{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T14:18:17Z","timestamp":1770041897944,"version":"3.49.0"},"reference-count":156,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2014,4,1]],"date-time":"2014-04-01T00:00:00Z","timestamp":1396310400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2014,4,1]],"date-time":"2014-04-01T00:00:00Z","timestamp":1396310400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,8,17]],"date-time":"2023-08-17T00:00:00Z","timestamp":1692230400000},"content-version":"vor","delay-in-days":3425,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":["neurotherapeuticsjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurotherapeutics"],"published-print":{"date-parts":[[2014,4]]},"DOI":"10.1007\/s13311-014-0265-2","type":"journal-article","created":{"date-parts":[[2014,3,17]],"date-time":"2014-03-17T22:03:12Z","timestamp":1395093792000},"page":"297-310","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":64,"title":["Mechanisms of Epileptogenesis in Pediatric Epileptic Syndromes: Rasmussen Encephalitis, Infantile Spasms, and Febrile Infection-related Epilepsy Syndrome (FIRES)"],"prefix":"10.1016","volume":"11","author":[{"given":"Carlos A.","family":"Pardo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rima","family":"Nabbout","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aristea S.","family":"Galanopoulou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1007\/s13311-014-0265-2_bib1","doi-asserted-by":"crossref","first-page":"2175","DOI":"10.1111\/j.1528-1167.2010.02657.x","article-title":"Infantile spasms: a U.S. consensus report","volume":"51","author":"Pellock","year":"2010","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib2","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/j.pediatrneurol.2011.08.010","article-title":"Genetic and biologic classification of infantile spasms","volume":"45","author":"Paciorkowski","year":"2011","journal-title":"Pediatr Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib3","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1038\/nature12439","article-title":"De novo mutations in epileptic encephalopathies","volume":"501","author":"Epi4K Consortium, Epilepsy Phenome\/Genome P","year":"2013","journal-title":"Nature"},{"key":"10.1007\/s13311-014-0265-2_bib4","doi-asserted-by":"crossref","first-page":"1751","DOI":"10.1093\/brain\/awf176","article-title":"The natural history of Rasmussen's encephalitis","volume":"125","author":"Bien","year":"2002","journal-title":"Brain"},{"issue":"Suppl.","key":"10.1007\/s13311-014-0265-2_bib5","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1111\/epi.12278","article-title":"FIRES and IHHE: Delineation of the syndromes","volume":"54","author":"Nabbout","year":"2013","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib6","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1212\/WNL.8.6.435","article-title":"Focal seizures due to chronic localized encephalitis","volume":"8","author":"Rasmussen","year":"1958","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib7","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.pediatrneurol.2004.12.002","article-title":"Rasmussen's syndrome: progressive autoimmune multi-focal encephalopathy","volume":"32","author":"Freeman","year":"2005","journal-title":"Pediatr Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib8","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1111\/j.1469-8749.1997.tb07396.x","article-title":"Rasmussen's encephalitis","volume":"39","author":"Gordon","year":"1997","journal-title":"Develop Med Child Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib9","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1111\/epi.12042","article-title":"Rasmussen encephalitis: incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins","volume":"54","author":"Bien","year":"2013","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib10","unstructured":"Lamb K, Scott W, Mensah A, et al. Incidence, prevalence and clinical outcome of Rasmussen encephalitis in children from the United Kingdom. Epilepsia 54(Suppl.3):4."},{"key":"10.1007\/s13311-014-0265-2_bib11","first-page":"511","article-title":"Rasmussen encephalitis","volume":"111","author":"Granata","year":"2012","journal-title":"Handbook Clin Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib12","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1111\/j.0013-9580.2004.33103.x","article-title":"The pathology of Rasmussen syndrome: stages of cortical involvement and neuropathological studies in 45 hemispherectomies","volume":"45","author":"Pardo","year":"2004","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib13","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1093\/brain\/awh415","article-title":"Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement","volume":"128","author":"Bien","year":"2005","journal-title":"Brain"},{"key":"10.1007\/s13311-014-0265-2_bib14","doi-asserted-by":"crossref","first-page":"1753","DOI":"10.1111\/epi.12334","article-title":"Clinical application and evaluation of the Bien diagnostic criteria for Rasmussen encephalitis","volume":"54","author":"Olson","year":"2013","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib15","unstructured":"Varadkar SM, Bien CG, Kruse CK, et al. Rasmussen encephalitis: current concepts and therapeutic advances. Lancet Neurol 2014 (in press)."