Immune Checkpoint Inhibitor–related Guillain-Barré Syndrome: A Case Series and Review of the Literature

Immune checkpoint inhibitors (ICIs) have been approved for the treatment of various malignancies with promising clinical outcomes. Treatment can, however, be accompanied by serious immune-related adverse events. Neurological adverse events like Guillain-Barré syndrome (GBS) are rare but potentially life-threatening. We present 3 cases of ICI-related GBS; review cases described in current literature, and discuss treatment strategies. Three patients developed GBS after ICI treatment. The first case with pembrolizumab had a fatal outcome despite treatment with multiple regimens, including steroids and intravenous immunoglobulin (IVIg). The other 2 cases with nivolumab-induced and pembrolizumab-induced GBS, respectively, responded well to treatment with IVIg and steroids. In the current literature, a total of 31 other cases were found. Treatment for ICI-related GBS mostly consisted of concurrent IVIg and steroids (44%), which led to clinical improvement in 73%. Most patients recovered with remaining symptoms (68%), while 10 patients developed respiratory failure (29%) and 6 patients (18%) died. ICI-related GBS should be suspected in patients on ICI treatment who develop subacute progressive weakness of the limbs, sensory loss, and areflexia. On the basis of the guidelines recommendations and our review of the literature, we advise first-line therapy with concurrent IVIg 0.4 g/kg/d for 5 days and prednisolone 1–2 mg/kg/d. Discontinuation of immunotherapy after ICI-related GBS is advised.