What is the recommended treatment and management approach for a patient suspected of having Guillain-Barré Syndrome (GBS)?

Guillain-Barré Syndrome: Treatment and Management

First-Line Treatment

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days immediately upon diagnosis in patients with moderate to severe weakness, rapid progression, or any signs of respiratory compromise, dysphagia, facial weakness, or bulbar weakness. 1

• IVIg is preferred over plasma exchange as first-line therapy because it is easier to administer, more widely available, and has higher completion rates 1
• Plasma exchange (12-15 L over 4-5 exchanges in 1-2 weeks) is equally effective and can be used as an alternative, particularly within 4 weeks of symptom onset in patients unable to walk unaided 2
• Treatment should be initiated as early as possible in the disease course to maximize effectiveness 1
• Do not use corticosteroids alone—they provide no benefit and oral corticosteroids may worsen outcomes 1, 2

Critical Initial Assessment and Monitoring

Respiratory Monitoring

• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to calculate probability of requiring mechanical ventilation 1
• Apply the "20/30/40 rule": patient is at high risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1
• Monitor vital capacity, maximum inspiratory/expiratory pressures, and use of accessory respiratory muscles regularly 1
• Admit patients to a unit with rapid transfer capability to ICU, as approximately 25% require artificial ventilation 3, 4

Autonomic and Cardiac Monitoring

• Monitor continuously for arrhythmias, blood pressure fluctuations, and cardiac complications throughout the acute phase 5, 4
• Autonomic dysfunction is a major cause of mortality (3-10% overall mortality rate) 1, 3

Neurological Assessment

• Perform frequent neurological assessments to track motor function progression, bulbar symptoms, and cranial nerve involvement 5
• Use the modified Erasmus GBS outcome score (mEGOS) to predict individual probability of regaining walking ability 5, 2

Medications to Avoid

Immediately discontinue or avoid the following medications that worsen neuromuscular function: 1

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

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 treatment-related fluctuations occur more frequently with shorter regimens 1
• IVIg is preferred over plasma exchange due to better tolerability and fewer complications 1

Pregnant Women

• IVIg is preferred over plasma exchange because it requires fewer monitoring considerations, though neither treatment is contraindicated during pregnancy 1

Miller-Fisher Syndrome

• Treatment is generally not recommended as most patients recover completely within 6 months without intervention, though close monitoring remains essential 1

Obese Patients

• Dose IVIg based on ideal body weight, not actual body weight, as immunoglobulin distributes in plasma and extracellular fluid spaces that correlate with lean body mass 1
• Using actual body weight in obese patients results in supraphysiologic dosing without additional therapeutic benefit 1

Pre-Treatment Safety Checks

• Verify serum IgA levels before first infusion—IgA deficiency increases anaphylaxis risk; use IVIg preparations with reduced IgA levels if deficiency is confirmed 1
• Assess for active infection clinically (fever, leukocytosis, positive cultures), but do not delay IVIg or plasma exchange while ruling out infection, as preceding infections typically resolve before GBS onset 1
• If active infection is documented, start appropriate antimicrobials concurrently with immunotherapy 1

Expected Response and Management of Insufficient Improvement

Timeline for Response

• Approximately 40% of patients do not show improvement within the first 4 weeks following standard IVIg treatment—this does not necessarily indicate treatment failure 1, 5
• Most recovery occurs within the first year, with 80% of patients regaining independent walking ability by 6 months 1, 5

Treatment-Related Fluctuations (TRFs)

• TRFs occur in 6-10% of patients, defined as disease progression within 2 months after initial treatment-induced improvement 1, 5
• Repeat the full course of IVIg or plasma exchange for TRFs 1, 5
• Consider switching from IVIg to plasma exchange (or vice versa) if no improvement is seen by 4 weeks 5
• Do not give a second IVIg course to patients with poor prognosis who have not had TRFs—evidence does not support this approach 2

Suspecting Chronic CIDP

• Suspect chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) if a patient experiences three or more TRFs and/or clinical deterioration ≥8 weeks after onset 5
• Approximately 5% of patients initially diagnosed with GBS actually have acute-onset CIDP (A-CIDP) 3, 2
• Maintenance IVIg may be required if diagnosis changes to CIDP 5

Essential Supportive Care

Pain Management

• Use gabapentin, pregabalin, or duloxetine for neuropathic pain—avoid opioids 1, 5, 2
• Pain is frequent and significantly impacts wellbeing, often preceding weakness and confusing initial diagnosis 5, 3

Preventing Complications

• Provide prophylaxis for deep vein thrombosis using standard measures 1, 5
• Implement pressure ulcer prevention protocols 1, 5
• Assess swallowing safety and provide nutritional support for dysphagia and bulbar weakness 1, 5
• Address constipation/ileus, which is common in GBS patients 1

Psychological Support

• Screen for and address anxiety, depression, and hallucinations, which are common but often underrecognized 5
• Many patients experience fatigue and residual complaints that persist for months or years 3

Diagnostic Considerations

When to Perform Testing

• CSF examination is valuable, particularly when diagnosis is uncertain 2
• Electrodiagnostic testing should be performed to support the diagnosis 2
• Anti-GQ1b antibody testing should be considered when Miller Fisher syndrome is suspected 2
• Nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected 2
• MRI or ultrasound imaging should be considered in atypical cases 2

Clinical Clues

• GBS is more likely if there is a history of recent diarrhea or respiratory infection (Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae are common antecedent pathogens) 2, 6
• Ascending paralysis progressing over days to 4 weeks is the typical presentation 3, 6

Prognosis

• Approximately 80% regain walking ability at 6 months 1
• Approximately 20% remain unable to walk after 6 months 3
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 5
• Risk factors for poor outcome include advanced age and severe disease at onset 1, 5