},{"key":"10.1007\/s13311-014-0265-2_bib16","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1111\/j.1528-1167.2012.03565.x","article-title":"Evolution of the EEG in children with Rasmussen's syndrome","volume":"53","author":"Longaretti","year":"2012","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib17","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1111\/jon19977116","article-title":"Neuroimaging findings in Rasmussen's syndrome","volume":"7","author":"Yacubian","year":"1997","journal-title":"J Neuroimag"},{"key":"10.1007\/s13311-014-0265-2_bib18","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1212\/WNL.58.2.250","article-title":"Diagnosis and staging of Rasmussen's encephalitis by serial MRI and histopathology","volume":"58","author":"Bien","year":"2002","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib19","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1016\/S0887-8994(02)00437-X","article-title":"A longitudinal MRI study in children with Rasmussen syndrome","volume":"27","author":"Kim","year":"2002","journal-title":"Pediatr Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib20","doi-asserted-by":"crossref","first-page":"2106","DOI":"10.1212\/01.WNL.0000128044.94294.87","article-title":"An open study of tacrolimus therapy in Rasmussen encephalitis","volume":"62","author":"Bien","year":"2004","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib21","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1111\/j.1528-1167.2011.03396.x","article-title":"Automated 3D MRI volumetry reveals regional atrophy differences in Rasmussen encephalitis","volume":"53","author":"Wagner","year":"2012","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib22","first-page":"1291","article-title":"18F-fluorodeoxyglucose positron emission tomography and MR imaging findings in Rasmussen encephalitis","volume":"22","author":"Fiorella","year":"2001","journal-title":"AJNR Am J Neuroradiol"},{"key":"10.1007\/s13311-014-0265-2_bib23","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1007\/BF00296786","article-title":"Neuropathologic findings in cortical resections (including hemispherectomies) performed for the treatment of intractable childhood epilepsy","volume":"83","author":"Farrell","year":"1992","journal-title":"Acta Neuropathol"},{"key":"10.1007\/s13311-014-0265-2_bib24","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1007\/BF00309624","article-title":"Chronic encephalitis associated with epilepsy: immunohistochemical and ultrastructural studies","volume":"89","author":"Farrell","year":"1995","journal-title":"Acta Neuropathol"},{"key":"10.1007\/s13311-014-0265-2_bib25","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1002\/ana.10100","article-title":"Destruction of neurons by cytotoxic T cells: a new pathogenic mechanism in Rasmussen's encephalitis","volume":"51","author":"Bien","year":"2002","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib26","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1186\/1742-2094-10-152","article-title":"Inflammasome induction in Rasmussen's encephalitis: cortical and associated white matter pathogenesis","volume":"10","author":"Ramaswamy","year":"2013","journal-title":"J Neuroinflammation"},{"key":"10.1007\/s13311-014-0265-2_bib27","doi-asserted-by":"crossref","first-page":"1236","DOI":"10.1093\/brain\/awp003","article-title":"CD8+ T-cell clones dominate brain infiltrates in Rasmussen encephalitis and persist in the periphery","volume":"132","author":"Schwab","year":"2009","journal-title":"Brain"},{"key":"10.1007\/s13311-014-0265-2_bib28","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1186\/1742-2094-10-56","article-title":"Differential expression of interferon-gamma and chemokine genes distinguishes Rasmussen encephalitis from cortical dysplasia and provides evidence for an early Th1 immune response","volume":"10","author":"Owens","year":"2013","journal-title":"J Neuroinflammation"},{"key":"10.1007\/s13311-014-0265-2_bib29","doi-asserted-by":"crossref","first-page":"835","DOI":"10.1586\/14737175.3.6.835","article-title":"Rasmussen's encephalitis: update on pathogenesis and treatment","volume":"3","author":"Bernasconi","year":"2003","journal-title":"Exp Rev Neurother"},{"key":"10.1007\/s13311-014-0265-2_bib30","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.eplepsyres.2009.06.001","article-title":"Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma","volume":"86","author":"Bien","year":"2009","journal-title":"Epilepsy Res"},{"key":"10.1007\/s13311-014-0265-2_bib31","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1111\/j.1528-1157.1993.tb00441.x","article-title":"Progressive unilateral encephalopathy of childhood (Rasmussen's syndrome): a reappraisal","volume":"34","author":"Vining","year":"1993","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib32","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1111\/j.0013-9580.2004.15303.x","article-title":"The cognitive outcome of hemispherectomy in 71 children","volume":"45","author":"Pulsifer","year":"2004","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib33","doi-asserted-by":"crossref","first-page":"903","DOI":"10.3171\/jns.1996.84.6.0903","article-title":"Hemispherectomy: a