Corticosteroids for Guillain-Barré Syndrome and AMSAN
Corticosteroids are not recommended for the treatment of idiopathic Guillain-Barré syndrome (GBS) or Acute Motor-Sensory Axonal Neuropathy (AMSAN), as multiple randomized controlled trials have demonstrated no benefit and oral corticosteroids may even worsen outcomes. 1, 2, 3
Evidence Against Corticosteroid Use in Idiopathic GBS
Eight randomized controlled trials examining corticosteroids for GBS showed no significant benefit, and treatment with oral corticosteroids demonstrated a negative effect on outcome 1
The American Academy of Neurology explicitly states that corticosteroids are not recommended for the management of GBS based on Level A evidence 2
A Cochrane systematic review of six randomized trials (382 total patients) found no significant difference in disability grade improvement at four weeks between corticosteroid and control groups (weighted mean difference 0.01 grade, 95% CI -0.27 to 0.29) 3
There is no indication that Guillain-Barré patients respond to corticosteroids, in contrast to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who do respond 4
Exception: Immune Checkpoint Inhibitor-Related GBS
The evidence diverges significantly for GBS occurring as an immune-related adverse event from checkpoint inhibitor therapy:
For immune checkpoint inhibitor (ICPi)-related GBS, corticosteroids are reasonable to trial despite lack of efficacy in idiopathic GBS, given the different pathophysiology 1
For severe ICPi-related GBS (Grade 3-4), methylprednisolone 2-4 mg/kg/day is recommended, followed by slow taper, or pulse dosing with methylprednisolone 1 g/day for 5 days may be considered alongside IVIG or plasmapheresis 1
The ASCO guideline notes that "corticosteroids are usually not recommended for idiopathic Guillain-Barré syndrome; however, in ICPi-related forms, a trial is reasonable" 1
Similarly, ESMO guidelines state that "use of steroids not recommended in idiopathic GBS; however, trial of (methyl)prednisolone 1-2 mg/kg reasonable" for ICPi-related cases 1
Established Effective Treatments
The proven effective treatments for GBS/AMSAN are IVIG and plasma exchange, not corticosteroids:
IVIG (0.4 g/kg/day for 5 days, total 2 g/kg) and plasma exchange (200-250 ml plasma/kg over 5 sessions) are equally effective and represent the only treatments with demonstrated efficacy 1, 2
IVIG is usually the treatment of choice because it is easier to administer and more widely available than plasma exchange 1
Combining plasma exchange followed by IVIG is no more effective than either treatment alone and is not recommended 1, 2
Clinical Pitfalls to Avoid
Do not delay IVIG or plasma exchange while attempting corticosteroid therapy in idiopathic GBS, as this wastes critical time during the acute phase 2
Do not confuse GBS with CIDP, as CIDP patients do respond to corticosteroids while GBS patients do not 4
If a patient with GBS requires corticosteroids for another medical indication, its use will probably not cause harm, but it should not be given specifically for GBS treatment 3
Treatment-related fluctuations (TRFs) occur in 6-10% of GBS patients and require repeat IVIG or plasma exchange, not corticosteroid initiation 5