What are the diagnosis and treatment options for Guillain Barre Syndrome (GBS)?

Diagnosis and Treatment of Guillain-Barré Syndrome (GBS)

Guillain-Barré Syndrome (GBS) requires prompt diagnosis and treatment with intravenous immunoglobulin (IVIG) as first-line therapy, initiated within 2 weeks of symptom onset at a dose of 0.4 g/kg daily for 5 consecutive days in patients unable to walk unaided. 1

Diagnosis

Clinical Presentation

• Progressive ascending weakness in limbs evolving over days to 4 weeks
• Often preceded by respiratory or gastrointestinal infection
• May include sensory disturbances, cranial nerve involvement, respiratory insufficiency, autonomic dysfunction, and pain

Diagnostic Approach

1. Neurological Examination:

   • Complete neurological evaluation focusing on pattern of weakness, sensory deficits, and reflexes
   • Assess for cranial nerve involvement, especially facial and bulbar muscles

2. Laboratory Tests:

   • Lumbar puncture: Typically shows albuminocytologic dissociation (elevated protein with normal cell count)
   • Note: CSF may be normal early in disease course (first week) 2
   • Anti-ganglioside antibody testing: Limited value in typical GBS but should be considered when Miller Fisher syndrome is suspected (anti-GQ1b antibodies) 1

3. Electrophysiological Studies:

   • Helps distinguish between subtypes: AIDP (demyelinating), AMAN (motor axonal), and AMSAN (sensorimotor axonal)
   • May be normal early in disease course 2

4. Imaging:

   • MRI with contrast of spine should be considered in atypical cases 1

Treatment

Immunotherapy

1. First-line Treatment:

   • IVIG: 0.4 g/kg daily for 5 consecutive days (total 2 g/kg)

     • Should be initiated within 2 weeks of symptom onset
     • Preferred over plasma exchange due to easier administration, wider availability, and fewer complications 1

   • Plasma Exchange: Alternative first-line treatment

     • 200-250 ml plasma/kg body weight in 4-5 sessions over 1-2 weeks
     • Equally effective as IVIG but requires specialized equipment and has higher complication rates 1

2. Treatment Considerations:

   • Combination therapy (plasma exchange followed by IVIG) is not recommended as it shows no additional benefit 1
   • Corticosteroids are not recommended as they show no benefit and may have negative effects 1
   • Second course of IVIG is not recommended for patients with poor prognosis, though this practice is common in patients who show deterioration after initial response 1

Respiratory Management

1. Monitoring:

   • Apply the "20/30/40 rule" for respiratory monitoring 2, 1:
     • Vital capacity < 20 ml/kg
     • Maximum inspiratory pressure < 30 cmH₂O
     • Maximum expiratory pressure < 40 cmH₂O
   • Single breath count ≤ 19 predicts need for mechanical ventilation 2

2. ICU Admission Criteria:

   • Evolving respiratory distress
   • Severe autonomic cardiovascular dysfunction
   • Severe swallowing dysfunction
   • Rapidly progressive weakness 1

3. Ventilatory Support:

   • Up to 30% of patients may require mechanical ventilation 3
   • Consider early tracheostomy in patients likely to require prolonged ventilation 4

Supportive Care

1. Pain Management:

   • First-line: Gabapentinoids (pregabalin, gabapentin)
   • Second-line: Tricyclic antidepressants or carbamazepine 1

2. Prevention of Complications:

   • DVT prophylaxis for immobilized patients
   • Pressure ulcer prevention
   • Prevention of hospital-acquired infections 2

3. Management of GBS-specific Complications:

   • Swallowing difficulties: Consider nasogastric feeding
   • Facial palsy: Eye protection to prevent corneal ulceration
   • Limb contractures: Early physiotherapy
   • Autonomic dysfunction: Cardiac monitoring, blood pressure management 2

4. Psychological Support:

   • Address anxiety, depression, and hallucinations
   • Remember patients typically have intact consciousness, vision, and hearing even when paralyzed 2

Monitoring Disease Progression and Prognosis

1. Regular Assessment:

   • Muscle strength using Medical Research Council grading scale
   • Functional disability using GBS disability scale 2, 1

2. Prognostic Tools:

   • Modified Erasmus GBS Outcome Score (mEGOS): Predicts probability of regaining walking ability
   • Erasmus GBS Respiratory Insufficiency Score (EGRIS): Identifies patients at risk of requiring mechanical ventilation 1

3. Disease Course:

   • Treatment-related fluctuations (TRFs) occur in 6-10% of patients within 2 months of treatment 2
   • About 5% of patients initially diagnosed with GBS develop chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with acute onset (A-CIDP) 5
   • Mortality rate: 3-10% despite optimal care 1
   • Approximately 80% regain independent walking by 6 months 1
   • Greatest recovery occurs in first year but may continue for >5 years 1

Special Populations

1. Children:

   • IVIG is preferred over plasma exchange due to lower complication rates
   • Standard 5-day IVIG regimen preferred over 2-day regimen (associated with more TRFs) 2

2. Pregnant Women:

   • IVIG preferred over plasma exchange, though neither is contraindicated 2

By following this structured approach to diagnosis and treatment, clinicians can optimize outcomes for patients with this potentially devastating but treatable condition.