What is the appropriate treatment and management for a patient diagnosed with Guillain-Barré Syndrome (GBS)?

Guillain-Barré Syndrome: Treatment and Management

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

Immediate Assessment Priorities

Upon suspecting GBS, your first actions must address life-threatening complications:

Respiratory Function Assessment

• 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 test: ≤19 predicts need for mechanical ventilation 4, 2, 5
• Monitor for clinical signs: breathlessness at rest or during talking, inability to count to 15 in one breath, use of accessory respiratory muscles, increased respiratory or heart rate 1
• Up to 22% of patients require mechanical ventilation within the first week, so serial monitoring is essential 1

Calculate Risk Using EGRIS Score

Use the Erasmus GBS Respiratory Insufficiency Score to predict probability of requiring ventilation within 1 week 1:

• Days from weakness onset to admission: ≤3 days (2 points), 4-7 days (1 point), >7 days (0 points) 1
• Facial/bulbar weakness present: 1 point 1
• MRC sum score: ≤20 (4 points), 21-30 (3 points), 31-40 (2 points), 41-50 (1 point), 51-60 (0 points) 1

Autonomic Dysfunction Monitoring

• Initiate continuous ECG monitoring for arrhythmias 1, 4, 5
• Monitor blood pressure continuously for marked variations 1, 5
• Assess for pupillary dysfunction, bowel and bladder dysfunction 1, 5

Bulbar Function Assessment

• Test swallowing ability and cough reflex to identify aspiration risk 1, 4, 5
• Check corneal reflex in patients with facial palsy to prevent corneal ulceration 4

ICU Admission Criteria

Admit to ICU immediately if any of the following are present 1, 2, 5:

• Evolving respiratory distress with imminent respiratory insufficiency 1
• Severe autonomic cardiovascular dysfunction (arrhythmias, marked blood pressure variation) 1, 5
• Severe swallowing dysfunction or diminished cough reflex 1
• Rapid progression of weakness 1

First-Line Treatment

IVIg vs Plasma Exchange Decision

IVIg is the preferred first-line treatment for the following reasons 1, 2, 3:

• Easier to administer and more widely available 1
• Higher completion rates (plasma exchange more likely to be discontinued) 1
• Better tolerability with fewer complications 2
• Particularly important in children and pregnant women 1, 2

IVIg dosing: 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg) 1, 2, 3

Plasma exchange alternative: 200-250 ml plasma/kg body weight in 4-5 sessions over 1-2 weeks 1, 3

Treatment Timing

• Start treatment as early as possible within 2 weeks of symptom onset 2, 3
• Treatment can be considered up to 4 weeks after onset if patient cannot walk unaided 1, 3
• Do not wait for antibody test results or CSF confirmation before starting treatment 4

What NOT to Use

Avoid corticosteroids: Eight randomized controlled trials showed no significant benefit, and oral corticosteroids had negative effects on outcome 1, 3

Do not combine plasma exchange followed by IVIg: No more effective than either treatment alone 1

Avoid medications that worsen neuromuscular function: β-blockers, intravenous magnesium, fluoroquinolones, aminoglycosides, macrolides 5

Managing Treatment Response

Insufficient Response (40% of patients)

• Approximately 40% of patients do not improve in the first 4 weeks following treatment 1, 2
• This does not mean treatment failed—progression might have been worse without therapy 1
• Currently no evidence supports repeating treatment or switching to alternative treatment in this scenario 1
• A clinical trial investigating second IVIg dose is ongoing 1

Treatment-Related Fluctuations (TRFs)

TRFs occur in 6-10% of patients and are defined as disease progression within 2 months following initial treatment-induced improvement or stabilization 1, 2, 3:

• Repeat the full course of IVIg or plasma exchange when TRFs occur, as this indicates the inflammatory phase is still ongoing 1, 2
• This is common practice despite lacking strong evidence 1, 2

Distinguishing Acute-Onset CIDP

• If progression continues beyond 8 weeks from onset or patient has ≥3 TRFs, consider diagnosis of acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) 1, 3
• This occurs in approximately 5% of patients initially diagnosed with GBS 1, 3

