Guillain-Barré Syndrome: Immediate Treatment
Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) as first-line treatment, while simultaneously ensuring respiratory monitoring and ICU-level care capability. 1, 2
Critical Immediate Actions
Respiratory Assessment (Priority #1)
- Measure vital capacity immediately - respiratory failure is the leading cause of death and can develop rapidly without obvious dyspnea 1
- Apply the "20/30/40 rule": patient is at risk if:
- Vital capacity <20 ml/kg, OR
- Maximum inspiratory pressure <30 cmH₂O, OR
- Maximum expiratory pressure <40 cmH₂O 1
- Perform single breath count (≤19 predicts need for mechanical ventilation) 1
- Up to 22% of GBS patients require mechanical ventilation within the first week 1
Hospital Admission
- Admit to a unit with rapid ICU transfer capability - GBS can progress to respiratory failure within hours 2
- ICU admission is required for: evolving respiratory distress, severe autonomic cardiovascular dysfunction, severe swallowing dysfunction/diminished cough reflex, or rapid progression of weakness 1
Definitive Immunotherapy
First-Line Treatment: IVIg
IVIg is the preferred first-line treatment over plasma exchange due to ease of administration, wider availability, reduced adverse effects, and higher completion rates 3
Dosing regimen:
- 0.4 g/kg/day for 5 consecutive days (total 2 g/kg) 1, 2, 4
- The 5-day regimen is mandatory - shorter regimens result in more frequent treatment-related fluctuations 5, 2
- Initiate within 2 weeks of weakness onset for patients unable to walk unaided 4
Alternative: Plasma Exchange
- Equally effective to IVIg but technically more demanding 3
- Dosing: 5 sessions at 200-250 ml/kg over 1-2 weeks 3, 1, 4
- Consider if IVIg is contraindicated or unavailable 5
- Can be initiated within 4 weeks of weakness onset 3, 4
What NOT to Use
- Do NOT use corticosteroids alone - they provide no benefit and may worsen outcomes 1, 5, 2, 6
- Do NOT combine plasma exchange followed immediately by IVIg - no additional benefit 4
Essential Monitoring During Treatment
Neurological Assessment
- Monitor muscle strength in neck, arms, and legs using Medical Research Council grading scale 1
- Assess functional disability using GBS disability scale 1
- Monitor for swallowing and coughing difficulties to prevent aspiration 1
Autonomic Dysfunction Monitoring
- Continuous ECG monitoring for arrhythmias 1, 2
- Blood pressure monitoring for instability 1, 2
- Assess bowel/bladder function 1, 2
- Cardiovascular and respiratory dysfunction cause two-thirds of GBS deaths 1
Prognostic Assessment
- Calculate modified Erasmus GBS Outcome Scale (mEGOS) on admission to predict walking ability at 6 months 5, 4
- Calculate Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict ventilation need 1, 5, 4
Critical Medications to AVOID
Strictly avoid drugs that worsen neuromuscular function: 1, 5
- β-blockers
- IV magnesium
- Fluoroquinolones
- Aminoglycosides
- Macrolides
Diagnostic Workup (Concurrent with Treatment)
- Lumbar puncture for CSF analysis - typically shows elevated protein with normal cell count 2, 4
- MRI spine with and without contrast - to rule out compressive lesions mimicking GBS 2
- Electrodiagnostic studies (NCS/EMG) - to confirm polyneuropathy and classify subtype 2, 4
- Anti-GQ1b antibody testing if Miller Fisher syndrome suspected 4
Treatment-Related Fluctuations
- Occur in 6-10% of patients within 2 months of initial improvement 1, 5
- Repeat full course of IVIg or plasma exchange if fluctuations occur 5
- Consider A-CIDP (acute-onset CIDP) if progression continues beyond 8 weeks - occurs in ~5% of patients 4
Pain Management
- Use gabapentinoids, pregabalin, or duloxetine for neuropathic pain 5, 4
- Avoid opioids as first-line 5