hemidecortication approach and review of 52 cases","volume":"84","author":"Carson","year":"1996","journal-title":"J Neurosurg"},{"key":"10.1007\/s13311-014-0265-2_bib34","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1212\/WNL.0b013e31829c5ceb","article-title":"Rasmussen encephalitis treated with natalizumab","volume":"81","author":"Bittner","year":"2013","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib35","doi-asserted-by":"crossref","first-page":"1460","DOI":"10.1177\/0883073812448838","article-title":"Autoimmune encephalitis in children","volume":"27","author":"Armangue","year":"2012","journal-title":"J Child Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib36","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1126\/science.8036512","article-title":"Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis","volume":"265","author":"Rogers","year":"1994","journal-title":"Science"},{"key":"10.1007\/s13311-014-0265-2_bib37","doi-asserted-by":"crossref","first-page":"1511","DOI":"10.1212\/WNL.57.8.1511","article-title":"GluR3 antibodies: prevalence in focal epilepsy but no specificity for Rasmussen's encephalitis","volume":"57","author":"Wiendl","year":"2001","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib38","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/S0896-6273(00)00118-5","article-title":"Autoimmunity to munc-18 in Rasmussen's encephalitis","volume":"28","author":"Yang","year":"2000","journal-title":"Neuron"},{"key":"10.1007\/s13311-014-0265-2_bib39","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1097\/WCO.0b013e32833735fe","article-title":"The growing recognition of immunotherapy-responsive seizure disorders with autoantibodies to specific neuronal proteins","volume":"23","author":"Vincent","year":"2010","journal-title":"Curr Opin Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib40","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1016\/j.seizure.2010.11.013","article-title":"Anti-NMDA receptor encephalitis presenting with imaging findings and clinical features mimicking Rasmussen syndrome","volume":"20","author":"Greiner","year":"2011","journal-title":"Seizure"},{"key":"10.1007\/s13311-014-0265-2_bib41","doi-asserted-by":"crossref","first-page":"1807","DOI":"10.1212\/01.WNL.0000099074.04539.E0","article-title":"Experience with immunomodulatory treatments in Rasmussen's encephalitis","volume":"61","author":"Granata","year":"2003","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib42","unstructured":"Laxer KD, Wilfong A, Morris III G, Andermann F. Pilot study of Rituximab to treat chronic focal encepahlitis. American Epilepsy Society Annual Conference 2008, Abst 1.277."},{"key":"10.1007\/s13311-014-0265-2_bib43","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1038\/nrneurol.2009.98","article-title":"A case of Rasmussen encephalitis treated with rituximab","volume":"5","author":"Thilo","year":"2009","journal-title":"Nat Rev Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib44","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1146\/annurev.immunol.021908.132528","article-title":"Microglial physiology: unique stimuli, specialized responses","volume":"27","author":"Ransohoff","year":"2009","journal-title":"Ann Rev Immunol"},{"key":"10.1007\/s13311-014-0265-2_bib45","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/B978-0-444-52898-8.00010-0","article-title":"Inflammation and epilepsy","volume":"107","author":"Vezzani","year":"2012","journal-title":"Handbook Clin Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib46","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1016\/j.bbi.2008.03.009","article-title":"The role of cytokines in the pathophysiology of epilepsy","volume":"22","author":"Vezzani","year":"2008","journal-title":"Brain Behav Immun"},{"key":"10.1007\/s13311-014-0265-2_bib47","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1016\/j.neuron.2012.03.026","article-title":"Microglia sculpt postnatal neural circuits in an activity and complement-dependent manner","volume":"74","author":"Schafer","year":"2012","journal-title":"Neuron"},{"key":"10.1007\/s13311-014-0265-2_bib48","doi-asserted-by":"crossref","first-page":"1391","DOI":"10.1126\/science.1212112","article-title":"Neuroscience. How many cell types does it take to wire a brain?","volume":"333","author":"Ransohoff","year":"2011","journal-title":"Science"},{"key":"10.1007\/s13311-014-0265-2_bib49","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1002\/ana.23795","article-title":"Detection of human papillomavirus in human focal cortical dysplasia type IIB","volume":"72","author":"Chen","year":"2012","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib50","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1172\/JCI58644","article-title":"Innate immunity in the central nervous system","volume":"122","author":"Ransohoff","year":"2012","journal-title":"J Clin Invest"},{"key":"10.1007\/s13311-014-0265-2_bib51","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1002\/ana.21148","article-title":"Astrocytes are a specific immunological target in Rasmussen's encephalitis","volume":"62","author":"Bauer","year":"2007","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib52","doi-asserted-by":"crossref","first-page":"193","DOI":"10.2174\/1566524043479185","article-title":"Astrocyte