{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T16:06:41Z","timestamp":1791389201392,"version":"4.3.3"},"reference-count":21,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,1,15]],"date-time":"2022-01-15T00:00:00Z","timestamp":1642204800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>This study was undertaken to report clinical presentations and outcomes of CASPR2\u2010IgG\u2010associated seizures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Mayo Clinic Neuroimmunology database was queried to identify CASPR2\u2010IgG\u2010seropositive (CASPR2\u2010IgG+) patients evaluated at our institution (2009\u20132019).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 53 CASPR2\u2010IgG+ patients (titer \u2265 1:10), 20\u00a0had seizures (38%). All seizure patients were male, with median onset age of 68\u00a0years. Eighteen (90%) had seizures at initial presentation. One patient was found to have malignancy (colon adenocarcinoma). Two patients had coexisting LGI1\u2010IgG. Twelve patients had archived sera, which on titration had CASPR2\u2010IgG titers \u2265 1:100. Fifteen patients (75%) met criteria for autoimmune encephalitis. Patients most commonly presented with focal onset, nonmotor seizures with impaired awareness (<jats:italic>n<\/jats:italic>\u00a0=\u00a014, 70%). Eleven patients also had focal motor and\/or sensory seizures as one of the seizure semiologies. The majority of patients (<jats:italic>n<\/jats:italic>\u00a0=\u00a011, 55%) developed generalized tonic\u2013clonic seizures during their disease course. Seizure clusters occurred in 12 patients. In addition to seizures, patients developed cognitive disturbance (<jats:italic>n<\/jats:italic>\u00a0=\u00a016, 80%), episodic emotional lability (<jats:italic>n<\/jats:italic>\u00a0=\u00a013, 65%), paroxysmal dizziness (<jats:italic>n<\/jats:italic>\u00a0=\u00a09, 45%), episodic ataxia (<jats:italic>n<\/jats:italic>\u00a0=\u00a06, 30%), and chronic ataxia (<jats:italic>n<\/jats:italic>\u00a0=\u00a09, 45%). Only three patients (15%) had coexisting peripheral nervous system involvement. Frontotemporal or temporal ictal and\/or interictal electroencephalographic abnormalities were present among nine patients, and three had multifocal epileptiform abnormalities. Eight patients (40%) had medial temporal T2\/fluid\u2010attenuated inversion recovery hyperintensity on brain magnetic resonance imaging. Elevated cerebrospinal fluid protein and\/or lymphocytic pleocytosis was present in most cases (13\/14, 93%). Thirteen patients reached seizure freedom following initiation of antiseizure medication (ASM; <jats:italic>n<\/jats:italic>\u00a0=\u00a04) or a combination of immunotherapy and ASM (<jats:italic>n<\/jats:italic>\u00a0=\u00a09). Median duration of follow\u2010up was 25\u00a0months (range = 2\u2013136\u00a0months).<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>CASPR2\u2010IgG evaluation should be considered among older male patients with new onset focal seizures and impaired awareness often occurring in clusters with\/without features of encephalitis. Coexisting neurological manifestations, including episodic emotional lability, ataxia, and paroxysmal dizziness, also aid in the diagnosis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.17164","type":"journal-article","created":{"date-parts":[[2022,1,15]],"date-time":"2022-01-15T09:18:16Z","timestamp":1642238296000},"page":"709-722","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["CASPR2\u2010IgG\u2010associated autoimmune seizures"],"prefix":"10.1111","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8223-610X","authenticated-orcid":false,"given":"Emilio R.","family":"Garrido Sanabria","sequence":"first","affiliation":[{"name":"Department of Neurology Mayo Clinic Health System  La Crosse Wisconsin USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anza","family":"Zahid","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey","family":"Britton","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory J.","family":"Kraus","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfonso Sebastian","family":"L\u00f3pez\u2010Chiriboga","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Jacksonville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anastasia","family":"Zekeridou","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"},{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota 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J.","family":"Pittock","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"},{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6865-9045","authenticated-orcid":false,"given":"Divyanshu","family":"Dubey","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester Minnesota USA"},{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota 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