{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T16:55:14Z","timestamp":1791824114305,"version":"4.3.4"},"reference-count":23,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,6,25]],"date-time":"2018-06-25T00:00:00Z","timestamp":1529884800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Mayo Clinic Neuroscience Focused Research Team Program"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia Open"],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To characterize seizure semiology and the utility of <jats:styled-content style=\"fixed-case\">antiepileptic drug (AED)<\/jats:styled-content> therapy in leucine\u2010rich glioma inactivated\u20101 ( <jats:styled-content style=\"fixed-case\">LGI<\/jats:styled-content>1\u2010Ab) <jats:styled-content style=\"fixed-case\">autoimmune epilepsy (AE)<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with voltage\u2010gated potassium channel complex (<jats:styled-content style=\"fixed-case\">VGKC<\/jats:styled-content>c) titers higher than 0.02\u00a0nmol\/L who were evaluated between May 2008 and June 2016 at the 3 Mayo Clinic sites (Arizona, Florida, or Minnesota) were identified. We then performed a retrospective review of those who were <jats:styled-content style=\"fixed-case\">LGI<\/jats:styled-content>1\u2010Ab positive and were treated for seizures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 1,095 patients with <jats:styled-content style=\"fixed-case\">VGKC<\/jats:styled-content>c titers higher than 0.02\u00a0nmol\/L were identified, in which 77 were <jats:styled-content style=\"fixed-case\">LGI<\/jats:styled-content>1 positive. Of these, 56 patients with seizures were included in the analysis. Mean age at symptom onset was 62.9\u00a0years; 66% (n\u00a0=\u00a037) were male. The most common seizure semiology was focal faciobrachial dystonic seizures with preserved awareness (<jats:styled-content style=\"fixed-case\">FBDS<\/jats:styled-content>) (n\u00a0=\u00a035, 63%), followed by focal with impaired awareness (<jats:styled-content style=\"fixed-case\">FIA<\/jats:styled-content>) (n\u00a0=\u00a029, 52%), generalized tonic\u2013clonic (<jats:styled-content style=\"fixed-case\">GTC<\/jats:styled-content>s) (n\u00a0=\u00a028, 50%), and focal non\u2010motor seizures with preserved awareness (n\u00a0=\u00a028, 50%). The majority had more than one seizure type (n\u00a0=\u00a049, 88%; median = 2.5). Thirty\u2010eight patients (68%) became seizure free: 29 (76%) with immunotherapy, 3 (5%) with <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s alone, 2 (3%) with <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s before any immunotherapy, and 4 (7%) with <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s after immunotherapy. Levetiracetam (n\u00a0=\u00a047, 84%) and valproic acid (n\u00a0=\u00a021, 38%) were the most commonly used <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s, but neither were associated with seizure freedom. Sodium channel blocking (<jats:styled-content style=\"fixed-case\">NCB<\/jats:styled-content>) <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s were associated with seizure freedom in 4 patients compared to none treated with non\u2010<jats:styled-content style=\"fixed-case\">NCB AED<\/jats:styled-content>s. Regardless of class, <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s prior to or apart from immunotherapy were associated with seizure freedom in only five patients (9%). In patients with <jats:styled-content style=\"fixed-case\">FBDS<\/jats:styled-content>, seizure freedom was more often associated with immunotherapy than <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s (20\/30 vs. 3\/34, p\u00a0=\u00a00.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>Although <jats:styled-content style=\"fixed-case\">FBDS<\/jats:styled-content> are the most characteristic seizure type seen in <jats:styled-content style=\"fixed-case\">LGI<\/jats:styled-content>1\u2010Ab <jats:styled-content style=\"fixed-case\">AE<\/jats:styled-content>, other seizure types including <jats:styled-content style=\"fixed-case\">FIA<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">GTC<\/jats:styled-content>s also occur. Immunotherapy was the treatment most frequently associated with seizure freedom in <jats:styled-content style=\"fixed-case\">LGI<\/jats:styled-content>1\u2010Ab <jats:styled-content style=\"fixed-case\">AE<\/jats:styled-content>. In general, <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s seemed to confer a very low chance for seizure freedom, although <jats:styled-content style=\"fixed-case\">AED<\/jats:styled-content>s with <jats:styled-content style=\"fixed-case\">NCB<\/jats:styled-content>\u2010blocking properties were associated with seizure freedom in a limited number. Levetiracetam in particular appears to be ineffective in this patient population.<\/jats:p><\/jats:sec>","DOI":"10.1002\/epi4.12226","type":"journal-article","created":{"date-parts":[[2018,5,10]],"date-time":"2018-05-10T16:25:40Z","timestamp":1525969540000},"page":"348-356","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["Antiepileptic drug therapy in autoimmune epilepsy associated with antibodies targeting the leucine\u2010rich glioma\u2010inactivated protein 1"],"prefix":"10.1002","volume":"3","author":[{"given":"Anteneh M.","family":"Feyissa","sequence":"first","affiliation":[{"name":"Department of Neurology Mayo Clinic Jacksonville Florida U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"Lamb","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Jacksonville Florida U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean J.","family":"Pittock","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota U.S.A."},{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Avi","family":"Gadoth","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota U.S.A."},{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"McKeon","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota U.S.A."},{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher J.","family":"Klein","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota U.S.A."},{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota U.S.A."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey W.","family":"Britton","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic Rochester Minnesota 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