What is the management approach for a patient presenting with suspected Guillain-Barré Syndrome (GBS)?

Management of Suspected Guillain-Barré Syndrome (GBS)

Patients with suspected Guillain-Barré Syndrome require immediate hospitalization with capability for rapid ICU transfer, prompt initiation of intravenous immunoglobulin (IVIG) or plasma exchange, and close monitoring for respiratory compromise. 1

Initial Assessment and Diagnosis

• Neurological evaluation:

  • Pattern of weakness (typically ascending, symmetrical)
  • Sensory deficits
  • Deep tendon reflexes (usually diminished or absent)
  • Cranial nerve involvement
  • Respiratory function

• Essential diagnostic tests:

  • Lumbar puncture: Look for albumino-cytological dissociation (elevated protein with normal cell count) 2, 1
  • MRI spine with/without contrast: Rule out compressive lesions 2, 1
  • Electrodiagnostic studies (NCS/EMG): Determine GBS subtype 1
  • Anti-ganglioside antibody testing (especially anti-GQ1b for Miller Fisher variant) 2, 1

Respiratory Monitoring

• Implement the "20/30/40 rule" for respiratory monitoring 1:

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

• Consider using the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to identify patients at risk for mechanical ventilation 1

• Frequent pulmonary function assessments and neurological checks 2

Treatment Protocol

First-line Immunotherapy (start within 2 weeks of symptom onset)

• IVIG: 0.4 g/kg/day for 5 consecutive days (total 2 g/kg) 2, 1, 3
  • Preferred in children and pregnant women due to lower complication rates 1
  • Consider in all patients unable to walk unaided

OR

• Plasma exchange: 200-250 ml plasma/kg body weight in 4-5 exchanges over 1-2 weeks 1, 3
  • Equally effective as IVIG but has higher complication rates
  • Requires specialized equipment

Important Treatment Considerations

• Do NOT use:
  • Combination therapy (PE followed by IVIG) - no additional benefit 1, 3
  • Corticosteroids alone - not recommended and may have negative effects 1, 3
  • Second IVIG course in patients with poor prognosis is not routinely recommended 3

Supportive Care

• Pain management:

  • First-line: Gabapentinoids (pregabalin, gabapentin) 2, 1
  • Second-line: Tricyclic antidepressants or carbamazepine 1
  • Avoid opioids when possible 2

• Autonomic dysfunction management:

  • Monitor for cardiac arrhythmias, blood pressure fluctuations
  • Treat hypotension with fluids and vasopressors if needed

• DVT prophylaxis:

  • Compression stockings and anticoagulation

• Prevent complications:

  • Pressure ulcer prevention
  • Bowel and bladder management
  • Prevention of hospital-acquired infections
  • Range-of-motion exercises to prevent contractures

Monitoring Disease Progression

• Use Medical Research Council grading scale for muscle strength 1
• Track functional disability using GBS disability scale 1
• Monitor for treatment-related fluctuations (occur in 6-10% of patients within 2 months) 3, 4
• Be alert for progression to chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with acute onset (occurs in ~5% of patients) 3, 4

Criteria for ICU Admission

• Rapidly progressive weakness
• Any signs of respiratory compromise
• Bulbar weakness affecting swallowing/airway protection
• Autonomic instability
• Severe disability (unable to walk)

Prognosis and Rehabilitation Planning

• Mortality rate is 3-10% despite optimal care 1, 3
• Approximately 80% regain independent walking by 6 months 1
• Use modified Erasmus GBS outcome score (mEGOS) to predict recovery of walking ability 2, 1
• Arrange comprehensive rehabilitation program before discharge 2
• Address potential long-term issues: fatigue, pain, and psychological distress 2

Despite advances in treatment, GBS remains a serious condition with potential for significant morbidity and mortality. Early recognition, prompt treatment, and meticulous supportive care are essential for optimizing outcomes.