Treatment of Guillain-Barré Syndrome
Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days in any patient with GBS who cannot walk unaided, starting as early as possible within 2 weeks of symptom onset. 1, 2, 3
First-Line Immunotherapy
IVIg is the preferred first-line treatment over plasma exchange because it is easier to administer, more widely available, has higher completion rates (better tolerability), and causes fewer complications—particularly important in children and pregnant women. 1, 2, 3
- Plasma exchange (PE) remains an equally effective alternative: 12-15 L over 4-5 exchanges during 1-2 weeks for patients within 4 weeks of symptom onset who cannot walk unaided. 3
- Do not combine PE followed immediately by IVIg—this does not improve outcomes. 3
- Corticosteroids alone are not recommended as randomized controlled trials show no significant benefit, and oral corticosteroids may worsen outcomes. 1, 2, 3
Critical Respiratory Monitoring and ICU Admission
Apply the "20/30/40 rule" to identify imminent respiratory failure risk: 1, 2, 4
- Vital capacity <20 ml/kg
- Maximum inspiratory pressure <30 cmH₂O
- Maximum expiratory pressure <40 cmH₂O
Single breath count ≤19 predicts need for mechanical ventilation. 2
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
Up to 30% of patients develop respiratory failure requiring mechanical ventilation. 4, 5
Monitoring During Treatment
Continuous monitoring is essential: 2, 4
- ECG monitoring for arrhythmias
- Blood pressure monitoring for hypertension/hypotension
- Muscle strength assessment using Medical Research Council grading scale
- Functional disability using GBS disability scale
- Bowel and bladder function
- Swallowing and coughing ability to prevent aspiration
Avoid medications that worsen neuromuscular function: β-blockers, IV magnesium, fluoroquinolones, aminoglycosides, and macrolides. 1
Managing Treatment-Related 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
For TRFs, repeat the full course of IVIg or switch to PE, though evidence supporting this is limited. 2
About 40% of patients do not improve in the first 4 weeks following treatment—this does not necessarily indicate treatment ineffectiveness. 1, 4
Special Populations
In children: 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. 1
In pregnant women: IVIg is preferred over plasma exchange due to fewer monitoring requirements, though neither is contraindicated. 1
For Miller-Fisher Syndrome: Treatment is generally not recommended as most recover completely within 6 months without intervention, though close monitoring is essential. 1
For immune checkpoint inhibitor-related GBS: Discontinue the causative agent permanently and consider concurrent corticosteroids with IVIg or plasma exchange. 1
Pain Management
Severe pain occurs in at least one-third of patients 1 year after onset and can persist >10 years, characterized by muscle pain in lower back and limbs, painful paresthesias, arthralgia, and radicular pain. 6
For neuropathic pain: Use gabapentinoids (gabapentin, pregabalin), tricyclic antidepressants, or carbamazepine. 1, 3
Encourage early mobilization for muscle pain and arthralgia related to immobility. 6
Rehabilitation and Long-Term Management
Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 2
Exercise programs should include: 6, 2
- Range-of-motion exercises
- Stationary cycling
- Walking and strength training
- Monitor exercise intensity closely—overwork causes fatigue
Fatigue affects 60-80% of patients and is often one of the most disabling complaints; graded, supervised exercise programs help reduce fatigue. 6
Prognosis
About 80% of patients regain independent walking ability at 6 months. 1, 2
Mortality occurs in 3-10% of cases, most commonly from cardiovascular and respiratory complications in both acute and recovery phases. 6, 1, 2
Risk factors for mortality: Advanced age and severe disease at onset. 6, 2
Use the modified Erasmus GBS outcome score (mEGOS) to calculate probability of regaining walking ability in individual patients. 6
Recurrent GBS is rare (2-5% of patients), but this is still higher than the lifetime risk in the general population (0.1%). 6
Common Pitfalls to Avoid
Do not delay treatment while ruling out active infection—preceding infections have usually resolved before weakness onset, and the autoimmune process begins 1-3 weeks after the triggering infection. 1
Do not confuse GBS with acute-onset CIDP (A-CIDP)—consider changing the diagnosis to A-CIDP if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. 4, 3
Recognize that about 5% of patients initially diagnosed with GBS actually have A-CIDP and may require different long-term management. 7