Guillain-Barré Syndrome



Background

  • Acute inflammatory demyelinating polyneuropathy (delayed conduction in nerve fibres)
  • Progressive symmetric ascending muscle weakness
  • Cranial nerve variant = Miller Fisher
  • Hyporeflexia
  • ± sensory
  • ± autonomic involvement
  • Autoimmune response following recent infection, symptoms resolving with remyelination
  • <10% death rate (usually ARDS, PE, Sepsis, pneumonia)
  • Young adults and elderly

History

  • Recent 'flu' like illness then
  • Paraesthesia in digits
  • Progressive, ascending, proximal muscle weakness (rising from sitting, combing hair)
  • Shoulder and hip girdle pain
  • Beware respiratory muscle or bulbar involvement

Examination

  • Absent reflexes knee and ankle reflexes
  • Loss vibration / proprioception first
  • Autonomic disturbance (BP, pulse, urinary retention, salivation)
  • Tonic pupils

Clinical Diagnosis

  • CSF - Raised protein, few WBC
  • Studies to out-rule viral and toxidromes
  • NB - check the FVC (forced vital capacity) - <20ml/kg may need intubation
  • ECG - AV block, T and ST abnormalities, Wide QRS

Treatment

  • O2 ± assisted ventilation (particularly low FVC, aspiration)
  • IV access
  • Monitor
  • Atropine (± pacing) for bradycardia
  • Short-acting β-blocker if severe hypertension
  • DVT prophylaxis
  • Plasma exchange (PE), immunoglobulin (IVIG) are effective
  • Steroids ineffective

Differential Dx


Content by Dr Íomhar O' Sullivan 28/11/2008. Last review Dr ÍOS 6/09/16.