Anti-Ma2 Encephalitis: An Atypical Case Associated With Klüver-Bucy Syndrome and Hypothalamic Dysfunction

Abstract

Anti-Ma2 encephalitis is a rare form of autoimmune encephalitis that has classically been described as a paraneoplastic neurobehavioral disorder due to its association with underlying malignancies. We discuss the case of a 30-year-old female with an exceptionally aggressive presentation of anti-Ma2 encephalitis accompanied by Klüver-Bucy syndrome and hypothalamic dysfunction. Her course was complicated by repeated aspiration events secondary to severe hyperphagia and delays in immunosuppressive treatment due to concerns of infection. The patient’s encephalitis was refractory to multiple immunosuppressive therapies and she ultimately expired before a primary malignancy could be detected and treated.