influences on ischemic neuronal death","volume":"4","author":"Swanson","year":"2004","journal-title":"Curr Mol Med"},{"key":"10.1007\/s13311-014-0265-2_bib53","first-page":"1","article-title":"Further observations on the syndrome of chronic encephalitis and epilepsy","volume":"41","author":"Rasmussen","year":"1978","journal-title":"Appl Neurophysiol"},{"key":"10.1007\/s13311-014-0265-2_bib54","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/S0140-6736(89)91292-0","article-title":"Epstein-Barr virus in brain and Rasmussen's encephalitis","volume":"1","author":"Walter","year":"1989","journal-title":"Lancet"},{"key":"10.1007\/s13311-014-0265-2_bib55","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1016\/0140-6736(90)92965-K","article-title":"Cytomegalovirus and Rasmussen's encephalitis","volume":"336","author":"Power","year":"1990","journal-title":"Lancet"},{"key":"10.1007\/s13311-014-0265-2_bib56","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1016\/0046-8177(93)90137-6","article-title":"Herpesviruses in chronic encephalitis associated with intractable childhood epilepsy","volume":"24","author":"Vinters","year":"1993","journal-title":"Human Pathol"},{"key":"10.1007\/s13311-014-0265-2_bib57","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1212\/WNL.45.1.108","article-title":"Chronic encephalitis and epilepsy (Rasmussen's encephalitis): detection of cytomegalovirus and herpes simplex virus 1 by the polymerase chain reaction and in situ hybridization","volume":"45","author":"Jay","year":"1995","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib58","first-page":"7","article-title":"Rasmussen's syndrome: a study of potential viral etiology","volume":"14","author":"Atkins","year":"1995","journal-title":"Clin Neuropathol"},{"key":"10.1007\/s13311-014-0265-2_bib59","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1111\/j.1528-1167.2007.01411.x","article-title":"Rasmussen encephalitis associated with SCN 1 A mutation","volume":"49","author":"Ohmori","year":"2008","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib60","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.eplepsyres.2008.03.007","article-title":"Autoantibodies to Munc18, cerebral plasma cells and B-lymphocytes in Rasmussen encephalitis","volume":"80","author":"Alvarez-Baron","year":"2008","journal-title":"Epilepsy Res"},{"key":"10.1007\/s13311-014-0265-2_bib61","doi-asserted-by":"crossref","first-page":"1159","DOI":"10.1212\/WNL.0b013e3181f4d7bf","article-title":"Clinical spectrum of early-onset epileptic encephalopathies associated with STXBP1 mutations","volume":"75","author":"Deprez","year":"2010","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib62","doi-asserted-by":"crossref","first-page":"e74","DOI":"10.1111\/epi.12124","article-title":"Reduction of seizure frequency after epilepsy surgery in a patient with STXBP1 encephalopathy and clinical description of six novel mutation carriers","volume":"54","author":"Weckhuysen","year":"2013","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib63","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1212\/WNL.50.3.731","article-title":"Double pathology in Rasmussen's syndrome: a window on the etiology?","volume":"50","author":"Hart","year":"1998","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib64","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1111\/j.1440-1789.2009.01079.x","article-title":"Dual pathology in Rasmussen's encephalitis: a study of seven cases and review of the literature","volume":"30","author":"Takei","year":"2010","journal-title":"Neuropathology"},{"key":"10.1007\/s13311-014-0265-2_bib65","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1684\/epd.2013.0555","article-title":"Clinico-pathological investigations of Rasmussen encephalitis suggest multifocal disease progression and associated focal cortical dysplasia","volume":"15","author":"Wang","year":"2013","journal-title":"Epileptic Disord"},{"key":"10.1007\/s13311-014-0265-2_bib66","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1542\/peds.100.2.163","article-title":"Why would you remove half a brain? The outcome of 58 children after hemispherectomy-the Johns Hopkins experience: 1968 to 1996","volume":"100","author":"Vining","year":"1997","journal-title":"Pediatrics"},{"key":"10.1007\/s13311-014-0265-2_bib67","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1016\/S0387-7604(01)00268-6","article-title":"What is West syndrome?","volume":"23","author":"Dulac","year":"2001","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib68","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/978-1-4615-1335-3_5","article-title":"West's syndrome (infantile spasms). Clinical description and diagnosis","volume":"497","author":"Hrachovy","year":"2002","journal-title":"Adv Exp Med Biol"},{"key":"10.1007\/s13311-014-0265-2_bib69","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1016\/S0140-6736(00)40184-4","article-title":"On a peculiar form of infantile convulsions","volume":"35","author":"West","year":"1841","journal-title":"Lancet"},{"key":"10.1007\/s13311-014-0265-2_bib70","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1212\/01.WNL.0000127773.72699.C8","article-title":"Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurology and the