What is the appropriate emergency management and treatment for a patient with suspected Guillain‑Barré syndrome?

Emergency Management and Treatment of Guillain-Barré Syndrome

Immediate Assessment and Stabilization

Admit all suspected GBS patients to an inpatient unit with rapid ICU transfer capability, as up to 30% develop respiratory failure requiring mechanical ventilation, often without obvious dyspnea. 1, 2

Critical Respiratory 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 3, 1
• Perform single breath count test: ≤19 predicts need for mechanical ventilation 1
• Measure vital capacity, negative inspiratory force (NIF), and maximum inspiratory/expiratory pressures serially, not just at presentation 4
• Monitor for use of accessory respiratory muscles, breathlessness during talking, or inability to count to 15 in one breath 3

ICU Admission Criteria

Admit to ICU immediately if any of the following are present: 3, 1

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

Cardiovascular and Autonomic Monitoring

• Initiate continuous ECG monitoring for arrhythmias 3, 4
• Monitor blood pressure continuously for hypertension/hypotension 3, 4
• Assess bowel and bladder function for autonomic dysfunction 3

Diagnostic Workup (Do Not Delay Treatment)

Do not wait for confirmatory testing to initiate treatment if GBS is clinically suspected. 4

Essential Testing

• CSF analysis: Look for albumino-cytological dissociation (elevated protein with normal cell count), but do not dismiss GBS based on normal CSF protein in the first week—this may be absent early 4, 5
• Nerve conduction studies and EMG: Support diagnosis and classify subtype (AIDP, AMAN, AMSAN), though may be normal within first week 4, 5
• MRI spine with contrast: Rule out compressive lesions and evaluate for nerve root enhancement 3, 4
• Screen for reversible causes: HbA1c, vitamin B12, TSH, vitamin B6, folate 4

First-Line Immunotherapy

Initiate IVIg 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) in any patient unable to walk unaided within 2 weeks of symptom onset. 1, 6, 5

Why IVIg Over Plasma Exchange

IVIg is preferred as first-line therapy because: 3, 1, 6

• Easier to administer and more widely available
• Higher completion rates with fewer discontinuations
• Particularly important in children and pregnant women due to better tolerability
• Equally effective as plasma exchange for mortality and morbidity outcomes

Alternative: Plasma Exchange

• Plasma exchange 200-250 ml/kg over 4-5 sessions is equally effective but reserved for settings where IVIg is unavailable or contraindicated 3, 6, 5
• Can be initiated within 4 weeks of symptom onset 5

What NOT to Use

• Do not use corticosteroids alone: Eight randomized controlled trials showed no benefit, and oral corticosteroids may worsen outcomes 3, 1, 6
• Do not use plasma exchange followed immediately by IVIg: No more effective than either treatment alone 3, 5

Medications to Avoid

Avoid medications that worsen neuromuscular transmission: β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides 1, 4

Neurological Monitoring During Treatment

• Grade muscle strength using Medical Research Council scale in neck, arms, and legs 3, 4
• Assess functional disability using GBS disability scale 3
• Test swallowing and coughing ability to identify aspiration risk 3, 4
• Check corneal reflex in patients with facial palsy to prevent corneal ulceration 4

Pain Management

Initiate gabapentinoids (gabapentin or pregabalin), tricyclic antidepressants, or carbamazepine immediately for neuropathic pain—do not delay waiting for IVIg to work first. 1, 5

• Severe pain occurs in at least one-third of patients and can be muscular, radicular, or neuropathic 1, 4
• Pain management is a distinct treatment goal separate from immunotherapy 4
• Avoid opioids; prioritize gabapentinoids 4

Managing Treatment Response

Expected Timeline

• 40% of patients do not improve in the first 4 weeks following treatment—this does not mean treatment failed 1, 6
• Recovery can continue for more than 3 years, with improvement possible even beyond 5 years 1, 6

Treatment-Related Fluctuations (TRFs)

• Occur in 6-10% of patients within 2 months after initial improvement 1, 6
• Represent disease reactivation while inflammatory phase continues 1
• For TRFs: Repeat full course of IVIg or switch to plasma exchange 1

When to Consider Acute-Onset CIDP

Consider changing diagnosis to acute-onset CIDP if: 4, 5

• Progression continues after 8 weeks from onset
• Patient has three or more TRFs
• This occurs in approximately 5% of patients initially diagnosed with GBS

Supportive Care and Complication Prevention

• Standard prophylaxis for deep vein thrombosis, pressure ulcers, and hospital-acquired infections 3, 4
• Assess swallowing function; provide nutritional support if dysphagia present 3
• Address constipation/ileus proactively 4
• Recognize that patients with complete paralysis usually have intact consciousness, vision, and hearing—explain all procedures to reduce anxiety 3, 4
• Screen for anxiety, depression, and hallucinations, which are frequent complications 3

Special Populations

Children

• Use the same 5-day IVIg regimen (0.4 g/kg/day for 5 days) rather than accelerated 2-day protocols, as TRFs occur more frequently with shorter regimens 3, 1
• IVIg strongly preferred over plasma exchange due to better tolerability 3

Pregnant Women

• IVIg is preferred over plasma exchange due to fewer monitoring requirements 1, 6
• Neither treatment is contraindicated during pregnancy 6

Immune Checkpoint Inhibitor-Related GBS

• Discontinue causative agent permanently 1
• Consider concurrent corticosteroids with IVIg or plasma exchange 1

Prognosis

• 80% of patients regain independent walking ability at 6 months 1, 6, 5
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications in both acute and recovery phases 1, 6
• Risk factors for poor outcome: Advanced age and severe disease at onset 1, 6
• Use modified Erasmus GBS outcome score (mEGOS) to predict probability of regaining walking ability 1, 5

Common Pitfalls

• Do not wait for CSF protein elevation in first week to diagnose GBS—it may be normal early 4, 5
• Do not delay treatment waiting for electrodiagnostic confirmation—studies may be normal in first week 4
• Do not assume improvement means safety—two-thirds of deaths occur during recovery phase from cardiovascular and respiratory complications 3
• Do not dismiss bilateral facial palsy as Bell's palsy—this is extremely rare in Bell's and should immediately raise suspicion for GBS 4