Guillain-Barré Syndrome: Clinical Presentation and Management
A patient with Guillain-Barré syndrome typically presents with tingling sensation in the extremities, ascending symmetric paralysis, and absent or reduced deep tendon reflexes. 1
Clinical Presentation
- Progressive, most often symmetrical muscle weakness with absent or reduced deep tendon reflexes 1
- Often starts with sensory symptoms/neuropathic pain localized to lower back and thighs 1
- Tingling sensations (paresthesias) in the extremities are typically the initial symptoms 1
- Weakness typically follows an ascending pattern (starting in lower extremities and progressing upward), though this is not universal 1
- May involve facial, respiratory, bulbar, and oculomotor nerves 1
- Dysregulation of autonomic nerves may occur, affecting blood pressure, heart rate, and bowel/bladder function 1
Diagnostic Evaluation
- Neurologic consultation is essential for proper diagnosis 1
- MRI of spine with or without contrast to rule out compressive lesions 1
- Lumbar puncture typically shows elevated protein with normal or mildly elevated WBCs (albuminocytologic dissociation) 1
- Serum antiganglioside antibody tests for GBS subtypes 1
- Electrodiagnostic studies to evaluate polyneuropathy 1
- Pulmonary function testing to assess respiratory status 1
Monitoring
- Regular assessment of respiratory function is critical as up to 30% of patients develop respiratory failure 1, 2
- Use the "20/30/40 rule" to assess risk of respiratory failure: vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 1, 3
- Monitor muscle strength in neck, arms, and legs using the Medical Research Council grading scale 1
- Assess swallowing and coughing difficulties to prevent aspiration 1, 3
- Monitor for autonomic dysfunction via ECG, heart rate, blood pressure, and bowel/bladder function 1, 3
Treatment
First-Line Therapies
- Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 days (total dose 2 g/kg) or plasmapheresis are the first-line treatments for GBS. 1, 3, 4
- Treatment should be initiated as early as possible in the disease course 3
- Corticosteroids alone are not recommended for GBS treatment as they have shown no significant benefit 3, 5
- For immune checkpoint inhibitor-related GBS, a trial of corticosteroids may be reasonable (methylprednisolone 2-4 mg/kg/day) 1
Management Based on Severity
- Grade 2 (moderate symptoms interfering with ADLs): Discontinue any immune checkpoint inhibitors if applicable 1
- Grade 3-4 (severe symptoms limiting self-care, walking, or causing respiratory compromise): Admission to inpatient unit with capability of rapid transfer to ICU 1, 6
- For severe cases, frequent neurologic checks and pulmonary function monitoring are essential 1, 2
Complications and Supportive Care
- Up to two-thirds of deaths in GBS occur during the recovery phase due to cardiovascular and respiratory dysfunction 1
- Common complications include pressure ulcers, hospital-acquired infections, and deep vein thrombosis 1, 3
- Pain, hallucinations, anxiety, and depression are frequent and require specific management 1, 3
- Multidisciplinary supportive care is crucial, including physiotherapy, occupational therapy, and speech therapy 1, 3
Prognosis
- About 40% of patients do not show improvement in the first 4 weeks following treatment 1, 3
- Treatment-related fluctuations occur in 6-10% of patients within 2 months of initial improvement 1
- Despite treatment, approximately 20% of patients have persistent neurologic symptoms 6
- Mortality rate is approximately 3-10%, primarily due to autonomic dysfunction and respiratory complications 6, 5
Special Considerations
- Monitor closely for respiratory insufficiency as clinical signs of dyspnea may occur too late 2
- Regular measurement of vital capacity and respiratory pressures helps determine the need for mechanical ventilation 2
- Rapid progression of weakness, involvement of both peripheral and axial muscles, ineffective cough, and bulbar weakness predict need for ventilatory support 2