Child Neurology Society","volume":"62","author":"Mackay","year":"2004","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib71","doi-asserted-by":"crossref","first-page":"1974","DOI":"10.1212\/WNL.0b013e318259e2cf","article-title":"Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society","volume":"78","author":"Go","year":"2012","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib72","doi-asserted-by":"crossref","first-page":"1289","DOI":"10.1177\/0883073812455692","article-title":"The current evaluation and treatment of infantile spasms among members of the Child Neurology Society","volume":"27","author":"Mytinger","year":"2012","journal-title":"J Child Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib73","doi-asserted-by":"crossref","first-page":"2168","DOI":"10.1111\/j.1528-1167.2010.02695.x","article-title":"The underlying etiology of infantile spasms (West syndrome): information from the United Kingdom Infantile Spasms Study (UKISS) on contemporary causes and their classification","volume":"51","author":"Osborne","year":"2010","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib74","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1002\/ana.22645","article-title":"Rare copy number variants are an important cause of epileptic encephalopathies","volume":"70","author":"Mefford","year":"2011","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib75","first-page":"4","article-title":"Mutations in ARX result in several defects involving GABAergic neurons","volume":"4","author":"Friocourt","year":"2010","journal-title":"Front Cell Neurosci"},{"key":"10.1007\/s13311-014-0265-2_bib76","doi-asserted-by":"crossref","first-page":"1484","DOI":"10.1093\/cercor\/bhs138","article-title":"An epilepsy-related ARX polyalanine expansion modifies glutamatergic neurons excitability and morphology without affecting GABAergic neurons development","volume":"23","author":"Beguin","year":"2013","journal-title":"Cereb Cortex"},{"key":"10.1007\/s13311-014-0265-2_bib77","doi-asserted-by":"crossref","first-page":"859","DOI":"10.1016\/j.conb.2012.04.006","article-title":"Interneuron, interrupted: molecular pathogenesis of ARX mutations and X-linked infantile spasms","volume":"22","author":"Olivetti","year":"2012","journal-title":"Curr Opin Neurobiol"},{"key":"10.1007\/s13311-014-0265-2_bib78","doi-asserted-by":"crossref","first-page":"8752","DOI":"10.1523\/JNEUROSCI.0915-09.2009","article-title":"A triplet repeat expansion genetic mouse model of infantile spasms syndrome, Arx(GCG)10+7, with interneuronopathy, spasms in infancy, persistent seizures, and adult cognitive and behavioral impairment","volume":"29","author":"Price","year":"2009","journal-title":"J Neurosci"},{"key":"10.1007\/s13311-014-0265-2_bib79","doi-asserted-by":"crossref","first-page":"1563","DOI":"10.1093\/brain\/awp107","article-title":"Targeted loss of Arx results in a developmental epilepsy mouse model and recapitulates the human phenotype in heterozygous females","volume":"132","author":"Marsh","year":"2009","journal-title":"Brain"},{"key":"10.1007\/s13311-014-0265-2_bib80","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1016\/j.braindev.2012.12.005","article-title":"Basic mechanisms of catastrophic epilepsy\u2014overview from animal models","volume":"35","author":"Galanopoulou","year":"2013","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib81","doi-asserted-by":"crossref","first-page":"18332","DOI":"10.1073\/pnas.0608507103","article-title":"Munc18-1 expression levels control synapse recovery by regulating readily releasable pool size","volume":"103","author":"Toonen","year":"2006","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1007\/s13311-014-0265-2_bib82","doi-asserted-by":"crossref","first-page":"1580","DOI":"10.1046\/j.1528-1157.2001.13501.x","article-title":"Infantile spasms in Down syndrome: good response to a short course of vigabatrin","volume":"42","author":"Nabbout","year":"2001","journal-title":"Epilepsia"},{"issue":"Suppl. 1","key":"10.1007\/s13311-014-0265-2_bib83","first-page":"213","article-title":"Infantile spasms in children with Down syndrome","volume":"35","author":"Lujic","year":"2011","journal-title":"Coll Antropol"},{"key":"10.1007\/s13311-014-0265-2_bib84","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/j.pediatrneurol.2012.09.006","article-title":"Treatment outcomes of West syndrome in infants with Down syndrome","volume":"48","author":"Sanmaneechai","year":"2013","journal-title":"Pediatr Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib85","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/S0387-7604(01)00239-X","article-title":"Seizure frequency and characteristics in children with Down syndrome","volume":"23","author":"Goldberg-Stern","year":"2001","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib86","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1203\/PDR.0b013e31819d9076","article-title":"Infantile spasms and Down syndrome: a new animal model","volume":"65","author":"Cortez","year":"2009","journal-title":"Pediatr Res"},{"key":"10.1007\/s13311-014-0265-2_bib87","doi-asserted-by":"crossref","first-page":"1236","DOI":"10.1111\/j.1528-1167.2009.02474.x","article-title":"The natural history of epilepsy in tuberous sclerosis