Abstract

An increasing number of neuronal autoantibodies which target cell surface or synaptic proteins have been discovered over the last decade. Autoimmune encephalitis refers to this new category of autoimmune-mediated neurological disorders, which involve the central nervous system. Recent studies have established that autoimmune encephalitis is now the major cause of encephalitis, which was previously considered to be encephalitis of an unknown etiology. Moreover, the fact that autoimmune encephalitis is potentially treatable with immunomodulating therapy has changed the paradigm for the diagnosis and treatment of acute encephalitis syndrome. We herein review the pathophysiology, clinical manifestations, diagnosis, and treatment of autoimmune encephalitis with a focus on corticosteroid therapy as the first-line immunotherapy. In addition, regarding the diagnostic approach, we emphasize the differentiation between autoimmune and infectious encephalitis, because this distinction is not necessarily clear-cut in real clinical practice and should be considered when determining the initiation and type of immunotherapy. Key Words: Autoimmune diseases of the nervous system; Immunotherapy; Glucocorticoids; Autoantibodies

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