Recent identification of syndromes encompassing psychiatric symptoms, seizures, and movement disorders has led to effective treatments for several previously obscure conditions now known to be immune-mediated encephalopathies. In contrast to long-recognized paraneoplastic neurologic syndromes associated with antibodies to intracellular antigens, these more recently described disorders are not always paraneoplastic, are associated with cell surface antibodies, and may respond to immunosuppressive therapies. In this review, the author discusses clinical presentations, differential diagnosis, immunologic evaluation, and therapeutic options for both groups of disorders.