Although AE can present with dramatic, life-threatening neuropsychiatric deficits, the potential for recovery with prompt treatment is remarkable. First- and second-line therapies for AE lead to clinical improvement in the majority of patients, including full recoveries in many. Early treatment and escalation to second-line therapy in those with refractory disease improves patient outcomes. Novel treatments including IL-6 blockade and proteasome inhibitors have shown promising results in patients with refractory disease.