complex","volume":"51","author":"Chu-Shore","year":"2010","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib88","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1111\/j.1528-1157.1995.tb02569.x","article-title":"Epidemiology of infantile spasms in Sweden","volume":"36","author":"Sidenvall","year":"1995","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib89","doi-asserted-by":"crossref","first-page":"502","DOI":"10.1016\/S0387-7604(01)00300-X","article-title":"Infantile spasms in tuberous sclerosis complex","volume":"23","author":"Curatolo","year":"2001","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib90","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1016\/S0387-7604(01)00263-7","article-title":"Epidemiological data of West syndrome in Finland","volume":"23","author":"Riikonen","year":"2001","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib91","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/j.seizure.2008.09.006","article-title":"A retrospective study on aetiology based outcome of infantile spasms","volume":"18","author":"Karvelas","year":"2009","journal-title":"Seizure"},{"key":"10.1007\/s13311-014-0265-2_bib92","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.ejpn.2009.03.003","article-title":"Early control of seizures improves long-term outcome in children with tuberous sclerosis complex","volume":"14","author":"Bombardieri","year":"2010","journal-title":"Eur J Paediatr Neurol"},{"issue":"Suppl. 10","key":"10.1007\/s13311-014-0265-2_bib93","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1111\/j.1528-1167.2005.00353.x","article-title":"Neurocutaneous syndromes and epilepsy-issues in diagnosis and management","volume":"46","author":"Cross","year":"2005","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib94","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.neuron.2012.03.010","article-title":"Somatic activation of AKT3 causes hemispheric developmental brain malformations","volume":"74","author":"Poduri","year":"2012","journal-title":"Neuron"},{"key":"10.1007\/s13311-014-0265-2_bib95","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1097\/WCO.0b013e32835ef373","article-title":"Hemimegalencephaly, a paradigm for somatic postzygotic neurodevelopmental disorders","volume":"26","author":"Baek","year":"2013","journal-title":"Curr Opin Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib96","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1038\/ng.2329","article-title":"De novo somatic mutations in components of the PI3K-AKT3-mTOR pathway cause hemimegalencephaly","volume":"44","author":"Lee","year":"2012","journal-title":"Nat Genet"},{"key":"10.1007\/s13311-014-0265-2_bib97","doi-asserted-by":"crossref","first-page":"1591","DOI":"10.1172\/JCI41592","article-title":"STRADalpha deficiency results in aberrant mTORC1 signaling during corticogenesis in humans and mice","volume":"120","author":"Orlova","year":"2010","journal-title":"J Clin Invest"},{"key":"10.1007\/s13311-014-0265-2_bib98","first-page":"182ra53","article-title":"Rapamycin prevents seizures after depletion of STRADA in a rare neurodevelopmental disorder","volume":"5","author":"Parker","year":"2013","journal-title":"Sc Transl Med"},{"key":"10.1007\/s13311-014-0265-2_bib99","doi-asserted-by":"crossref","first-page":"1929","DOI":"10.1093\/brain\/awm100","article-title":"Polyhydramnios, megalencephaly and symptomatic epilepsy caused by a homozygous 7-kilobase deletion in LYK5","volume":"130","author":"Puffenberger","year":"2007","journal-title":"Brain"},{"key":"10.1007\/s13311-014-0265-2_bib100","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1002\/ana.10251","article-title":"Focal cortical dysplasia of Taylor's balloon cell type: mutational analysis of the TSC1 gene indicates a pathogenic relationship to tuberous sclerosis","volume":"52","author":"Becker","year":"2002","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib101","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1002\/ana.20211","article-title":"mTOR cascade activation distinguishes tubers from focal cortical dysplasia","volume":"56","author":"Baybis","year":"2004","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib102","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1002\/ana.20234","article-title":"Insulin signaling pathways in cortical dysplasia and TSC-tubers: tissue microarray analysis","volume":"56","author":"Miyata","year":"2004","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib103","doi-asserted-by":"crossref","first-page":"35664","DOI":"10.1074\/jbc.M403385200","article-title":"Human papillomavirus 16 E6 oncoprotein interferences with insulin signaling pathway by binding to tuberin","volume":"279","author":"Lu","year":"2004","journal-title":"J Biol Chem"},{"key":"10.1007\/s13311-014-0265-2_bib104","doi-asserted-by":"crossref","first-page":"9398","DOI":"10.1128\/JVI.00974-10","article-title":"The human papillomavirus type 16 E6 oncoprotein activates mTORC1 signaling and increases protein synthesis","volume":"84","author":"Spangle","year":"2010","journal-title":"J Virol"},{"key":"10.1007\/s13311-014-0265-2_bib105","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1016\/j.nbd.2011.03.021","article-title":"A pulse rapamycin therapy for infantile spasms and associated cognitive decline","volume":"43","author":"Raffo","year":"2011","journal-title":"Neurobiol