Autoimmune limbic encephalitis

Abstract

Limbic encephalitis (LE) was first described in 1960 by Brierley et al. and was defined by an acute or subacute temporal lobe epilepsy, memory loss and psychiatric disturbances. Corsellis et al. then demonstrated that LE was associated with small-cell lung cancer and a paraneoplastic origin of LE was suggested. In the 1980s, onconeuronal antibodies specific to paraneoplastic neurological syndromes were described and some of them, such as Hu-Ab, were clearly associated with LE. In the last 20 years, other onconeuronal antibodies targeting intracellular antigens were subsequently identified and LE was considered as a rare paraneoplastic neurological syndrome with a poor outcome and as mainly being associated with Hu-Ab, Ma2-Ab or CV2/CRMP5-Ab. The concept of LE has dramatically evolved since 2004, with the description of LE becoming associated with novel autoantibodies that target neuronal surface rather than intracellular antigens. In these cases, LE was not always paraneoplastic. Interestingly, these newly described LE cases offer a much better prognosis than the previous ones. At present, LE is considered to be an autoimmune disorder that can be paraneoplastic or not and could be more frequent than previously suspected. The associated antibodies lead to the classification of different subtypes of LE and are used as prognostic markers. Those that are directed against cell surface antigens could play a direct role in the neurological symptoms of LE.