What is the treatment for Guillain-Barré Syndrome (GBS) symptoms?

Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days is the first-line treatment for any patient with GBS who cannot walk unaided, and should be initiated as early as possible within 2 weeks of symptom onset. 1, 2, 3

Immediate Assessment and Monitoring

Respiratory Function Assessment (Critical Priority)

• Apply the "20/30/40 rule" immediately: patient is at imminent risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 4, 2, 5
• Perform single breath count: ≤19 predicts need for mechanical ventilation 2, 5
• Monitor for use of accessory respiratory muscles 5
• Up to 30% of patients develop respiratory failure requiring mechanical ventilation, often without obvious dyspnea 6, 5

ICU Admission Criteria

Admit to ICU if any of the following are present: 4, 2, 5

• Evolving respiratory distress with imminent respiratory insufficiency
• Severe autonomic cardiovascular dysfunction
• Severe swallowing dysfunction or diminished cough reflex
• Rapid progression of weakness

First-Line Immunotherapy

IVIg (Preferred First-Line)

• Dose: 0.4 g/kg body weight daily for 5 consecutive days (total 2 g/kg) 1, 4, 2, 3
• Start as early as possible within 2 weeks of symptom onset 1, 2, 3
• IVIg is preferred over plasma exchange because it is easier to administer, more widely available, has higher completion rates, and better tolerability with fewer complications—particularly important in children and pregnant women 2

Plasma Exchange (Alternative First-Line)

• Dose: 200-250 ml plasma/kg body weight in 5 sessions over 1-2 weeks 1, 5, 3
• Equally effective as IVIg but more difficult to administer 1, 2
• May be preferred option for life-threatening symptoms 1
• Consider contraindications: renal failure, hypercoagulable states, sepsis, hemodynamic instability 1

What NOT to Use

• Corticosteroids alone are NOT recommended for idiopathic GBS as they have shown no significant benefit and may have negative effects 1, 2, 5, 3
• Do not use PE followed immediately by IVIg—no added benefit 3

Special Context: Immune Checkpoint Inhibitor-Related GBS

Unlike idiopathic GBS, corticosteroids ARE recommended for ICI-related GBS: 1

• Methylprednisolone 2-4 mg/kg/day followed by slow taper 1
• For grade 3-4 events: pulse methylprednisolone 1 g daily for 5 days along with IVIg or plasmapheresis 1
• Permanently discontinue ICI for severe cases 1

Management of Treatment Failures and Fluctuations

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months of initial improvement 1, 4, 2
• Represent disease reactivation while inflammatory phase continues 1, 2
• Repeat the full course of IVIg or plasma exchange, though evidence supporting this is limited 1, 4, 2

Poor Initial Response

• About 40% of patients do not improve in first 4 weeks following treatment—this does NOT necessarily indicate treatment ineffectiveness 1, 4
• Do NOT administer a second IVIg course in GBS patients with poor prognosis as routine practice (no proven benefit) 3

Ongoing Monitoring and Supportive Care

Neurological Monitoring

• Assess muscle strength using Medical Research Council grading scale 4, 5
• Document functional disability using GBS disability scale 4, 5
• Frequent neurological assessments 1, 4

Autonomic Dysfunction Monitoring

• Continuous ECG monitoring for arrhythmias 4, 2
• Blood pressure monitoring for hypertension/hypotension 4, 2
• Monitor bowel and bladder function 1, 4

Swallowing and Airway Protection

• Monitor for swallowing and coughing difficulties to prevent aspiration 5
• Assess for bulbar palsy 4

Pain Management

• Pain is common and significantly impacts quality of life 2
• Use nonopioid management: pregabalin, gabapentin, or duloxetine 1, 4
• Weak recommendation for gabapentinoids, tricyclic antidepressants, or carbamazepine 3

Complication Prevention

Medications to AVOID

Do not use medications that worsen neuromuscular function: 4, 5

• β-blockers
• IV magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolides

Other Preventive Measures

• Implement preventive measures for pressure ulcers 4
• Prevent hospital-acquired infections 4
• DVT prophylaxis 4
• Address corneal ulceration in patients with facial palsy 4
• Prevent limb contractures 4

Rehabilitation

• Initiate early rehabilitation with multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians 2
• Include range-of-motion exercises, stationary cycling, walking, and strength training 1, 2
• Monitor exercise intensity closely—overwork causes fatigue 1, 2

Prognosis

• 80% of patients regain independent walking ability at 6 months 1, 4, 2
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 4, 2
• Risk factors for mortality: advanced age and severe disease at onset 1, 2
• Up to two-thirds of deaths occur during the recovery phase—continued vigilance is necessary even after apparent improvement 4
• Recovery can continue for >5 years after disease onset 1

Critical Pitfalls to Avoid

• Respiratory failure can develop rapidly without obvious dyspnea—do not wait for subjective complaints 5
• Only 25-30% of patients are diagnosed on initial healthcare visit—maintain high index of suspicion 7
• Changing diagnosis to acute-onset CIDP should be considered if progression continues after 8 weeks from onset (occurs in ~5% of patients) 1, 3