What is the diagnosis, treatment, and prognosis of Guillain Barre (Guillain-Barré) syndrome?

Guillain-Barré Syndrome: Diagnosis, Treatment, and Prognosis

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyneuropathy characterized by rapidly progressive bilateral weakness that requires prompt diagnosis and treatment with either intravenous immunoglobulin or plasma exchange to improve outcomes and reduce mortality. 1

Diagnosis

Clinical Presentation

• Required diagnostic features: 2

  • Progressive bilateral weakness of arms and legs (may initially involve only legs)
  • Absent or decreased tendon reflexes in affected limbs

• Supportive clinical features: 2, 1

  • Progressive phase lasting days to 4 weeks (usually <2 weeks)
  • Relative symmetry of symptoms
  • Mild sensory symptoms (absent in pure motor variant)
  • Cranial nerve involvement, especially bilateral facial palsy
  • Autonomic dysfunction
  • Muscular or radicular back/limb pain
  • History of preceding infection (approximately 2/3 of patients report respiratory or diarrheal illness 4-6 weeks before onset)

Diagnostic Testing

1. Cerebrospinal fluid (CSF) examination: 2, 1

   • Classic finding: Albumino-cytological dissociation (elevated protein with normal cell count)
   • Important caveat: Protein levels are normal in 30-50% of patients in the first week and 10-30% in the second week
   • Marked pleocytosis (>50 cells/μl) suggests alternative diagnoses

2. Electrodiagnostic studies: 2, 1

   • Not required but helpful, especially in atypical presentations
   • Typically shows sensorimotor polyradiculoneuropathy/polyneuropathy
   • Characteristic "sural sparing pattern"
   • May be normal early in disease course (within first week)
   • Can help differentiate subtypes: AIDP (demyelinating), AMAN (axonal motor), AMSAN (axonal sensorimotor)

3. Anti-ganglioside antibody testing: 2, 1

   • Limited diagnostic value in typical GBS
   • Anti-GQ1b antibodies found in up to 90% of Miller Fisher syndrome cases

4. MRI: 2

   • Not part of routine diagnosis
   • Consider in atypical cases to rule out other causes

Clinical Variants

• Classic sensorimotor GBS (most common)
• Pure motor variant
• Miller Fisher syndrome (ophthalmoplegia, areflexia, ataxia)
• Pharyngeal-cervical-brachial weakness
• Paraparetic variant (legs only)
• Bilateral facial palsy with paresthesias 2, 1

Treatment

Immunotherapy

1. Intravenous immunoglobulin (IVIg): 1, 3

   • Recommended dose: 0.4 g/kg/day for 5 consecutive days
   • Should be initiated as soon as possible after diagnosis
   • For patients who cannot walk unaided within 2-4 weeks of symptom onset

2. Plasma exchange (PE): 1, 3

   • Alternative to IVIg: 12-15 L in 4-5 exchanges over 1-2 weeks
   • Effective when initiated within 4 weeks of symptom onset
   • For patients who cannot walk unaided

3. Treatment considerations:

   • IVIg is generally preferred for practical reasons 4
   • Combining PE followed by IVIg is not recommended 3
   • Second IVIg course is not recommended for patients with poor prognosis 3
   • Corticosteroids are not recommended as monotherapy 1, 3

Supportive Care

1. Respiratory monitoring:

   • About 20-25% of patients develop respiratory failure requiring mechanical ventilation 1, 4
   • Use modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess risk 3

2. Pain management: 1, 3

   • Consider gabapentinoids, tricyclic antidepressants, or carbamazepine

3. Autonomic dysfunction management:

   • Close monitoring of blood pressure and heart rate 1

4. Thromboembolism prophylaxis 1

Prognosis

• Mortality: 3-10% despite optimal treatment 1

• Recovery timeline: 1, 4

  • 60-80% of patients able to walk independently by 6 months
  • Use modified Erasmus GBS outcome score (mEGOS) to predict recovery

• Poor prognostic factors: 1, 4

  • Advanced age
  • Rapid progression of symptoms
  • Need for mechanical ventilation
  • Severe axonopathy on electrophysiological studies

• Long-term complications: 4

  • Pain, fatigue, and other residual symptoms may persist for months to years

• Recurrence: Rare (2-5% of patients) 1

• Treatment-related fluctuations (TRF): About 10% of patients have secondary deterioration within 8 weeks after IVIg, requiring repeated treatment 4

• Acute-onset CIDP: About 5% of patients initially diagnosed with GBS develop chronic inflammatory demyelinating polyradiculoneuropathy with acute onset (A-CIDP) 4, 3

Special Considerations

• Pediatric presentation: Young children (<6 years) may present with nonspecific features like poorly localized pain, refusal to bear weight, and irritability 1

• Atypical features that cast doubt on diagnosis: 2

  • Marked persistent asymmetry
  • Bladder/bowel dysfunction at onset
  • Fever at onset
  • Nadir <24 hours
  • Continued progression >4 weeks
  • Sharp sensory level
  • Hyperreflexia or clonus

By recognizing the clinical presentation early, confirming with appropriate diagnostic tests, and promptly initiating immunotherapy, outcomes for patients with GBS can be optimized despite the potentially severe nature of this condition.