Centre de référence de diagnostic et de traitement des syndromes neurologiques paranéoplasiques, Hôpital Pierre Wertheimer, Hospices Civils de Lyon, 59 boulevard Pinel, 69003 Lyon, Service d'explorations fonctionnelles neurologiques, Hôpital Lyon Sud, Hospices Civils de Lyon, Unité d'épilepsie et département de neurophysiologie clinique, Hôpital de la Pitié-Salpêtrière, Paris
- Key words: limbic encephalitis, paraneoplastic neurological syndrome, onconeuronal antibody, neuronal surface cell antibody, NMDAr-antibody, LGI1-antibody, Hu-antibody
- DOI : 10.1684/epi.2010.0352
- Page(s) : 281-7
- Published in: 2010
The association of an acute or subacute memory impairment, seizures and psychiatric disorders defines limbic encephalitis (LE). This disease has been early identified as paraneoplastic but the pathophysiological link between cancer and neurological disorders remained unknown. Studies performed in the last few years leaded to reshape the concept of LE and to precise the pathophysiology. Thus, the type of autoantibody associated with each subtype EL determines the main autoimmune mechanism underlying the neurological disorders, type of associated tumour (if the identified autoantibody suggests a paraneoplastic form), clinical syndrome and treatment that is most likely to be effective. The recent description of new autoantibodies targeting neuronal surface cell antigens or secreted proteins (i.e., LGI1) allow the identification of new clinical presentation no longer limited to LE symptoms. Autoimmune encephalitis, which were previously considered viral are subsequently defined.