical improvement is most extensive in the first year but can continue for >5 years 3, 4
- Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 1
Prognostic Tools
Use the modified Erasmus GBS Outcome Score (mEGOS) to assess outcome and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. 2
Common Pitfalls to Avoid
Do not delay treatment waiting for diagnostic confirmation—initiate immunotherapy promptly within 2 weeks if clinical suspicion is high and the patient cannot walk independently 1, 2
Do not assume dyspnea will precede respiratory failure—respiratory function can deteriorate rapidly without warning symptoms 3, 1
Do not discontinue monitoring after initial improvement—two-thirds of deaths occur during the recovery phase 1
Do not confuse lack of early improvement with treatment failure—40% of patients do not improve in the first 4 weeks, which doesn't indicate treatment ineffectiveness 1, 4
Do not use corticosteroids as monotherapy for typical GBS—they are ineffective and not recommended 1, 2, 5