Management of Guillain-Barré Syndrome Associated with Infection
For patients with Guillain-Barré Syndrome (GBS) associated with infection, the recommended management includes discontinuation of any immune checkpoint inhibitors, admission to an inpatient unit with capability for rapid transfer to intensive care, and prompt initiation of intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 days or plasmapheresis for 5 days. 1, 2
Diagnostic Workup
- Obtain neurology consultation to guide the diagnostic approach and management 1
- Perform MRI of spine with and without contrast to rule out compressive lesions and evaluate for nerve root enhancement/thickening 1
- Conduct lumbar puncture with CSF analysis for cell count, differential, cytology, protein, glucose, and cultures (note: CSF typically shows elevated protein and may show elevated WBCs, which is not typical in classical GBS) 1
- Test for serum antiganglioside antibodies for GBS and its subtypes (e.g., anti-GQ1b for Miller Fisher variant) 1
- Perform electrodiagnostic studies (NCS and EMG) to evaluate polyneuropathy 1
- Screen for reversible neuropathy causes: HbA1c, vitamin B12, TSH, vitamin B6, folate, serum protein electrophoresis, and immunofixation 1
Treatment Algorithm
First-Line Immunotherapy (initiate as early as possible)
- Administer IVIg 0.4 g/kg/day for 5 days (total dose 2 g/kg) 2 OR
- Perform plasmapheresis (5 sessions over 1-2 weeks) 2
Corticosteroid Use
- Corticosteroids alone are not recommended for idiopathic GBS 1
- However, in immune checkpoint inhibitor-related GBS, a trial of corticosteroids is reasonable (methylprednisolone 2-4 mg/kg/day) 1
- For severe cases (Grade 3-4), consider pulse steroid dosing (methylprednisolone 1 g daily for 5 days) along with IVIg or plasmapheresis 1
Monitoring and Supportive Care
- Perform frequent neurological assessments and pulmonary function monitoring 1
- Consider using the "20/30/40 rule" to assess respiratory failure risk: patient is at risk if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1, 2
- Monitor for concurrent autonomic dysfunction (heart rate, blood pressure, bowel and bladder function) 1
- Implement nonopioid management of neuropathic pain with pregabalin, gabapentin, or duloxetine 1
- Address constipation/ileus, which is common in GBS patients 1
- Provide appropriate positioning, nutrition, and psychological support 3
Management of Complications
- Implement preventive measures for pressure ulcers, hospital-acquired infections, and deep vein thrombosis 1
- Address specific GBS complications such as inability to swallow in patients with bulbar palsy, corneal ulceration in patients with facial palsy, and limb contractures 1
- Actively assess and manage pain, hallucinations, anxiety, and depression, which are frequent in GBS patients 1
- Avoid medications that can worsen neuromuscular function, such as β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides 1, 2
Management of Clinical Progression
- About 40% of patients do not improve in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness 1
- For treatment-related fluctuations (occurring in 6-10% of patients within 2 months of initial improvement), consider repeating the full course of IVIg or plasma exchange 1, 2
Prognosis
- Approximately 80% of patients regain walking ability at 6 months after disease onset 2
- Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications 2
- Up to two-thirds of deaths occur during the recovery phase, so continued vigilance is necessary even after apparent improvement 1
Special Considerations for Infection-Associated GBS
- When GBS is triggered by a specific infection (e.g., Campylobacter jejuni, Zika virus, Chikungunya), the management approach remains the same, but additional attention to the underlying infection may be necessary 4
- For immune checkpoint inhibitor-related GBS, permanently discontinue the causative agent 1