Special Populations

Miller Fisher Syndrome (MFS)

• Pure MFS patients tend to have mild disease course with complete recovery within 6 months 1
• Treatment generally not recommended but monitor closely as subgroup can develop limb weakness, bulbar/facial palsy, or respiratory failure 1

Pregnant Women

• Neither IVIg nor plasma exchange is contraindicated during pregnancy 1
• IVIg is preferred as plasma exchange requires additional considerations and monitoring 1

Children

• Same treatment principles apply 1
• IVIg particularly preferred due to ease of administration 2

Multidisciplinary Supportive Care

Complication Prevention

• Standard prophylaxis for all bed-bound patients: Deep vein thrombosis prophylaxis, pressure ulcer prevention, hospital-acquired infection prevention 1, 2, 5
• GBS-specific complications to monitor: Inability to swallow safely in bulbar palsy, corneal ulceration in facial palsy, limb contractures, ossification, pressure palsies 1

Pain Management

• Pain affects approximately two-thirds of patients and can be muscular, radicular, or neuropathic 4
• Weakly recommend gabapentinoids, tricyclic antidepressants, or carbamazepine for pain treatment 3
• Recognize and treat pain early as it significantly impacts quality of life 1, 2, 5

Psychological Support

• Patients with GBS, even those with complete paralysis, usually have intact consciousness, vision, and hearing 1, 4
• Screen for anxiety, depression, and hallucinations—these are frequent complications 1, 4
• Be mindful of bedside conversations and explain all procedures to reduce anxiety 1, 4

Rehabilitation

• Initiate early rehabilitation with multidisciplinary team: Physiotherapists, occupational therapists, speech therapists, dietitians 1, 2, 5
• Include range-of-motion exercises, stationary cycling, walking, strength training 2, 5
• Monitor exercise intensity closely as overwork causes fatigue 2

Monitoring Disease Progression

Neurological Assessment

• Grade muscle strength using Medical Research Council scale in neck, arms, and legs 4, 5
• Document functional disability using GBS disability scale 4, 2, 5
• Perform serial assessments to track progression and recovery 4

Mechanical Ventilation Considerations

• Risk factors for prolonged mechanical ventilation: inability to lift arms from bed at 1 week after intubation, axonal subtype or unexcitable nerves on electrophysiology 1
• Consider early tracheostomy in patients with these risk factors 1

Prognosis

Recovery Timeline

• Most patients reach maximum disability within 2 weeks 1, 4
• 80% of patients regain independent walking ability at 6 months 1, 2, 5, 3
• Clinical improvement is usually most extensive in the first year but can continue for >5 years 1, 2, 5

Mortality and Risk Factors

• Mortality is 3-10%, primarily from cardiovascular and respiratory complications occurring in both acute and recovery phases 1, 2, 5
• Risk factors for mortality: Advanced age and severe disease at onset 1, 2, 5

Long-Term Outcomes

• Despite generally positive prospects, long-term residual complaints are common: neuropathic pain, weakness, fatigue 1
• Recovery from these complaints may still occur >5 years after disease onset 1

Recurrence

• Recurrent GBS is rare, affecting 2-5% of patients 1, 2
• Prior GBS is not a strict contraindication for vaccination 1, 2
• Discuss with experts for patients diagnosed with GBS <1 year before planned vaccination or who previously developed GBS shortly after receiving the same vaccination 1

Common Pitfalls to Avoid

• Do not dismiss GBS based on normal CSF protein in the first week—protein elevation may not yet be present 4
• Do not delay treatment waiting for diagnostic confirmation—start IVIg if clinical suspicion is high 4
• Do not use corticosteroids alone—they are ineffective and potentially harmful 1, 3
• Do not assume treatment failure if patient continues to worsen in first 4 weeks—this is expected in 40% of patients 1, 2
• Marked persistent asymmetry, bladder dysfunction at onset, or marked CSF pleocytosis should prompt reconsideration of diagnosis 4