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 (total dose 2 g/kg) immediately in patients unable to walk unaided or showing rapid progression, respiratory compromise, dysphagia, facial weakness, or bulbar weakness. 1, 2

• IVIg is preferred over plasma exchange as first-line therapy because it is easier to administer, more widely available, has higher completion rates, and requires fewer monitoring considerations 1
• Plasma exchange (12-15 L over 4-5 sessions in 1-2 weeks) is equally effective and should be considered as an alternative if IVIg is unavailable or contraindicated 2
• Do not use corticosteroids alone—randomized controlled trials show no benefit and oral corticosteroids may worsen outcomes 1, 2
• Treatment should be initiated as early as possible, ideally within 2 weeks of symptom onset for IVIg and within 4 weeks for plasma exchange 2

Critical Respiratory Monitoring

Admit all patients to a unit with rapid ICU transfer capability and monitor respiratory function using the "20/30/40 rule"—patient is at risk of respiratory failure if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O. 3, 1

• Assess single breath count (≤19 predicts need for mechanical ventilation), vital capacity, and maximum inspiratory/expiratory pressures at regular intervals 3
• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to calculate probability of requiring ventilation 1
• Up to 30% of patients develop respiratory failure requiring mechanical ventilation 4
• Monitor for swallowing and coughing difficulties that may compromise airway protection 3

Autonomic and Cardiovascular Monitoring

Monitor continuously for autonomic dysfunction via electrocardiography, heart rate, blood pressure, and bowel/bladder function—two-thirds of deaths occur during the recovery phase from cardiovascular and respiratory complications. 3, 5

• Stay vigilant even after apparent improvement, especially in patients recently transferred from ICU and those with cardiovascular risk factors 3
• Watch for arrhythmias, blood pressure fluctuations, and respiratory distress from mucus plugs 3

Medications to Avoid

Avoid β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides—these medications worsen neuromuscular function and can exacerbate clinical deterioration. 1, 5

Diagnostic Workup

• Perform lumbar puncture for CSF analysis—classic finding is elevated protein with normal cell count (albumino-cytological dissociation), though protein is normal in 30-50% of patients in the first week 3
• Marked pleocytosis (>50 cells/μl) suggests alternative diagnoses such as leptomeningeal malignancy or infectious polyradiculitis 3
• Electrodiagnostic studies support the diagnosis and help distinguish AIDP from AMAN variants, but are not required to initiate treatment 3
• Anti-GQ1b antibody testing should be performed when Miller Fisher syndrome is suspected (found in up to 90% of MFS cases) 3
• Do not delay treatment while waiting for antibody test results 3

Multidisciplinary Supportive Care

Implement a multidisciplinary approach involving nurses, physiotherapists, rehabilitation specialists, occupational therapists, speech therapists, and dietitians to prevent and manage complications. 3

• Pain management: Use gabapentin, pregabalin, or duloxetine for neuropathic pain—avoid opioids 1, 5
• DVT prophylaxis: Standard measures for all bed-bound patients 3, 5
• Pressure ulcer prevention: Frequent repositioning and skin care 3, 5
• Dysphagia management: Assess swallowing safety and provide nutritional support as needed 3, 5
• Psychological support: Actively screen for and treat anxiety, depression, and hallucinations, which are common but underrecognized 3, 5
• Remember that patients with complete paralysis usually have intact consciousness, vision, and hearing—explain procedures and be mindful of bedside conversations 3

Management of Insufficient Response

Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does not necessarily indicate treatment failure, as progression might have been worse without therapy. 3, 6

• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement, defined as disease progression after initial treatment-induced stabilization 3, 6
• Repeat the full course of IVIg or plasma exchange for TRFs 6, 5
• Suspect chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) if patient experiences three or more TRFs or clinical deterioration ≥8 weeks after onset—these patients may require maintenance IVIg 6, 2

Special Populations

• Children: Use the same 5-day IVIg regimen (0.4 g/kg/day) rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 1
• Pregnant women: IVIg is preferred over plasma exchange due to fewer monitoring requirements, though neither is contraindicated 1
• Miller Fisher Syndrome: Treatment is generally not recommended as most patients recover completely within 6 months without intervention, though close monitoring is essential 1
• IgA deficiency: Check serum IgA levels before first infusion—use IVIg preparations with reduced IgA content if deficiency is confirmed, as this increases anaphylaxis risk 1

Prognosis

• Approximately 80% of patients regain walking ability at 6 months after disease onset 1, 5
• 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
• Use the modified Erasmus GBS outcome score (mEGOS) to calculate individual probability of regaining walking ability 6, 2