Over the past two decades, significant advances have been made in the characterisation of autoimmune encephalitis, its pathophysiology, and the associated autoantibodies. Given the lack of robust clinical trials, the choice of therapy is principally based on observational studies and expert consensus. Current management strategies include immunotherapy, removal of immunological triggers such as tumours when present, and symptomatic treatment of seizures and psychiatric manifestations. With an improved understanding of the underlying pathogenic mechanisms in this rapidly evolving field, the pharmacological treatment of autoimmune encephalitis has evolved over the years, now encompassing various novel therapeutic targets, particularly in the context of third-line immunotherapies. These modalities include B cell depletion, cytokine-targeted therapies, plasma cell-depleting agents, interventions aimed at intrathecal immune cells or their trafficking across the blood-brain barrier, and blockade of the neonatal Fc receptor. This article reviews both established and novel therapeutic approaches for autoimmune encephalitis, with a focus on disease associated with neural surface antibodies, covering immunotherapy and symptomatic management. Additionally, we discuss the unmet needs of patients and the burden of care within this population.
Bunni et al. (Tue,) studied this question.