What is the approach to managing a patient with Guillain-Barré Syndrome (GBS)?

Approach to Managing Guillain-Barré Syndrome

Immediate Assessment and Risk Stratification

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

Initial Diagnostic Evaluation

• Confirm the diagnosis by assessing for recent diarrhea or respiratory infection (particularly Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus, or Mycoplasma pneumoniae), as these are commonly identified antecedent pathogens 2
• Perform CSF examination to support diagnosis, particularly when clinical presentation is atypical 3
• Obtain electrodiagnostic testing to confirm the diagnosis and differentiate between acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) subtypes 3
• Consider anti-GQ1b antibody testing when Miller Fisher syndrome is suspected, though anti-ganglioside antibody testing has limited clinical value in typical motor-sensory GBS 3
• Test for nodal-paranodal antibodies when autoimmune nodopathy is suspected 3

Critical Respiratory Assessment

Apply the "20/30/40 rule" immediately to identify imminent respiratory failure risk: vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, and maximum expiratory pressure <40 cmH₂O 1, 4

• Use single breath count where ≤19 predicts need for mechanical ventilation 1
• Calculate the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess probability of requiring artificial ventilation 5, 3
• Monitor pulmonary function continuously including negative inspiratory force and vital capacity, as respiratory compromise can occur even after initial treatment 5

Admission and Monitoring Strategy

ICU Admission Criteria

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

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

Continuous Monitoring Requirements

• Perform continuous ECG monitoring for arrhythmias and blood pressure monitoring for hypertension/hypotension 1, 5
• Monitor bowel and bladder function regularly 1
• Assess muscle strength using Medical Research Council grading scale and document functional disability using GBS disability scale 1
• Conduct frequent neurological assessments to track motor function progression, bulbar symptoms, and respiratory status 5

Medications to Avoid

Avoid β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides as they can worsen neuromuscular function and exacerbate the clinical condition 4

First-Line Immunotherapy

IVIg is preferred 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, 4

IVIg Administration Protocol

• Dose: 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg) 1, 4
• Timing: Initiate as early as possible within 2 weeks of symptom onset 1
• Pre-infusion screening: Verify serum IgA levels before first infusion, as IgA deficiency increases anaphylaxis risk; use preparations with reduced IgA levels if deficiency is confirmed 4
• Monitor rigorously during and after each infusion for neurological function (motor strength, reflexes, bulbar symptoms) and potential adverse reactions 4

Alternative: Plasma Exchange

Plasma exchange (12-15 L in four to five exchanges over 1-2 weeks) is equally effective and should be considered in patients within 4 weeks of onset who cannot walk unaided, or when IVIg is contraindicated 3

What NOT to Do

• Do not use corticosteroids alone as randomized controlled trials show no significant benefit and oral corticosteroids may have negative effects 4, 3
• Do not combine plasma exchange followed immediately by IVIg as this provides no additional benefit 3
• Do not administer a second IVIg course routinely in GBS patients with poor prognosis, as evidence does not support this approach 3

Managing Treatment Response and Complications

Expected Timeline

Approximately 40% of patients do not show improvement within the first 4 weeks following standard IVIg treatment, which does not necessarily indicate treatment ineffectiveness 4, 5

Treatment-Related Fluctuations (TRFs)

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

• For TRFs: Repeat the full course of IVIg or switch to plasma exchange 1, 5
• Suspect chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) if a patient experiences three or more TRFs and/or clinical deterioration ≥8 weeks after onset 5
• Consider maintenance IVIg if diagnosis changes to acute-onset CIDP (A-CIDP), which occurs in approximately 5% of patients initially diagnosed with GBS 3

Multidisciplinary Supportive Care

Pain Management

Pain is common and significantly impacts quality of life—recognize and treat early 1

• Use gabapentin, pregabalin, or duloxetine for neuropathic pain management 4, 5, 3
• Avoid opioids for pain control 4

Autonomic Dysfunction Management

• Monitor and manage autonomic cardiovascular dysfunction including arrhythmias and blood pressure fluctuations 5
• Address constipation/ileus which is common in GBS patients 4

Prevention of Complications

• Provide prophylaxis for deep vein thrombosis and pressure ulcers through standard prophylactic measures 4, 5
• Evaluate dysphagia and provide safe swallowing assessment and nutritional support as needed 4, 5
• Address psychological symptoms including anxiety, depression, and hallucinations, which are common but often underrecognized 5

Rehabilitation

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

• Include range-of-motion exercises, stationary cycling, walking, and strength training 1
• Monitor exercise intensity closely as overwork causes fatigue 1

Prognostic Assessment

Use the modified Erasmus GBS outcome score (mEGOS) to calculate individual probability of regaining walking ability 5, 3

Expected Outcomes

• 80% of patients regain independent walking ability at 6 months 1, 4, 5
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 4, 5
• Risk factors for mortality and poor outcome: advanced age and severe disease at onset 1, 5
• Approximately 20% of patients are still unable to walk after 6 months and many have persistent pain, fatigue, or other residual complaints 6

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 4

Pregnant Women

IVIg is preferred over plasma exchange because it requires fewer monitoring considerations and additional precautions, though neither treatment is contraindicated during pregnancy 4

Miller Fisher Syndrome

Treatment is generally not recommended as most patients recover completely within 6 months without intervention, though close monitoring is essential 4

Immune Checkpoint Inhibitor-Related GBS

Discontinue the causative agent permanently and consider concurrent corticosteroids with IVIg or plasma exchange 4