Guillain Barré Syndrome
- inflammatory disease of the PNS and most common cause of acute flaccid paralysis
- typical presentation - weakness and sensory signs in legs progressing to arms and cranial muscles
- clinical presentation is heterogeneous with several distinct clinical variants.
- Disease progression can be rapid with most patients reaching maximum disability ≤2/52.
- ~20% develop respiratory failure and require mechanical ventilation
- After initial progressive phase, most reach a plateau phase that can last from days to weeks or months, after which recovery follows
- beware similarities with Dry Beriberi so history is important
10 step approach to Dx and Mx - an expert consensus 2019 |
|
|---|---|
Diagnosis |
|
| 1. When to suspect GBS | • Rapidly progressive bilateral limb weakness +/- sensory deficits • hypo/areflexia • facial or bulbar palsy • opthalmoplegia & ataxia |
| 2. How to diagnose GBS | • check diagnostic criteria • exclude other causes • Consider - routine bloods, CSF exam, Electrophysiological studies |
Acute Care |
|
| 3. When to admit to ICU one or more of: | • Rapid progression of weakness • severe autonomic or swallowing dysfunction • Evolving resp distress • EGRIS>4 (Erasmus GBS Respiratory Insufficiency Score) |
| 4. When to start Rx one or more of: | • inability to walk >10m independently • rapid progression of weakness • severe autonomic or swallowing dysfunction • resp insufficiency |
| 5. Treatment options | • IV immunoglobulin (0.4g/kg daily for 5/7) • plasma exchange (200-250ml/kg for 5 sessions |
| 6. Monitoring | • regular assessments of muscle strength, resp function, swallowing function, BP, HR/rhythm, bowel & bladder control |
| 7. Early complications | • choking • cardiac arrhythmias • infections • DVT • dietary insuffiency, Hyponatraemia • constipation, urinary retention • compression neuropathy, limb contractures • corneal ulceration, pressure ulcers • depression, delirium |
| 8. Clinical progression | • Treatment related fluctuation: repeat same Rx • no initial response or incomplete: no evidence for repeating Rx |
Long term care |
|
| 9. Predicting outcome | • calculate mEGOS (modified Erasmus GBS Outcome Score) on admission • recovery can continue >3y after onset • recurrence is rare (2-5%) |
| 10. Rehabilitation | • start rehab early • manage long term complaints (fatigue, pain, psychological) • support with GBS organisations |