title: [Recent changes in the paradigm of limbic encephalitis].
abstract: In the recent years, novel antibodies associated with limbic encephalitis have been described, which target such extracellular receptors or proteins that have been already indicated in the pathogenesis of hereditary or degenerative diseases. In a number of cases, where pathogenic role of antibodies generated against the voltage-gated potassium channel (VGKC) had been presumed, antibodies against a trans-synaptic scaffolding protein, LGI1 were indicated. Antibody response against NMDA-receptors has been suggested as a major cause of limbic encephalitis especially in young females, resulting in a typical clinical syndrome sometimes triggered by an ovarian teratoma. Antibodies against other receptors essential in synaptic transmission and plasticity (AMPA and GABA, receptors) have been also indicated, partially elicited by paraneoplastic processes. Such antibodies against surface proteins result in severe but potentially treatable diseases due to reversible internalization of the antigens crosslinked by the bivalent antibodies. In contrast, the rare classical onconeural antibodies reacting with intracellular targets (anti-Hu, anti-Ta/Ma2, anti-CV2/CRMP5) may elicit additional symptoms beside limbic encephalitis and the prognosis of such syndromes is poor.
authors.affiliation: Pécsi Tudományegyetem, Neurológiai Klinika, Pécs. zsolt.illes@aok.pte.hu
authors.forename: Zsolt
authors.initials: Z
authors.lastname: Illés
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D020363:Limbic Encephalitis / Q000175:diagnosis* / Q000378:metabolism* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D009473:Neuronal Plasticity; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000378:metabolism; D011506:Proteins / Q000378:metabolism*; D018091:Receptors, AMPA / Q000378:metabolism*; D018080:Receptors, GABA-B / Q000378:metabolism*; D016194:Receptors, N-Methyl-D-Aspartate / Q000378:metabolism*; D013569:Synapses / Q000378:metabolism
chemical_list: D047908:Intracellular Signaling Peptides and Proteins; D009419:Nerve Tissue Proteins; D011506:Proteins; D018091:Receptors, AMPA; D018080:Receptors, GABA-B; D016194:Receptors, N-Methyl-D-Aspartate; C116499:LGI1 protein, human; C104722:glutamate receptor ionotropic, AMPA 2