title: [Neuroimmunological diseases associated with VGKC complex antibodies].
abstract: Antibodies to voltage-gated potassium channels(VGKC) were first identified by radioimmunoassay of radioisotope labeled alpha-dendrotoxin-VGKCs solubilized from rabbit brain. These antibodies were found only in a proportion of patients with acquired neuromyotonia (Isaacs' syndrome). VGKC antibodies were also detected in Morvan's syndrome and in a form of autoimmune limbic encephalitis. Recent studies indicated that the "VGKC" antibodies are mainly directed toward associated proteins(for example LGI-1, Caspr-2) that complex with the VGKCs themselves. The "VGKC" antibodies are now usually known as VGKC-complex antibodies. In general, LGI-1 antibodies are most common in limbic encephalitis with SIADH. Caspr-2 antibodies are present in the majority of patients with Morvan's syndrome. These patients develop combinations of CNS symptoms, autonomic dysfunction, and peripheral nerve hyperexcitability.
authors.affiliation: Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences.
authors.forename: Osamu
authors.initials: O
authors.lastname: Watanabe
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis / Q000276:immunology*; D000072481:DNA Ligase ATP; D011088:DNA Ligases / Q000276:immunology; D006801:Humans; D008565:Membrane Proteins / Q000276:immunology; D009419:Nerve Tissue Proteins / Q000276:immunology; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D013595:Syringomyelia / Q000175:diagnosis / Q000276:immunology
chemical_list: D001323:Autoantibodies; C403049:CNTNAP2 protein, human; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D024642:Potassium Channels, Voltage-Gated; D011088:DNA Ligases; D000072481:DNA Ligase ATP