Treatment of Guillain-Barré Syndrome
Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg body weight daily for 5 consecutive days as first-line treatment for any patient with GBS who cannot walk unaided, starting as early as possible within 2 weeks of symptom onset. 1, 2
First-Line Immunotherapy Selection
IVIg is the preferred first-line treatment over plasma exchange because it is easier to administer, more widely available, has higher completion rates, and demonstrates better tolerability with fewer complications—particularly important in children and pregnant women. 2 Both treatments show equal efficacy, but the practical advantages of IVIg make it the treatment of choice in most clinical settings. 1
Alternative First-Line Option
- Plasma exchange (PE) is an effective alternative when IVIg is contraindicated or unavailable, administered as 200-250 ml plasma/kg in five sessions over 1-2 weeks for patients within 4 weeks of symptom onset. 1, 3
- Do not combine PE followed immediately by IVIg, as this approach does not provide additional benefit. 4
What NOT to Use
- Corticosteroids alone are not recommended for GBS treatment and are ineffective. 3, 4
- IV corticosteroids should be avoided in standard GBS management. 4
Critical Respiratory Monitoring and ICU Admission
Apply the "20/30/40 rule" to identify imminent respiratory failure risk: vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, and maximum expiratory pressure <40 cmH₂O. 2 A single breath count ≤19 also predicts need for mechanical ventilation. 2
ICU Admission Criteria
Admit to ICU if any of the following are present: 2
- Evolving respiratory distress with imminent respiratory insufficiency
- Severe autonomic cardiovascular dysfunction
- Severe swallowing dysfunction or diminished cough reflex
- Rapid progression of weakness
All patients with GBS should be admitted to an inpatient unit with capability for rapid transfer to ICU-level monitoring, regardless of initial severity. 1
Managing Treatment Failures and Fluctuations
Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of initial improvement, representing disease reactivation while the inflammatory phase continues. 1, 2 This differs from patients who never respond to initial treatment.
Management of TRFs
- Repeat the full course of IVIg or switch to PE for patients experiencing TRFs, though evidence supporting this approach is limited. 1, 2
- About 40% of patients do not show improvement in the first 4 weeks following treatment, which doesn't necessarily indicate treatment ineffectiveness. 1
- Do not administer a second IVIg course in GBS patients with poor prognosis outside of TRF scenarios, as this is not recommended. 4
Distinguishing A-CIDP from GBS
- Consider changing the diagnosis to acute-onset CIDP (A-CIDP) if progression continues after 8 weeks from onset or if three or more TRFs occur, which happens in approximately 5% of patients initially diagnosed with GBS. 5, 4
- In A-CIDP cases, corticosteroids can be considered, unlike in standard GBS. 6
Multidisciplinary Supportive Care
Autonomic Monitoring
- Implement continuous ECG monitoring for arrhythmias and blood pressure monitoring for hypertension/hypotension. 2
- Monitor bowel and bladder function as dysautonomia is common. 3
Functional Assessment
- Assess muscle strength using Medical Research Council grading scale and document functional disability using GBS disability scale. 2
Complication Prevention
- Implement preventive measures for pressure ulcers, hospital-acquired infections, and deep vein thrombosis. 1, 3
- Monitor swallowing to prevent aspiration. 3
Pain Management
Pain is common and significantly impacts quality of life—recognize and treat early. 2 Severe pain is reported in at least one-third of patients 1 year after disease onset and can persist for >10 years. 5
Pain Treatment Options
- Use gabapentinoids, tricyclic antidepressants, or carbamazepine for neuropathic pain treatment. 4
- Manage pain with non-opioid treatments when possible. 1
- Chronic pain in GBS includes muscle pain in the lower back and limbs, painful paresthesias, arthralgia, and radicular pain. 5
Rehabilitation
Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 2, 3
Exercise Program Components
- Include range-of-motion exercises, stationary cycling, walking, and strength training to improve physical fitness, walking ability, and independence in activities of daily living. 5, 3
- Monitor exercise intensity closely—overwork causes fatigue. 2 This is a critical pitfall to avoid, as excessive exercise can worsen outcomes.
Fatigue Management
- Fatigue occurs in 60-80% of patients and is often one of the most disabling complaints, unrelated to residual motor deficits. 5
- A graded, supervised exercise program has been shown to reduce fatigue. 5
Prognosis and Long-Term Outcomes
About 80% of patients regain independent walking ability at 6 months after disease onset. 5, 1, 2, 3
Mortality and Risk Factors
- Mortality occurs in 3-10% of cases, most commonly due to cardiovascular and respiratory complications that can occur in both acute and recovery phases. 5, 1, 2, 3
- Risk factors for mortality include advanced age and severe disease at onset. 5, 2
Long-Term Residual Complaints
- Long-term residual complaints include neuropathic pain, weakness, and fatigue, but recovery may still occur >5 years after disease onset. 5, 1, 2, 3
- Recurrent episodes of GBS are rare, affecting 2-5% of patients, but this is still higher than the lifetime risk in the general population (0.1%). 5, 3