What is the treatment for geoborrea syndrome?

Treatment for Guillain-Barré Syndrome

The treatment of choice for Guillain-Barré syndrome (GBS) is intravenous immunoglobulin (IVIg) at 0.4 g/kg daily for 5 days in patients unable to walk unaided within 2 weeks of symptom onset. 1

First-Line Treatment Options

• IVIg (0.4 g/kg daily for 5 days) is recommended for patients within 2 weeks after onset of weakness if they are unable to walk unaided 1
• Plasma exchange (PE) (200-250 ml plasma/kg body weight in five sessions over 1-2 weeks) is an equally effective alternative for patients within 4 weeks after onset of weakness if unable to walk unaided 1, 2
• IVIg is generally preferred over PE due to:
  • Easier administration
  • Wider availability
  • Reduced frequency of adverse effects
  • Lower discontinuation rates 1

Treatment Considerations for Specific Patient Groups

• GBS variants:

  • Pure Miller Fisher Syndrome (MFS): Treatment generally not recommended as most patients recover completely without treatment within 6 months, but close monitoring is essential 1
  • Bickerstaff's brainstem encephalitis (BBE): Treatment with IVIg or PE is justified due to severity, despite limited evidence 1
  • For other clinical variants, many experts administer IVIg or PE despite limited evidence 1

• Pregnant women:

  • Both IVIg and PE are not contraindicated during pregnancy
  • IVIg is preferred as PE requires additional monitoring and considerations 1

• Children:

  • Standard adult treatment practices apply to children
  • IVIg is usually the first-line therapy due to:
    • Limited availability of PE in pediatric centers
    • Greater discomfort with PE
    • Higher complication rates with PE 1
  • The 5-day IVIg regimen is preferred over a 2-day regimen due to fewer treatment-related fluctuations 1

Treatment Limitations and Considerations

• Corticosteroids are not recommended:

  • Eight randomized controlled trials showed no significant benefit
  • Oral corticosteroids may have negative effects on outcomes 1
  • Evidence does not support intravenous methylprednisolone as add-on treatment with IVIg 1

• Combination therapy of PE followed by IVIg is no more effective than either treatment alone 1

• In resource-limited settings:

  • Small-volume plasma exchange (SVPE) might be an economical alternative (~$500 vs. $4,500-5,000 for standard PE or $12,000-16,000 for IVIg) 1
  • However, SVPE requires further validation in larger trials before routine clinical use 1

Monitoring and Supportive Care

• Regular assessment of respiratory function is essential as up to 22% of patients require mechanical ventilation within the first week 1
• The Erasmus GBS Respiratory Insufficiency Score (EGRIS) can help identify patients at risk of respiratory failure 1
• Risk factors for prolonged mechanical ventilation include:
  • Inability to lift arms from bed at 1 week after intubation
  • Axonal subtype or unexcitable nerves in electrophysiological studies 1
• Early tracheostomy should be considered in patients with these risk factors 1

Pain Management

• Pain is a common symptom that may persist for months or years 3
• Gabapentinoids, tricyclic antidepressants, or carbamazepine are weakly recommended for pain management 2

Prognosis and Follow-up

• Despite treatment, GBS remains a severe disease:
  • About 25% of patients require artificial ventilation
  • About 20% are still unable to walk after 6 months
  • 3-10% of patients die 3
• The modified Erasmus GBS outcome score (mEGOS) can be used to assess outcome 2
• The modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) helps assess the risk of requiring artificial ventilation 2
• About 5% of patients initially diagnosed with GBS develop chronic inflammatory demyelinating polyradiculoneuropathy with acute onset (A-CIDP) 3

Common Pitfalls to Avoid

• Failing to monitor respiratory function closely, even in patients without obvious respiratory symptoms 1
• Delaying treatment while waiting for diagnostic confirmation - treatment should begin promptly when GBS is suspected 2
• Misdiagnosing treatment-related fluctuations (occurring in about 10% of patients within 8 weeks after IVIg) as treatment failure 3
• Not recognizing progression beyond 8 weeks as possible A-CIDP rather than GBS 2, 3
• Using oral corticosteroids, which may worsen outcomes 1