Table 5

Summary of treatment dilemmas in GBS and recommendations

DilemmaCurrent personal view
Start of treatmentTime windowTreatment should be initiated as soon as possible after diagnosis to prevent further nerve damage (LOE: 3). The effect of IVIg started after 2 weeks and of PE after 4 weeks onset of weakness is unknown (LOE: 4).
Mild formsConsider treating mildly affected patients with a rapidly progressive course or with additional features such as autonomic dysfunction, bulbar or facial weakness (LOE: 2).
 VariantsPatients with typical MFS likely require supportive care only (LOE: 3). In complicated MFS (limb weakness, bulbar weakness) and BBE, treatment with IVIg or PE should be considered (LOE: 4). Other GBS variants should be treated according to local guidelines until results of specific treatment trials show otherwise (LOE: 4).
ChildrenTreatment with IVIg is beneficial in children and IVIg is preferred over PE because it is easier to administer (LOE: 2).
Repeat or change of treatmentInsufficient clinical responseThere is not enough evidence that switching to IVIg after PE is effective in patients who are severely affected (LOE: 2). IVIg followed by PE should probably be avoided (LOE: 4). The effect of a 2nd IVIg course in patients with a poor prognosis is currently investigated.
TRFAlthough there are no RCTs, there is some rationale to re-treat patients who experience a TRF with either IVIg or PE (LOE: 4). When a patient develops 3 or more TRFs or deteriorates 8 weeks after onset, A-CIDP should be considered.
  • A-CIDP, acute-onset chronic inflammatory demyelinating polyneuropathy; BBE, Bickerstaff's brainstem encephalitis; GBS, Guillain-Barré syndrome; IVIg, intravenous immunoglobulin; LOE, level of evidence; MFS, Miller Fisher syndrome; PE, plasma exchange; RCT, randomised controlled trial; TRF, treatment-related fluctuation.