{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T15:12:49Z","timestamp":1790867569261,"version":"4.1.0"},"reference-count":48,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2013,3,28]],"date-time":"2013-03-28T00:00:00Z","timestamp":1364428800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100002337","name":"Petre Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100002337","id-type":"DOI","asserted-by":"crossref"}]},{"name":"The National Health and Medical Research Council postgraduate scholarship scheme"},{"name":"Epilepsy Research United Kingdom"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2013,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Purpose<\/jats:title><jats:p>Antibodies against neuronal surface proteins are increasingly recognized in autoimmune central nervous system (<jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>) disorders in which seizures are the main or an important feature. The disorders include antibody\u2010associated limbic encephalitis and <jats:styled-content style=\"fixed-case\"><jats:italic>N<\/jats:italic><\/jats:styled-content>\u2010methyl\u2010<jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>\u2010aspartate receptor (NMDAR)encephalitis; however, seizures of autoimmune etiology may exist beyond the spectrum of these recognized syndromes. Because these seizures are potentially treatable with immune therapy, guidelines are needed to help in their early recognition.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We describe 13 representative children seen at our tertiary institution over a period of 3.5\u00a0years with suspected autoimmune epilepsy. Autoimmune epilepsy was suspected clinically when there was any of the following: (1) recognizable syndromes such as <jats:styled-content style=\"fixed-case\">NMDAR<\/jats:styled-content> encephalitis or limbic encephalitis, (2) evidence of <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content> inflammation in cerebrospinal fluid or on magnetic resonance imaging (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>), (3) the presence of other autoimmune diseases, or (4) positive response to immunotherapy. We tested these patients for neuronal surface antibodies (voltage gated potassium channel [VGKC]\u2010complex, leucine rich glioma inactivated 1 [LGI1], contactin\u2010associated protein\u2010like 2 [CASPR2], and <jats:styled-content style=\"fixed-case\">NMDAR<\/jats:styled-content>) and <jats:styled-content style=\"fixed-case\">glutamic acid decarboxylase (GAD)<\/jats:styled-content> antibodies. We modified the <jats:italic>J Neurol Neurosurg Psychiatry<\/jats:italic>, 83, 2012, 638 guidelines that were designed to classify adults with neuronal surface antibody syndromes (<jats:styled-content style=\"fixed-case\">NSAS<\/jats:styled-content>), to be more appropriate for children with suspected autoimmune epilepsy. Using the modified guidelines, the 13 patients were classified into definite, probable, possible, unlikely, or unknown autoimmune epilepsy according to the presence of neuronal surface or <jats:styled-content style=\"fixed-case\">GAD<\/jats:styled-content> antibodies, and the response to immune therapy when given.<\/jats:p><\/jats:sec><jats:sec><jats:title>Key Findings<\/jats:title><jats:p>Of the 13 patients, 11 were females, and the mean age was 6\u00a0years (range 1\u201313 years). Three patients had classical <jats:styled-content style=\"fixed-case\">NMDAR<\/jats:styled-content> encephalitis, two had <jats:styled-content style=\"fixed-case\">VGKC<\/jats:styled-content> encephalitis, two had limbic encephalitis with negative antibodies, three had epilepsy with other autoimmune diseases (one with high titer <jats:styled-content style=\"fixed-case\">GAD<\/jats:styled-content> antibodies), two had fever\u2010induced refractory epileptic encephalopathy in school\u2010aged children (FIRES), and one epileptic encephalopathy associated with <jats:styled-content style=\"fixed-case\">VGKC<\/jats:styled-content> antibodies. Seven patients of the 13 children with suspected autoimmune epilepsy were positive for neuronal surface antibodies (<jats:styled-content style=\"fixed-case\">NMDAR<\/jats:styled-content>, n\u00a0=\u00a03; <jats:styled-content style=\"fixed-case\">VGKC<\/jats:styled-content>\u2010complex, n\u00a0=\u00a03; and <jats:styled-content style=\"fixed-case\">GAD<\/jats:styled-content>, n\u00a0=\u00a01). Immunotherapy was given to nine cases, and a positive response was more common in patients with positive neuronal surface antibodies (5\/5) compared to those with negative antibodies (2\/4). Applying the proposed guidelines, the classification of autoimmune epilepsy was definite in five, probable in one, possible in three, unlikely in two, and unknown in two patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>Neuronal surface antibodies and <jats:styled-content style=\"fixed-case\">GAD<\/jats:styled-content> antibodies are present in a proportion of children with suspected autoimmune epilepsy and may define a treatable subgroup of childhood epilepsy. The proposed guidelines can be useful in the recognition of children with seizures of autoimmune etiology.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.12142","type":"journal-article","created":{"date-parts":[[2013,3,28]],"date-time":"2013-03-28T17:16:58Z","timestamp":1364491018000},"page":"1036-1045","source":"Crossref","is-referenced-by-count":75,"title":["Autoimmune epilepsy in children: Case series and proposed guidelines for identification"],"prefix":"10.1111","volume":"54","author":[{"given":"Jehan","family":"Suleiman","sequence":"first","affiliation":[{"name":"Neuroimmunology Group Institute for Neuroscience and Muscle Research, the Children's Hospital at Westmead University of Sydney Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabienne","family":"Brilot","sequence":"additional","affiliation":[{"name":"Neuroimmunology Group Institute for Neuroscience and Muscle Research, the Children's Hospital at Westmead University of Sydney 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