Dis"},{"key":"10.1007\/s13311-014-0265-2_bib106","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1016\/j.braindev.2009.11.001","article-title":"Peripheral lymphocyte subset and serum cytokine profiles of patients with West syndrome","volume":"32","author":"Shiihara","year":"2010","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib107","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1016\/j.eplepsyres.2009.03.013","article-title":"Reduced levels of interleukin-1 receptor antagonist in the cerebrospinal fluid in patients with West syndrome","volume":"85","author":"Haginoya","year":"2009","journal-title":"Epilepsy Res"},{"key":"10.1007\/s13311-014-0265-2_bib108","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1016\/j.jns.2010.07.018","article-title":"Increased level of serum interleukin-1 receptor antagonist subsequent to resolution of clinical symptoms in patients with West syndrome","volume":"298","author":"Yamanaka","year":"2010","journal-title":"J Neurol Sci"},{"key":"10.1007\/s13311-014-0265-2_bib109","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1016\/S0387-7604(01)00313-8","article-title":"Serum cytokine levels are altered in patients with West syndrome","volume":"23","author":"Liu","year":"2001","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib110","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.braindev.2005.03.007","article-title":"Cerebrospinal fluid interleukin-6 levels in patients with West syndrome","volume":"28","author":"Tekgul","year":"2006","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib111","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1016\/j.nbd.2009.11.011","article-title":"A model of symptomatic infantile spasms syndrome","volume":"37","author":"Scantlebury","year":"2010","journal-title":"Neurobiol Dis"},{"key":"10.1007\/s13311-014-0265-2_bib112","first-page":"300","article-title":"Intracortical injection of lipopolysaccharide induces epileptic spasms in neonatal rats: another animal model of symptomatic infantile spasms","volume":"12","author":"Ono","year":"2012","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib113","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1055\/s-2008-1071556","article-title":"Infantile spasms: infectious disorders","volume":"24","author":"Riikonen","year":"1993","journal-title":"Neuropediatrics"},{"key":"10.1007\/s13311-014-0265-2_bib114","first-page":"502","article-title":"A case with infantile spasms due to herpes simplex type 1 virus encephalitis","volume":"19","author":"Ohtaki","year":"1987","journal-title":"No To Hattatsu"},{"key":"10.1007\/s13311-014-0265-2_bib115","first-page":"849812","article-title":"Chronic granulomatous herpes encephalitis in a child with clinically intractable epilepsy","volume":"2012","author":"Hackney","year":"2012","journal-title":"Case Rep Pediatr"},{"key":"10.1007\/s13311-014-0265-2_bib116","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1016\/S0387-7604(01)00278-9","article-title":"Spontaneous remission of spasms in West syndrome\u2014implications of viral infection","volume":"23","author":"Hattori","year":"2001","journal-title":"Brain Dev"},{"key":"10.1007\/s13311-014-0265-2_bib117","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1046\/j.1328-8067.2001.01511.x","article-title":"Decreased frequency of seizures in infantile spasms associated with lissencephaly by human herpes virus 7 infection","volume":"44","author":"Ono","year":"2002","journal-title":"Pediatr Int"},{"key":"10.1007\/s13311-014-0265-2_bib118","doi-asserted-by":"crossref","first-page":"e1045","DOI":"10.1542\/peds.2005-1867","article-title":"Herpes simplex virus central nervous system relapse during treatment of infantile spasms with corticotropin","volume":"117","author":"Bonkowsky","year":"2006","journal-title":"Pediatrics"},{"key":"10.1007\/s13311-014-0265-2_bib119","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1590\/S0004-282X1984000200004","article-title":"Demonstration of antibody and cellular immune response to brain extract in West and Lennox-Gastaut syndromes","volume":"42","author":"Mota","year":"1984","journal-title":"Arq Neuropsiquiatr"},{"key":"10.1007\/s13311-014-0265-2_bib120","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1111\/j.1469-8749.2011.04096.x","article-title":"Immune-mediated steroid-responsive epileptic spasms and epileptic encephalopathy associated with VGKC-complex antibodies","volume":"53","author":"Suleiman","year":"2011","journal-title":"Dev Med Child Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib121","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1016\/j.yebeh.2012.05.021","article-title":"Electroencephalographic and seizure manifestations in two patients with folate receptor autoimmune antibody-mediated primary cerebral folate deficiency","volume":"24","author":"Steele","year":"2012","journal-title":"Epilepsy Behav"},{"key":"10.1007\/s13311-014-0265-2_bib122","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1097\/01.WNP.0000149893.12678.44","article-title":"Pathogenesis of infantile spasms: a model based on developmental desynchronization","volume":"22","author":"Frost","year":"2005","journal-title":"J Clin