What is the recommended first‑line treatment and monitoring for Guillain‑Barré syndrome?

Guillain-Barré Syndrome: First-Line Treatment and Monitoring

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) is the recommended first-line treatment for any patient with Guillain-Barré syndrome who cannot walk unaided within 2–4 weeks of symptom onset, combined with immediate admission to a unit capable of rapid ICU transfer and continuous respiratory and autonomic monitoring. 1, 2

Immediate Assessment Priorities

Respiratory function assessment is the highest priority because approximately 20% of patients develop respiratory failure requiring mechanical ventilation, which can occur rapidly and sometimes without obvious dyspnea. 3, 4

Apply the "20/30/40 Rule" for Respiratory Risk:

• Vital capacity <20 ml/kg
• Maximum inspiratory pressure <30 cmH₂O
• Maximum expiratory pressure <40 cmH₂O

Any of these thresholds indicates imminent respiratory failure requiring intubation. 1, 5

Additional Respiratory Indicators:

• Single breath count ≤19 predicts need for mechanical ventilation 1, 5
• Use of accessory respiratory muscles 5
• Perform serial vital capacity and negative inspiratory force measurements at least daily 1, 3

Admission and Monitoring Requirements

All patients with suspected GBS require hospital admission with capability for rapid ICU transfer, regardless of initial severity, because progression can be unpredictable and rapid. 1, 3, 6

Continuous Cardiovascular Monitoring:

• Electrocardiographic monitoring for arrhythmias 5
• Blood pressure monitoring for hypertension or hypotension 5
• Heart rate variability assessment 5
• Pupillary dysfunction evaluation 5
• Bowel and bladder function monitoring 5

Autonomic dysfunction contributes to the 3–10% mortality rate even with optimal care. 3, 5

First-Line Immunotherapy Protocol

IVIg is preferred over plasma exchange because it is easier to administer, more widely available, and has higher completion rates. 1, 2

IVIg Dosing:

• 0.4 g/kg/day for 5 consecutive days (total 2 g/kg) 1, 2
• Use ideal body weight for dosing in obese patients 1
• Check serum IgA levels before first infusion; IgA deficiency increases anaphylaxis risk 1
• Initiate as early as possible, ideally within 2 weeks of symptom onset 1, 5

Alternative: Plasma Exchange

• 200–250 ml/kg total over 4–5 sessions within 4 weeks of onset 1, 2
• Equally effective as IVIg but requires more complex administration 1

What NOT to Do:

• Do not use corticosteroids alone—they show no benefit and may worsen outcomes 1, 2
• Do not use sequential therapy (plasma exchange followed by IVIg or vice versa)—no additional benefit 2
• Do not delay treatment waiting for antibody test results 3

Neurological Monitoring

Daily Assessments Should Include:

• Muscle strength grading (Medical Research Council scale) in neck, arms, and legs 1, 5
• Functional disability using GBS disability scale 1, 5
• Swallowing and coughing ability to assess aspiration risk 1, 5
• Cranial nerve examination, particularly for facial weakness and ophthalmoplegia 3
• Deep tendon reflexes 3

Supportive Care Essentials

Pain Management:

Gabapentinoids (gabapentin or pregabalin) or duloxetine are first-line for neuropathic pain; these can be started concurrently with IVIg without drug interaction. 1, 3, 2

Medications to AVOID:

These worsen neuromuscular function and must be discontinued:

• β-blockers
• IV magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolides 1, 5

Standard Preventive Measures:

• Deep vein thrombosis prophylaxis 3
• Pressure ulcer prevention 3, 5
• Constipation/ileus management 7, 3
• Hospital-acquired infection prevention 5

Expected Treatment Response

Approximately 40% of patients do not improve in the first 4 weeks after treatment—this does NOT mean treatment failed, as progression might have been worse without therapy. 1, 3

Treatment-Related Fluctuations (TRFs):

• Occur in 6–10% of patients within 2 months after initial improvement 1, 3
• Defined as disease progression after initial stabilization 1
• Repeating full course of IVIg or plasma exchange is common practice, though evidence is limited 1, 3

When to Reconsider Diagnosis:

Consider acute-onset CIDP if:

• Progression continues beyond 8 weeks from onset
• Patient experiences ≥3 treatment-related fluctuations
• This occurs in approximately 5% of initial GBS diagnoses 3, 2

Diagnostic Workup (Do Not Delay Treatment)

While awaiting results, initiate treatment based on clinical suspicion alone. 3

Essential Tests:

• Lumbar puncture: Look for albumino-cytological dissociation (elevated protein with normal cell count); normal protein in first week does NOT exclude GBS 3, 2
• Nerve conduction studies and EMG: Support diagnosis and classify subtype; repeat in 2–3 weeks if initially normal 3, 2
• MRI spine with contrast: Exclude compressive lesions, assess nerve root enhancement 3
• Serum antiganglioside antibodies: Test for anti-GQ1b if Miller Fisher syndrome suspected 7, 3, 2

Prognosis

• 80% regain independent walking by 6 months 1, 3, 5
• Recovery can continue for >3 years, with improvements possible beyond 5 years 1, 3
• Mortality 3–10%, primarily from cardiovascular and respiratory complications 3, 5
• Risk factors for poor outcome: advanced age and severe disease at onset 1, 3, 5
• Fatigue affects 60–80% of survivors and is a major disabling symptom 3
• Severe pain persists in at least one-third of patients at 1 year 3

Early Rehabilitation

Initiate structured rehabilitation immediately with physiotherapists, occupational therapists, and rehabilitation specialists. 3, 5

Exercise Programs Should Include:

• Range-of-motion exercises
• Stationary cycling
• Walking
• Strength training

Monitor intensity to avoid excessive fatigue. 3, 5

Special Considerations: Immune Checkpoint Inhibitor-Related GBS

If GBS develops during immune checkpoint inhibitor therapy:

• Permanently discontinue the checkpoint inhibitor 7, 3
• Add concurrent corticosteroids (methylprednisolone 2–4 mg/kg/day) to IVIg or plasma exchange 7, 1
• For Grade 3–4 severity, consider pulse therapy (methylprednisolone 1 g/day for 5 days) 7, 3

This is the only scenario where corticosteroids are recommended in GBS. 7