Table: Management of Guillain Barre Syndrome vs. Myasthenia Gravis

Management of Guillain Barre Syndrome vs. Myasthenia Gravis
Guillain Barre Syndrome
  • Intubation and ventilation
  • Corticosteroids are counterproductive.
  • Plasmapheresis works: 4 exchanges of 1-2 plasma volumes, over 1-2 weeks.
    Plasma exchange for Guillain-Barre syndrome aims to clear the aetiological autoantibody from the bloodstream. In essence, we say "we have no idea which antibody is causing the demyelination, so we will get rid of all of them". The evidence seems to support a 5-treatment regimen; it seems that six treatments are no better than four. Because there is no missing proteins to replace, the exchanged plasma can be FFP or albumin - it does not seem to matter to the resolution of disease. However, because FFP has a slightly higher risk of transfusion reactions, so in general albumin is the recommended replacement solution, unless there are specific reasons to replace blood proteins.
  • IV immunoglobulin is at least as effective as plasmapheresis. Dose is 2g/kg, over 5 days. The college answer mentions a Cochrane review, probably referring to Hughes et al (2014) who demonstrated that in severe disease IVIG within the first 2 weeks "hastens recovery as much as plasma exchange".
Myasthenia Gravis
  • Thymectomy
    • Clear-cut indication in thymoma
    • In absence of thymoma, likelihood of remission is still twice as high if you get your thymus removed (Gronseth et al, 2000)
    • The college answer suggests that thymectomy is "not recommended routinely for age>60", which probably refers to the recent British guidelines (Sussman et al, 2015). They recommend thymectomy for under-45s within 2 years of diagnosis.
  • Maintenance therapy:
    • Acetylcholinesterase inhibitors:  pyridostigmine is the mainstay
    • Immunosuppressants:
      • Corticosteroids
      • Azathioprine
      • Mycophenolate
      • Cyclosporine
  • Crisis therapy:
    • Intubation and ventilation
    • Escalate steroids: eg. prednisolone 1mg/kg/d
    • Acetylcholinesterase inhibitors:  pyridostigmine as IV preparation
    • Plasmapheresis
      • Only useful as a short-term treatment
      • Only applicable in myasthenic crisis
      • Useful as a bridge to slower-acting immunosuppressants
      • Useful preoperatively before thymectomy
      • No real difference in outcomes when compared to IVIG (Gajdos et al, 2002)
    • Intravenous immunoglobulin
      • Like plasmapheresis, only useful as a short-term treatment and only applicable in myasthenic crisis
      • Usually given as 2g/kg over 5 days (i.e. 0.4g/kg per day)
      • A single dose of 1g/kg is probably equally effective (Gajdos, 2005)

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