What is the treatment for Guillain-Barré Syndrome (GBS)?

Treatment of Guillain-Barré Syndrome

Initiate intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days as first-line treatment for any patient with GBS who cannot walk unaided, has respiratory compromise, dysphagia, facial weakness, or bulbar weakness—starting as early as possible within 2 weeks of symptom onset. 1, 2, 3

First-Line Immunotherapy

IVIg is strongly preferred 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, 3

IVIg Dosing Protocol

• Standard regimen: 0.4 g/kg/day for 5 consecutive days (total dose 2 g/kg) 1, 2, 3
• In children: Use the same 5-day regimen rather than accelerated 2-day protocols, as treatment-related fluctuations occur more frequently with shorter courses 1
• In pregnant women: IVIg is preferred over plasma exchange due to fewer monitoring requirements, though neither is contraindicated 1

Alternative: Plasma Exchange

• Use plasma exchange (12-15 L over 4-5 exchanges in 1-2 weeks) if IVIg is unavailable or contraindicated, within 4 weeks of symptom onset 4
• Do not combine: PE followed immediately by IVIg is not recommended 4

What NOT to Use

• Corticosteroids alone are not recommended—randomized trials show no benefit and oral corticosteroids may worsen outcomes 1, 3
• Do not give a second IVIg course to patients with poor prognosis as routine practice 4

Critical Respiratory Monitoring

The "20/30/40 Rule" for Imminent Respiratory Failure

Patient is at high risk if ANY of the following are present: 1, 2, 3

• Vital capacity <20 ml/kg
• Maximum inspiratory pressure <30 cmH₂O
• Maximum expiratory pressure <40 cmH₂O
• Single breath count ≤19 2

ICU Admission Criteria

Admit immediately if: 2

• Evolving respiratory distress with imminent respiratory insufficiency
• Severe autonomic cardiovascular dysfunction
• Severe swallowing dysfunction or diminished cough reflex
• Rapid progression of weakness

Approximately 20-30% of GBS patients require mechanical ventilation, so admit all patients to a unit with rapid ICU transfer capability. 3, 5

Medications to AVOID

Never use these medications in GBS patients as they worsen neuromuscular function: 1, 3

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

Managing Treatment-Related Fluctuations (TRFs)

• TRFs occur in 6-10% of patients within 2 months of initial improvement 1, 2
• Repeat the full course of IVIg or switch to plasma exchange when TRFs occur 1, 2
• Important caveat: About 40% of patients do not improve in the first 4 weeks following treatment—this doesn't necessarily indicate treatment failure 1
• Consider A-CIDP (acute-onset CIDP) if progression continues beyond 8 weeks from onset, which occurs in ~5% of patients initially diagnosed with GBS 4

Essential Supportive Care

Pain Management

Neuropathic pain is common and significantly impacts quality of life—treat early: 2, 3

• First-line: Gabapentinoids (gabapentin or pregabalin), tricyclic antidepressants, duloxetine, or carbamazepine 1, 3, 4
• Avoid opioids for neuropathic pain 3
• Cautious use of narcotic analgesics may be needed for severe pain 6

Cardiovascular and Autonomic Monitoring

• Continuous ECG monitoring for arrhythmias 2
• Blood pressure monitoring for hypertension/hypotension 2
• Monitor bowel and bladder function (constipation/ileus is common) 1, 2

DVT Prophylaxis

• Use heparin and graduated pressure stockings in bed-bound adult patients 6

Nutritional Support

• Evaluate for dysphagia and provide nutritional support if necessary 1

Functional Assessment

• Assess muscle strength using Medical Research Council grading scale 2
• Document functional disability using GBS disability scale 2

Rehabilitation

Initiate early rehabilitation with a multidisciplinary team including physiotherapists, occupational therapists, speech therapists, and dietitians. 2, 3

• Include range-of-motion exercises, stationary cycling, walking, and strength training 2
• Monitor exercise intensity closely—overwork causes fatigue 2
• Persistent fatigue following GBS is common and may be helped by an exercise program 6

Special Populations

Miller-Fisher Syndrome

• Treatment is generally not recommended as most recover completely within 6 months without intervention 1
• Close monitoring is essential 1

Immune Checkpoint Inhibitor-Related GBS

• Discontinue the causative agent permanently 1
• Consider concurrent corticosteroids with IVIg or plasma exchange 1

Prognosis

• 80% of patients regain independent walking ability at 6 months 1, 2, 3
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications 1, 2, 3
• Risk factors for mortality: advanced age and severe disease at onset 1, 2
• Recovery can continue for more than 5 years after disease onset 3
• Recurrence is rare (2-5%) 3

Prognostic Tools

• Use the modified Erasmus GBS Outcome Score (mEGOS) to assess outcome 4
• Use the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to calculate probability of requiring ventilation 1, 4