Background: The spectrum of central nervous system autoimmune disorders has recently expanded with the discovery of disorders associated with antibodies directed against the neuronal membrane surface. Although many of these disorders have an underlying malignancy and present with signs of dysfunction of the limbic system (paraneoplastic limbic encephalitis, PLE), a high proportion of cases is non-paraneoplastic. They may occur with milder symptoms, and present no abnormalities in the exams usually used in the investigation of PLE. A striking number of cases may be misdiagnosed as primary psychiatric or other neurological disorders. This paper aims to review the current knowledge on this topic, and provide physicians with an updated text on the neuropsychiatric presentation, diagnostic approach and current management of autoimmune encephalopathies.