Guillain-Barré Syndrome (GBS) Viva Questions
Based on the 2019 Nature Reviews Neurology international consensus guideline 1, here are comprehensive viva questions organized by clinical domains:
Clinical Presentation & Diagnosis
1. What are the cardinal diagnostic features of classic GBS?
- Rapidly progressive bilateral weakness of legs and/or arms without CNS involvement 1
- Distal paraesthesias or sensory loss followed by ascending weakness (legs → arms → cranial muscles) 1
- Decreased or absent reflexes at presentation, universally absent at nadir 1
- Maximum disability reached within 2 weeks in most patients 1
- Critical pitfall: If maximum disability occurs within 24 hours or after 4 weeks, consider alternative diagnoses 1
2. Describe the typical CSF findings in GBS and their timing.
- Classic albuminocytologic dissociation: elevated protein with normal cell count 1
- Important caveat: CSF can be completely normal early in disease course 1
- CSF examination is most valuable when diagnosis is uncertain 2
3. What are the electrophysiological subtypes of GBS?
- AIDP (Acute Inflammatory Demyelinating Polyradiculoneuropathy) 1
- AMAN (Acute Motor Axonal Neuropathy) 1
- AMSAN (Acute Motor Sensory Axonal Neuropathy) 1
- Key point: The demyelinating vs. axonal dichotomy is increasingly challenged and does not guide treatment selection 3
4. What atypical presentations of GBS occur in young children (<6 years)?
- Poorly localized pain 1
- Refusal to bear weight 1
- Irritability and meningism 1
- Unsteady gait 1
- Clinical trap: These nonspecific features frequently cause diagnostic delay 1
Clinical Variants
5. Name the recognized clinical variants of GBS.
- Pure motor variant (weakness without sensory signs) 1
- Miller Fisher Syndrome (ophthalmoplegia, areflexia, ataxia) 1
- Pharyngeal-cervical-brachial weakness 1
- Paraparetic variant (lower limb predominant) 1
- Bilateral facial palsy with paraesthesias 1
6. What is Miller Fisher Syndrome and how is it diagnosed?
- Classic triad: ophthalmoplegia, areflexia, and ataxia 1
- Associated with anti-GQ1b IgG antibodies 1
- Often overlaps with other GBS variants 1
- Testing recommendation: Anti-GQ1b antibody testing should be performed when MFS is suspected 2
7. When should you consider changing the diagnosis from GBS to A-CIDP?
- If progression continues beyond 8 weeks from onset 2
- Occurs in approximately 5% of patients initially diagnosed with GBS 2
Autonomic Dysfunction & Complications
8. What autonomic manifestations occur in GBS?
- Blood pressure instability 1
- Heart rate variability and cardiac arrhythmias 1
- Pupillary dysfunction 1
- Bowel and bladder dysfunction 1
- Mortality impact: Autonomic dysfunction contributes significantly to the 3-10% mortality rate 1
9. What percentage of GBS patients require mechanical ventilation?
- Approximately 20% develop respiratory failure requiring mechanical ventilation 1
- Critical monitoring point: Respiratory failure can occur without dyspnea symptoms 1
- Respiratory function must be monitored in all patients 1
10. What is the mEGRIS score and when should it be used?
- Modified Erasmus GBS Respiratory Insufficiency Score 2
- Used to assess risk of requiring artificial ventilation 2
- Should be calculated on admission 4
Treatment
11. What are the two proven effective treatments for GBS?
- Intravenous immunoglobulin (IVIg): 0.4 g/kg daily for 5 days 1
- Plasma exchange (PE): 200-250 ml/kg over 4-5 sessions 1
- Both are equally effective 1
- Evidence level: These remain the only proven treatments after 30 years 5
12. When should treatment be initiated in GBS?
- IVIg recommended within 2 weeks of weakness onset if unable to walk unaided 2
- PE recommended within 4 weeks of weakness onset if unable to walk unaided 2
- Practice point: Treatment is frequently given even in mild cases (75% of ambulatory patients) despite lack of trial evidence 6
13. Should corticosteroids be used in GBS?
- Recommend against oral corticosteroids 2
- Weakly recommend against IV corticosteroids 2
- No proven benefit in GBS 1
14. What is the approach to treatment failure in GBS?
- Occurs in approximately 32% of patients 6
- Only 35% of treatment failures receive second immunotherapy 6
- Evidence gap: Efficacy of repeat treatment is uncertain 1
- Current practice: Second course is common despite lack of evidence 1
15. Should PE followed immediately by IVIg be used?
Prognosis & Recovery
16. What is the mEGOS score and its clinical utility?
- Modified Erasmus GBS Outcome Score 1
- Should be calculated on admission to predict outcome 1
- Moderately reliable predictor of independent ambulation at 3 months and beyond 4
17. What is the typical recovery timeline in GBS?
- 60-80% walk independently at 6 months 1
- Most extensive improvement occurs in first year 1
- Recovery can continue for >3 years (some sources state >5 years) 1
- Long-term perspective: Clinical improvement may continue beyond 5 years 1
18. What is the recurrence rate of GBS?
19. What are treatment-related fluctuations (TRFs)?
- Deterioration after initial stabilization or improvement on therapy 1
- Occurs in approximately 5% of patients 1
- 68% are re-treated with IVIg or PE 6
- Evidence limitation: No trial data supports re-treatment, but commonly practiced 6
Epidemiology & Etiology
20. What is the global incidence of GBS?
- Approximately 1-2 per 100,000 person-years 1
- More frequent in males than females 1
- Incidence increases with age, though all ages can be affected 1
21. What proportion of GBS patients report preceding infection?
- About two-thirds report infection symptoms in the 6 weeks preceding onset 1
- Recent association: Zika virus epidemics linked to increased GBS incidence 1
- Six pathogens have been temporally associated with GBS 1
Pain Management
22. What pharmacological options exist for pain management in GBS?
- Weakly recommend: Gabapentinoids, tricyclic antidepressants, or carbamazepine 2
- Pain is frequently reported and can be muscular, radicular, or neuropathic 1
- Evidence gap: No specific treatment recommended for fatigue 2
Diagnostic Pitfalls
23. What reflexes might be preserved in atypical GBS?
- Pure motor variant with AMAN subtype may show normal or exaggerated reflexes throughout disease course 1
- Classic teaching challenged: Areflexia is not universal in all GBS presentations 1
24. When is anti-ganglioside antibody testing clinically useful?
- Limited value in typical motor-sensory GBS 2
- Anti-GQ1b testing indicated when Miller Fisher syndrome suspected 2
- Nodal-paranodal antibodies should be tested when autoimmune nodopathy suspected 2
25. When should advanced imaging (MRI/ultrasound) be considered?