What is the recommended treatment for a patient diagnosed with Guillain-Barré Syndrome (GBS)?

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 critical in children and pregnant women. 1, 2

• Dosing regimen: 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg over the treatment course). 1, 2, 3
• Timing: Treatment should be initiated as early as possible, ideally within 2 weeks of symptom onset, though benefit may extend to 4 weeks. 1, 2, 3
• Alternative option: Plasma exchange (12-15 L in 4-5 exchanges over 1-2 weeks) is equally effective but reserved for situations where IVIg is contraindicated, unavailable, or has failed. 2, 3

What NOT to Do

• Do not use corticosteroids alone—randomized controlled trials show no significant benefit, and oral corticosteroids may worsen outcomes. 1, 2, 3
• Do not combine plasma exchange followed immediately by IVIg—this provides no additional benefit. 3
• Do not give a second course of IVIg to patients with poor prognosis unless they have treatment-related fluctuations (see below). 3

Critical Respiratory Monitoring and ICU Admission

Apply the "20/30/40 rule" to identify imminent respiratory failure: vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O. 1, 2, 4

• Single breath count ≤19 also predicts need for mechanical ventilation. 2
• Admit to ICU if: evolving respiratory distress with imminent respiratory insufficiency, severe autonomic cardiovascular dysfunction, severe swallowing dysfunction or diminished cough reflex, or rapid progression of weakness. 2
• Up to 30% of patients develop respiratory failure requiring mechanical ventilation during hospitalization. 4, 5
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to calculate probability of requiring ventilation. 6, 3

Monitoring During Treatment

Admit patients to an inpatient unit with capability for rapid transfer to intensive care monitoring. 1

• Continuous ECG monitoring for arrhythmias and blood pressure monitoring for hypertension/hypotension due to autonomic dysfunction. 2, 4
• Assess muscle strength using Medical Research Council grading scale and document functional disability using GBS disability scale. 2, 4
• Monitor for aspiration risk: assess swallowing and coughing difficulties. 4
• Verify serum IgA levels before first IVIg infusion—IgA deficiency increases risk of anaphylaxis; use preparations with reduced IgA levels if deficiency is confirmed. 1

Medications to Avoid

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

Managing Treatment-Related Fluctuations (TRFs)

TRFs occur in 6-10% of patients within 2 months of initial improvement and represent disease reactivation while the inflammatory phase continues. 1, 2

• For TRFs, repeat the full course of IVIg (0.4 g/kg/day for 5 days) or switch to plasma exchange. 1, 2
• This is distinct from the 40% of patients who simply do not improve in the first 4 weeks—lack of early improvement does not necessarily indicate treatment ineffectiveness. 1, 4

Distinguishing A-CIDP from GBS

Consider changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) if progression continues after 8 weeks from onset—this occurs in approximately 5% of patients initially diagnosed with GBS. 3

• A-CIDP may benefit from corticosteroids, whereas GBS does not. 3, 7

Multidisciplinary Supportive Care

Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 2

• Exercise programs should include range-of-motion exercises, stationary cycling, walking, and strength training. 6, 2
• Monitor exercise intensity closely—overwork causes fatigue. 6, 2
• Provide DVT prophylaxis and pressure ulcer prevention. 1, 4

Pain Management

Severe pain occurs in at least one-third of patients at 1 year and can persist for >10 years. 6

• First-line for neuropathic pain: gabapentin, pregabalin, or duloxetine. 1, 3
• Avoid opioids when possible. 1
• Pain includes muscle pain in lower back and limbs, painful paresthesias, arthralgia, and radicular pain. 6

Fatigue

Fatigue affects 60-80% of patients and is often one of the most disabling complaints, unrelated to residual motor deficits. 6

• Graded, supervised exercise programs have been shown to reduce fatigue. 6
• Rule out other causes before attributing fatigue solely to GBS. 6

Psychological Support

Early recognition and management of psychological distress is important—rapid loss of physical function can cause severe anxiety and/or depression. 6

• Referral to a psychologist or psychiatrist may be beneficial. 6
• Provide accurate information: 80% regain independent walking at 6 months, and recurrence risk is only 2-5%. 6, 2

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—treatment-related fluctuations occur more frequently with shorter regimens. 1, 7

In pregnant women: IVIg is preferred over plasma exchange because it requires fewer monitoring considerations, though neither is contraindicated. 1

For immune checkpoint inhibitor-related GBS: Discontinue the causative agent permanently and consider concurrent corticosteroids with IVIg or plasma exchange. 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

Prognosis

About 80% of patients regain independent walking ability at 6 months. 6, 2

• Mortality is 3-10%, primarily from cardiovascular and respiratory complications occurring in both acute and recovery phases. 6, 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, 3
• Long-term residual complaints (pain, weakness, fatigue) are common but recovery may still occur >5 years after disease onset. 6
• Recurrence is rare (2-5%) but still higher than the lifetime risk in the general population (0.1%). 6