Limbic encephalitis: a cause of temporal lobe epilepsy with onset in adult life.
Limbic encephalitis (LE) was described in the 1960s as a clinical-pathological syndrome in adults. Initially, the paraneoplastic form was the center of interest. An increasing number of diagnostically valuable autoantibodies in patients' sera (and cerebrospinal fluid) have been identified. Lately, the impact of non-paraneoplastic LE cases has been acknowledged. In the serum of some of these patients, antibodies against voltage-dependent potassium channels (VGKC antibodies) have been detected. The characteristic MRI course of LE patients has recently been described in detail: hippocampal swelling and T2/FLAIR signal increase are early findings. After a few months, the swelling regresses, followed by hippocampal atrophy with continuous signal increase. A general consensus on formal diagnostic criteria for all LE subsyndromes has not yet been reached. This article proposes such diagnostic criteria and formulates suggestions for treatment.
Bien
Christian G
CG
Department of Epileptology, University of Bonn, Sigmund-Freud-Strasse 25, 53105 Bonn, Germany. christian.bien@ukb.uni-bonn.de
Elger
Christian E
CE
D017668:Age of Onset; D001323:Autoantibodies / Q000032:analysis; D002462:Cell Membrane / Q000276:immunology; D003937:Diagnosis, Differential; D004833:Epilepsy, Temporal Lobe / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D020363:Limbic Encephalitis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D008279:Magnetic Resonance Imaging
D001323:Autoantibodies