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

Treatment of Guillain-Barré Syndrome

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg body weight daily for 5 consecutive days as first-line treatment for any patient with GBS who cannot walk unaided, starting as early as possible within 2 weeks of symptom onset. 1, 2, 3

First-Line Immunotherapy

IVIg is the preferred first-line treatment over plasma exchange because it is easier to administer, more widely available, has higher completion rates, and has better tolerability with fewer complications—particularly important in children and pregnant women. 1, 2, 3

Alternative First-Line Option

• Plasma exchange (PE) is equally effective and should be used when IVIg is contraindicated, not tolerated, or unavailable. 1, 2, 3
• PE dosing: 200-250 ml plasma/kg body weight administered in 4-5 exchanges over 1-2 weeks (total 12-15 L). 3
• PE can be initiated up to 4 weeks after symptom onset in patients unable to walk unaided. 3

What NOT to Do

• Do not use corticosteroids alone—randomized controlled trials show no benefit and oral corticosteroids may worsen outcomes. 1, 2, 3
• Do not combine PE followed immediately by IVIg—this combination provides no additional benefit. 3
• Do not routinely give a second course of IVIg to patients with poor prognosis who fail to improve, as current evidence does not support this approach. 4, 3

Critical Respiratory Monitoring

Apply the "20/30/40 rule" to identify patients at imminent risk of respiratory failure:

• Vital capacity <20 ml/kg 4, 1, 2
• Maximum inspiratory pressure <30 cmH₂O 4, 1, 2
• Maximum expiratory pressure <40 cmH₂O 4, 1, 2

Additional respiratory assessments:

• Single breath count ≤19 predicts need for mechanical ventilation 4, 5
• Monitor for use of accessory respiratory muscles 4, 1
• Up to 30% of patients require mechanical ventilation, so maintain high vigilance. 6, 7

ICU Admission Criteria

Admit to ICU if any of the following are present:

• Evolving respiratory distress with imminent respiratory insufficiency 5
• Severe autonomic cardiovascular dysfunction 5
• Severe swallowing dysfunction or diminished cough reflex 5
• Rapid progression of weakness 5

Managing Treatment Failures and Fluctuations

Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement and represent disease reactivation while the inflammatory phase continues. 4, 1

• For TRFs: Repeat the full course of IVIg or switch to PE, though evidence supporting this is limited. 4, 1, 2
• For insufficient response: About 40% of patients don't improve in the first 4 weeks—this doesn't mean treatment failed, as progression might have been worse without therapy. 4, 1
• Consider acute-onset CIDP if patients have ≥3 TRFs or clinical deterioration ≥8 weeks after onset (occurs in ~5% of cases). 4, 2, 3

Multidisciplinary Supportive Care

Autonomic Dysfunction Monitoring

• Continuous ECG monitoring for arrhythmias 4, 5
• Blood pressure monitoring for hypertension/hypotension 4, 5
• Monitor bowel and bladder function 4, 5
• Critical pitfall: Up to two-thirds of GBS deaths occur during the recovery phase from cardiovascular and respiratory complications—maintain vigilance even after ICU discharge. 4, 1

Neurological Monitoring

• Assess muscle strength using Medical Research Council grading scale 4, 5
• Document functional disability using GBS disability scale 4, 5
• Monitor swallowing and coughing to prevent aspiration 4, 1, 5

Pain and Psychological Management

• Pain is common—recognize and treat early as it significantly impacts quality of life. 4
• Use gabapentinoids, tricyclic antidepressants, or carbamazepine for neuropathic pain. 3
• Screen for anxiety, depression, and hallucinations, especially in ICU patients with limited communication. 4, 1
• Remember: Patients with complete paralysis usually have intact consciousness, vision, and hearing—be mindful of bedside conversations. 4

Standard Hospital Complications Prevention

• Prevent pressure ulcers, hospital-acquired infections (pneumonia, UTI), and deep vein thrombosis using standard protocols. 4, 1
• Monitor for corneal ulceration in facial palsy 4
• Prevent limb contractures and pressure palsies 4

Medication Precautions

Avoid medications that worsen neuromuscular function:

• β-blockers 2, 5
• IV magnesium 2, 5
• Fluoroquinolones 2, 5
• Aminoglycosides 2, 5
• Macrolides 2, 5

Rehabilitation

Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 4, 1

• Include range-of-motion exercises, stationary cycling, walking, and strength training. 1
• Monitor exercise intensity closely—overwork causes fatigue. 1
• Recovery may continue >5 years after onset for residual pain, weakness, and fatigue. 4, 1

Prognosis

• 80% of patients regain independent walking ability at 6 months. 4, 2
• Use the modified Erasmus GBS Outcome Score (mEGOS) to predict individual walking recovery probability. 4, 3
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess ventilation risk. 2, 3
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications. 4, 2
• Risk factors for mortality: advanced age and severe disease at onset. 4, 2