What is the treatment for a patient suspected to have Guillain-Barré Syndrome (GBS)?

Treatment of Guillain-Barré Syndrome

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days immediately upon diagnosis in any patient unable to walk independently, as this is the preferred first-line therapy due to superior ease of administration, wider availability, and higher treatment completion rates compared to plasma exchange. 1, 2, 3

Immediate Assessment and Triage

Upon suspecting GBS, rapidly assess three critical domains that determine ICU admission:

Respiratory function:

• Calculate vital capacity (admit to ICU if <15-20 ml/kg or <1 L) 1
• Assess single breath count (concerning if unable to count to 15 in one breath) 1, 2
• Observe for accessory muscle use, increased respiratory rate, or breathlessness during talking 1
• Apply the "20/30/40 rule": patient at risk if vital capacity <20 ml/kg, maximum inspiratory pressure <30 cmH₂O, or maximum expiratory pressure <40 cmH₂O 2, 3
• Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to calculate probability of requiring mechanical ventilation within 1 week, as up to 22% of patients require ventilation in the first week 1

Autonomic dysfunction:

• Monitor for arrhythmias, marked blood pressure fluctuations, or cardiovascular instability requiring ICU-level monitoring 1

Bulbar involvement:

• Assess swallowing function and cough reflex to prevent aspiration 1, 2

First-Line Treatment Selection

IVIg is preferred over plasma exchange for the following reasons 1, 3:

• No requirement for special equipment or central venous access 4
• Significantly higher completion rates in clinical trials 1
• Equal efficacy to plasma exchange when initiated within 2 weeks of symptom onset 4, 3
• More practical in most clinical settings 4

Plasma exchange remains an effective alternative (200-250 ml plasma/kg over 5 sessions spanning 1-2 weeks) when 1, 5:

• IVIg is contraindicated (such as IgA deficiency with anaphylaxis risk) 3
• IVIg is not tolerated 2
• IVIg is unavailable 2
• For severe GBS requiring ventilation, 4-6 plasma exchange sessions are recommended 4

Critical Treatment Timing

Treatment must be initiated within 2 weeks of symptom onset to modify disease course, as most patients reach maximum disability within this window 1, 4, 3. The European Academy of Neurology/Peripheral Nerve Society guidelines recommend IVIg for patients unable to walk unaided within 2 weeks of weakness onset, with a good practice point extending this to 2-4 weeks 5. Plasma exchange is recommended within 4 weeks of onset 5.

Treatments That Do NOT Work

Do not use corticosteroids alone or in combination with IVIg, as eight randomized controlled trials demonstrated no benefit, and oral corticosteroids showed negative effects on outcomes 1, 2, 3, 5. The European Academy of Neurology/Peripheral Nerve Society weakly recommends against IV corticosteroids 5.

Do not use plasma exchange followed immediately by IVIg, as combination therapy is no more effective than either treatment alone 1, 5.

Special Populations

Children:

• Use the same IVIg dose (0.4 g/kg/day for 5 days) rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter regimens 2, 3
• IVIg is strongly preferred over plasma exchange due to better tolerability and fewer complications 2, 3

Pregnant women:

• Neither IVIg nor plasma exchange is contraindicated 3
• IVIg is generally preferred due to fewer monitoring requirements 3

Obese patients:

• Dose IVIg based on ideal body weight, not actual body weight, as immunoglobulin distributes in plasma and extracellular fluid spaces that correlate with lean body mass 3

Patients with suspected active infection:

• Do not delay IVIg or plasma exchange while investigating infection, as preceding infections typically resolve before GBS weakness onset 1, 3
• Start appropriate antimicrobials concurrently if active infection is documented 3

Monitoring During Treatment

Avoid medications that worsen neuromuscular function 3:

• β-blockers
• IV magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolides

Check IgA levels before first IVIg infusion, as IgA deficiency increases anaphylaxis risk; use IVIg preparations with reduced IgA content if deficiency is confirmed 3.

Monitor rigorously during each infusion for neurological function (motor strength, reflexes, bulbar symptoms) and adverse reactions 3.

Managing Treatment-Related Fluctuations (TRFs)

TRFs occur in 6-10% of patients within 2 months of initial improvement, representing recurrent inflammation rather than treatment failure 1, 2, 4, 3. When TRFs occur, repeat the full course of IVIg or switch to plasma exchange, though evidence supporting this practice is limited 1, 2.

Distinguish TRFs from insufficient initial response: approximately 40% of patients show no improvement in the first 4 weeks, which does not indicate treatment failure—progression might have been worse without therapy 1, 2, 3.

Consider diagnosis revision to acute-onset CIDP if three or more TRFs occur or clinical deterioration continues ≥8 weeks after onset, which happens in approximately 5% of initially diagnosed GBS patients 1, 5.

Supportive Care Essentials

Pain management (severe pain occurs in at least one-third of patients at 1 year) 1:

• The European Academy of Neurology/Peripheral Nerve Society weakly recommends gabapentinoids, tricyclic antidepressants, or carbamazepine 5
• Avoid opioids 3

Prevent complications 2, 3:

• Deep vein thrombosis prophylaxis
• Pressure ulcer prevention
• Hospital-acquired infection surveillance
• Nutritional support if dysphagia present

Psychological support for anxiety, depression, and hallucinations, which are frequent in GBS patients 2.

Prognosis and Recovery

Most patients show extensive recovery, with approximately 80% regaining independent walking ability at 6 months 1, 3. However, mortality occurs in 3-10% of cases, most commonly from cardiovascular and respiratory complications that can occur during both acute and recovery phases 1. Risk factors for mortality include advanced age and severe disease at onset 1, 3.

Recovery continues beyond the first year, potentially for >5 years after disease onset 1, 2. Use the modified Erasmus GBS Outcome Score (mEGOS) on admission to calculate individual probability of regaining walking ability 1, 5.

Long-term residual complaints (neuropathic pain, weakness, fatigue) affect 60-80% of patients but may still improve years after onset 1, 2.

Common Pitfalls to Avoid

Do not wait for CSF albumino-cytological dissociation to confirm diagnosis, as not all patients demonstrate this finding 6.

Do not withhold treatment in patients presenting between 2-4 weeks of symptom onset, as benefit may still occur despite the optimal window being within 2 weeks 4, 3, 5.

Do not give a second course of IVIg to patients with poor prognosis, as the European Academy of Neurology/Peripheral Nerve Society recommends against this practice 5.

Do not confuse Miller Fisher Syndrome with typical GBS: treatment is generally not recommended for Miller Fisher Syndrome as most patients recover completely within 6 months without intervention, though close monitoring remains essential 3.