What is the treatment for Guillain-Barre Syndrome?

Treatment of Guillain-Barré Syndrome

Intravenous immunoglobulin (IVIg) at 0.4 g/kg/day for 5 consecutive days is the first-line treatment for Guillain-Barré syndrome, and should be initiated immediately in any patient who cannot walk independently or shows signs of respiratory compromise. 1, 2, 3

First-Line Immunotherapy

IVIg is preferred over plasma exchange as the initial treatment because it is easier to administer, more widely available, has higher completion rates, and requires less intensive monitoring. 1, 2, 3 Both treatments are equally effective in hastening recovery and reducing long-term morbidity, but IVIg has practical advantages that make it the treatment of choice. 3, 4

Standard IVIg Dosing Protocol

• Dose: 0.4 g/kg body weight daily for 5 consecutive days (total dose 2 g/kg) 1, 2, 3
• Timing: Initiate as early as possible, preferably within 2 weeks of symptom onset 3
• Do NOT use the 2-day accelerated regimen - treatment-related fluctuations occur more frequently with shorter protocols 1, 2

When to Initiate Treatment

Start IVIg immediately if the patient has: 5, 2

• Inability to walk independently (functional grade ≥3)
• Rapidly progressive weakness
• ANY signs of respiratory compromise, dysphagia, facial weakness, or bulbar symptoms
• Respiratory parameters meeting the "20/30/40 rule" (see monitoring section below)

Critical Respiratory Monitoring

Approximately 20% of GBS patients will require mechanical ventilation, making respiratory monitoring the most critical aspect of care. 1, 3, 6

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

A patient is at high risk of respiratory failure if: 1, 3

• Vital capacity <20 mL/kg, OR
• Maximum inspiratory pressure <30 cmH₂O, OR
• Maximum expiratory pressure <40 cmH₂O

Additional Respiratory Assessment

Monitor continuously for: 1, 3

• Single breath count ≤19 (predicts need for mechanical ventilation)
• Use of accessory respiratory muscles
• Inability to cough effectively
• Autonomic dysfunction via ECG, heart rate, and blood pressure

All patients should be admitted to a unit with rapid ICU transfer capability, as respiratory compromise can develop suddenly even during treatment. 5, 2, 3

Medications to AVOID

These medications worsen neuromuscular function and must be avoided: 5, 1, 2, 3

• β-blockers
• Intravenous magnesium
• Fluoroquinolones
• Aminoglycosides
• Macrolide antibiotics

Management of Treatment-Related Fluctuations

6-10% of patients experience treatment-related fluctuations (TRFs) within 2 months of initial improvement. 1, 2 If TRFs occur, repeat the full 5-day course of IVIg (0.4 g/kg/day × 5 days). 1, 2

Important: 40% of patients do not show improvement in the first 4 weeks following treatment - this does NOT indicate treatment failure. 1, 2, 3

Essential Supportive Care

Pain Management

Use non-opioid neuropathic pain medications: 1, 3

• Gabapentin
• Pregabalin
• Duloxetine
• Carbamazepine (alternative)

Complication Prevention

Implement immediately: 1, 3

• DVT prophylaxis (due to immobility)
• Pressure ulcer prevention protocols
• Bowel regimen for constipation/ileus management
• Prevention of hospital-acquired infections (pneumonia, UTIs)
• Psychological support for anxiety, depression, and hallucinations

Special Populations

Pediatric Patients

IVIg is strongly preferred over plasma exchange in children due to better tolerability and fewer complications. 2, 3 Use the same 5-day regimen (0.4 g/kg/day × 5 days). 2

Pregnant Women

IVIg is preferred over plasma exchange during pregnancy because it requires fewer monitoring considerations and additional precautions, though neither treatment is contraindicated. 2, 3

Immune Checkpoint Inhibitor-Related GBS

For patients developing GBS while on immune checkpoint inhibitors: 5

• Permanently discontinue the checkpoint inhibitor for Grade 3-4 symptoms
• Start IVIg (0.4 g/kg/day × 5 days) or plasma exchange
• Add corticosteroids (methylprednisolone 2-4 mg/kg/day OR pulse dosing 1 g/day × 5 days) - this differs from idiopathic GBS where corticosteroids alone are NOT recommended 5
• Taper steroids slowly over 4-6 weeks after pulse dosing 5

Role of Corticosteroids in Idiopathic GBS

Corticosteroids alone are NOT recommended for standard GBS treatment - randomized controlled trials show no significant benefit and oral corticosteroids may worsen outcomes. 2 Corticosteroids should only be used in the specific context of immune checkpoint inhibitor-related GBS as described above. 5

Plasma Exchange as Alternative

Plasma exchange is equally effective to IVIg but is reserved for situations where: 3, 4

• IVIg is contraindicated (e.g., IgA deficiency with anaphylaxis risk)
• IVIg is unavailable
• Patient has failed IVIg treatment

Do NOT perform plasma exchange immediately after IVIg - it will remove the administered immunoglobulin. 5

Prognosis and Recovery

Expected outcomes: 1, 2, 3

• 80% of patients regain walking ability at 6 months
• Mortality is 3-10%, primarily from cardiovascular and respiratory complications
• Recovery can continue for more than 5 years after disease onset
• Recurrence is rare (2-5%) but higher than general population risk (0.1%)

Risk factors for poor outcome: 3

• Advanced age
• Severe disease at onset
• Lack of ICU support when needed

Diagnostic Workup

Before initiating treatment, obtain: 5

• Neurology consultation
• MRI spine with/without contrast (rule out compressive lesions, evaluate nerve root enhancement)
• Lumbar puncture: CSF analysis for elevated protein, cell count, cytology
• Serum antiganglioside antibody tests (including anti-GQ1b for Miller Fisher variant)
• Electrodiagnostic studies (nerve conduction studies and EMG)
• Baseline pulmonary function testing