What is the recommended acute management for Guillain‑Barré syndrome?

Acute Management of Guillain-Barré Syndrome

For patients with Guillain-Barré syndrome unable to walk unaided, immediately initiate intravenous immunoglobulin (IVIg) 0.4 g/kg daily for 5 consecutive days (total dose 2 g/kg) or plasma exchange 200-250 mL/kg over 4-5 sessions within 4 weeks of symptom onset. 1, 2, 3

Immediate Life-Threatening Assessment

Assess respiratory function and autonomic stability immediately upon presentation, as approximately 20% of patients develop respiratory failure requiring mechanical ventilation, which can occur rapidly and sometimes without obvious dyspnea. 1, 4

Respiratory Monitoring

• Measure vital capacity, negative inspiratory force (NIF), and maximum inspiratory/expiratory pressures at presentation and serially. 1
• Apply 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. 1, 2
• Single breath count ≤19 predicts need for mechanical ventilation. 1
• Admit Grade 3-4 patients (severe weakness limiting self-care, any dysphagia, facial weakness, respiratory muscle weakness, or rapidly progressive symptoms) to inpatient unit with capability for rapid transfer to ICU-level monitoring. 1

Cardiac and Autonomic Monitoring

• Perform electrocardiography and continuously monitor heart rate and blood pressure for arrhythmias and blood pressure instability. 1
• Monitor for dysautonomia including blood pressure/heart rate instability, pupillary dysfunction, and bowel/bladder dysfunction. 1

Diagnostic Workup

Obtain neurology consultation for all suspected GBS cases. 1

Essential Laboratory and Imaging Studies

• Cerebrospinal fluid analysis: Look for albumino-cytological dissociation (elevated protein with normal cell count), though this may be absent in the first week—do not dismiss GBS based on normal CSF protein early in the disease course. 1, 2
• Electrodiagnostic studies (nerve conduction studies and EMG) to evaluate polyneuropathy and classify the subtype (AIDP, AMAN, or AMSAN). 1
• MRI of spine with and without contrast to rule out compressive lesions and evaluate for nerve root enhancement/thickening. 1
• Serum antiganglioside antibody tests for GBS subtypes (e.g., anti-GQ1b for Miller Fisher variant). 1
• Screen for reversible neuropathy causes: HbA1c, vitamin B12, TSH, vitamin B6, folate. 1

First-Line Immunotherapy

Initiate treatment in patients unable to walk unaided within 2-4 weeks of symptom onset. 1, 2, 3

Treatment Options (Equally Effective)

• IVIg 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg). 1, 2, 3
• Plasma exchange 200-250 mL/kg over 4-5 sessions within 4 weeks of symptom onset. 1, 2, 3
• Corticosteroids are NOT recommended for idiopathic GBS. 1, 3

Important Treatment Considerations

• Do not wait for antibody test results before starting treatment if GBS is suspected. 1
• Approximately 40% of patients do not improve in the first 4 weeks following treatment—this does not necessarily mean treatment failed, as progression might have been worse without therapy. 1
• Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months after initial improvement—repeating a full course of IVIg or plasma exchange is common practice. 1

Medications to Avoid

Avoid medications that can worsen neuromuscular transmission: β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides. 5, 1

Supportive Care and Symptom Management

Pain Management

• Use gabapentinoids (gabapentin, pregabalin) or duloxetine for neuropathic pain. 1, 3
• Gabapentin can be initiated alongside IVIg without interaction or contraindication. 1
• Consider tricyclic antidepressants or carbamazepine as alternatives. 1, 3

Neurological Monitoring

• Grade muscle strength using Medical Research Council scale in neck, arms, and legs. 1
• Assess functional disability using GBS disability scale. 1
• Test swallowing and coughing ability to identify aspiration risk. 1
• Check for corneal reflex in patients with facial palsy to prevent corneal ulceration. 1
• Daily neurologic evaluation. 1

General Supportive Measures

• Standard preventive measures for pressure ulcers, hospital-acquired infections, and deep vein thrombosis. 1
• Treatment of constipation/ileus. 1
• Screen for anxiety, depression, and hallucinations, which are frequent complications. 1

Prognosis and Outcome

• 80% of patients regain independent walking ability at 6 months. 1, 2
• Recovery can continue for more than 3 years, with improvement possible even more than 5 years after onset. 1, 2
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications. 1, 2, 4
• Advanced age and severe disease at onset are risk factors for poor outcome. 1
• Use the modified Erasmus GBS outcome score (mEGOS) to predict probability of regaining walking ability. 1, 3

Common Pitfalls

• Do not delay treatment waiting for diagnostic confirmation—initiate immunotherapy based on clinical suspicion if patient cannot walk unaided. 1
• Do not dismiss GBS based on normal CSF protein in the first week—albumino-cytological dissociation may not appear until later. 1
• Do not assume respiratory distress will be obvious—respiratory failure can occur rapidly without dyspnea, requiring serial pulmonary function monitoring. 1, 4
• Consider changing diagnosis to acute-onset CIDP if progression continues after 8 weeks from onset or if patient has three or more TRFs—this occurs in approximately 5% of patients initially diagnosed with GBS. 1, 3