What is the recommended workup and treatment for Guillain-Barré Syndrome (GBS)?

Workup and Treatment for Guillain-Barré Syndrome

Diagnostic Workup

When GBS is suspected, immediately obtain neurologic consultation, perform lumbar puncture for CSF analysis (expect elevated protein with normal or mildly elevated WBC), and order electrodiagnostic studies (NCS and EMG) to confirm polyneuropathy. 1, 2

Essential Initial Testing

• Neurologic consultation for phenotype determination and ongoing management 1
• Lumbar puncture: CSF analysis showing elevated protein with normal/mildly elevated WBC (albuminocytologic dissociation) supports diagnosis 1
• Electrodiagnostic studies (nerve conduction studies and EMG) to confirm polyneuropathy pattern 1, 3
• MRI spine with and without contrast to rule out compressive lesions and evaluate for nerve root enhancement/thickening 1
• Serum antiganglioside antibody testing for GBS subtypes, particularly anti-GQ1b for Miller Fisher variant (ataxia and ophthalmoplegia) 1, 3

Additional Diagnostic Considerations

• History of recent infection: Ask specifically about diarrhea or respiratory infection in preceding 1-4 weeks, as this increases likelihood of GBS 3
• CSF examination is particularly valuable when diagnosis is uncertain 3
• Nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected 3
• Ultrasound imaging should be considered in atypical presentations 3
• Consider A-CIDP (acute-onset chronic inflammatory demyelinating polyneuropathy) if progression continues beyond 8 weeks from onset, which occurs in approximately 5% of initially diagnosed GBS patients 3

Immediate Treatment

Start intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) as first-line treatment for patients unable to walk unaided or with rapidly progressive symptoms. 2, 4, 5, 3

First-Line Immunotherapy

• IVIg is preferred over plasma exchange due to easier administration, wider availability, higher completion rates, and fewer adverse effects 2, 5, 6
• Timing is critical: IVIg is most effective when started within 2 weeks of symptom onset 5, 3, 6
• Plasma exchange (200-250 mL/kg over 4-5 sessions) is equally effective but harder to complete and has more complications 1, 5, 3
• Do NOT use corticosteroids alone as randomized trials show no benefit and oral corticosteroids may worsen outcomes 2, 3

Treatment Indications

• Grade 2 (moderate): Symptoms interfering with activities of daily living warrant treatment initiation 1
• Grade 3-4 (severe): Any patient with weakness limiting walking, dysphagia, facial weakness, respiratory muscle weakness, or rapidly progressive symptoms requires immediate admission and treatment 1, 2
• GBS disability score ≥3 (unable to walk unaided) is the threshold for treatment 5, 3

Critical Monitoring Requirements

Admit all patients with moderate-to-severe GBS to an inpatient unit with rapid ICU transfer capability, and monitor respiratory function using the "20/30/40 rule": patient is at high risk for mechanical ventilation if vital capacity <20 mL/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O. 2, 4, 5

Respiratory Monitoring

• Approximately 20% of GBS patients require mechanical ventilation 4, 5
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to calculate probability of requiring ventilation 2, 3
• Single breath count ≤19 predicts requirement for mechanical ventilation 4
• Monitor for use of accessory respiratory muscles and ability to cough 4
• Perform frequent pulmonary function testing including vital capacity and maximum inspiratory/expiratory pressures 1, 2

Neurologic and Autonomic Monitoring

• Frequent neurologic checks to assess motor strength, reflexes, and bulbar symptoms 1, 2
• Monitor for autonomic dysfunction via electrocardiography, heart rate, blood pressure, and bowel/bladder function 4
• Daily neurologic review during acute phase 1

Medications to AVOID

Do NOT administer β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, or macrolides as these worsen neuromuscular function and can precipitate respiratory failure. 2, 4, 5

Supportive Care

Pain Management

• Use gabapentinoids (gabapentin, pregabalin), tricyclic antidepressants, or carbamazepine for neuropathic pain 3
• Avoid opioids for neuropathic pain management 1, 2
• Pain is common in GBS and requires aggressive treatment 4, 5

Complication Prevention

• DVT prophylaxis is essential due to immobility 4, 5
• Pressure ulcer prevention through regular repositioning 4, 5
• Prevention of hospital-acquired infections (pneumonia, urinary tract infections) 4, 5
• Treatment of constipation/ileus which is common 1, 4
• Psychological support for anxiety, depression, and hallucinations 4

Management of Treatment Non-Response

Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does NOT indicate treatment failure, as recovery can continue for more than 5 years. 4, 5

Treatment-Related Fluctuations

• 6-10% of patients experience treatment-related fluctuations (TRFs) within 2 months of initial improvement 2, 4, 5
• Repeat the full course of IVIg or plasma exchange for TRFs, although evidence is limited 4, 3
• Do NOT give a second IVIg course in patients with poor prognosis based on mEGOS score 3
• Do NOT combine plasma exchange followed immediately by IVIg as this provides no additional benefit and PE removes the immunoglobulin 1, 3

Special Populations

Pediatric Patients

• 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 2
• IVIg is strongly preferred over plasma exchange in children due to better tolerability and fewer complications 2, 5

Pregnant Women

• IVIg is preferred over plasma exchange as it requires fewer monitoring considerations, though neither is contraindicated 2

Miller Fisher Syndrome

• Treatment is generally not recommended as most patients recover completely within 6 months without intervention, though close monitoring is essential 2

Immune Checkpoint Inhibitor-Related GBS

• Permanently discontinue the checkpoint inhibitor 1
• Consider concurrent corticosteroids (methylprednisolone 2-4 mg/kg/day or pulse dosing 1 g daily for 5 days) along with IVIg or plasma exchange 1, 2

Prognosis Assessment

Use the modified Erasmus GBS Outcome Score (mEGOS) to predict individual patient outcomes. 3

Expected Outcomes

• 80% of patients regain walking ability at 6 months after disease onset 2, 4, 5
• Mortality is 3-10%, most commonly from cardiovascular and respiratory complications 2, 4, 5
• Risk factors for mortality include advanced age, severe disease at onset, and lack of ICU support when needed 5
• Recurrence is rare (2-5%) but higher than general population lifetime risk (0.1%) 4, 5
• Long-term residual complaints include neuropathic pain, weakness, and fatigue 4, 5

Common Pitfalls to Avoid

• Do not delay treatment waiting for CSF results if clinical suspicion is high—start IVIg while awaiting confirmatory testing 2, 3
• Do not assume treatment failure if no improvement occurs in first 4 weeks, as this is expected in 40% of patients 4, 5
• Do not use corticosteroids alone as they are ineffective and may worsen outcomes 2, 3
• Do not overlook respiratory monitoring as deterioration can be rapid and unpredictable 1, 2
• Do not forget to check IgA levels before first IVIg infusion, as IgA deficiency increases anaphylaxis risk 2