Neurophysiol"},{"key":"10.1007\/s13311-014-0265-2_bib123","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1111\/j.1528-1167.2007.01377.x","article-title":"A new animal model of infantile spasms with unprovoked persistent seizures","volume":"49","author":"Lee","year":"2008","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib124","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/0165-3806(92)90178-Y","article-title":"N-methyl-D-aspartate (NMDA)-induced seizures in developing rats","volume":"65","author":"Mares","year":"1992","journal-title":"Brain Res Develop Brain Res"},{"key":"10.1007\/s13311-014-0265-2_bib125","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1111\/j.1528-1157.1999.tb02006.x","article-title":"Age-specific N-methyl-D-aspartate-induced seizures: perspectives for the West syndrome model","volume":"40","author":"Kabova","year":"1999","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib126","first-page":"115","article-title":"West syndrome model: seek and you will find","volume":"98","author":"Kabova","year":"1997","journal-title":"Sb Lek"},{"key":"10.1007\/s13311-014-0265-2_bib127","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/j.eplepsyres.2012.02.001","article-title":"Effects of adrenal dysfunction and high-dose adrenocorticotropic hormone on NMDA-induced spasm seizures in young Wistar rats","volume":"100","author":"Wang","year":"2012","journal-title":"Epilepsy Res"},{"key":"10.1007\/s13311-014-0265-2_bib128","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1002\/ana.21082","article-title":"Model of infantile spasms induced by N-methyl-D-aspartic acid in prenatally impaired brain","volume":"61","author":"Velisek","year":"2007","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib129","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1002\/ana.410330302","article-title":"Pathophysiology of massive infantile spasms: perspective on the putative role of the brain adrenal axis","volume":"33","author":"Baram","year":"1993","journal-title":"Ann Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib130","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/0165-3806(91)90118-3","article-title":"Corticotropin-releasing hormone is a rapid and potent convulsant in the infant rat","volume":"61","author":"Baram","year":"1991","journal-title":"Brain Res Develop Brain Res"},{"key":"10.1007\/s13311-014-0265-2_bib131","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1111\/j.1528-1157.1995.tb00977.x","article-title":"ACTH does not control neonatal seizures induced by administration of exogenous corticotropin-releasing hormone","volume":"36","author":"Baram","year":"1995","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib132","doi-asserted-by":"crossref","first-page":"e46","DOI":"10.1111\/j.1528-1167.2011.03357.x","article-title":"Prenatal stress promotes development of spasms in infant rats","volume":"53","author":"Yum","year":"2012","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib133","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/S0074-7742(02)49010-1","article-title":"Role of subcortical structures in the pathogenesis of infantile spasms: what are possible subcortical mediators?","volume":"49","author":"Lado","year":"2002","journal-title":"Int Rev Neurobiol"},{"key":"10.1007\/s13311-014-0265-2_bib134","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1111\/epi.12424","article-title":"CPP-115, a vigabatrin analogue, suppresses spasms in the multiple-hit rat model of infantile spasms","volume":"55","author":"Briggs","year":"2014","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib135","doi-asserted-by":"crossref","first-page":"1678","DOI":"10.1111\/j.1528-1167.2011.03173.x","article-title":"Carisbamate acutely suppresses spasms in a rat model of symptomatic infantile spasms","volume":"52","author":"Ono","year":"2011","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib136","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.eplepsyres.2013.10.015","article-title":"Efficacy and tolerability of the galanin analog NAX 5055 in the multiple-hit rat model of symptomatic infantile spasms","volume":"108","author":"Jequier Gygax","year":"2014","journal-title":"Epilepsy Res"},{"key":"10.1007\/s13311-014-0265-2_bib137","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1111\/j.1528-1167.2010.02535.x","article-title":"Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood","volume":"51","author":"van Baalen","year":"2010","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib138","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.eplepsyres.2006.01.002","article-title":"Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis","volume":"69","author":"Mikaeloff","year":"2006","journal-title":"Epilepsy Res"},{"key":"10.1007\/s13311-014-0265-2_bib139","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1111\/j.1528-1167.2010.02522.x","article-title":"Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005\u20132009","volume":"51","author":"Berg","year":"2010","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib140","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/S1474-4422(10)70214-3","article-title":"Acute encephalopathy with inflammation-mediated status epilepticus","volume":"10","author":"Nabbout","year":"2011","journal-title":"Lancet Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib141","doi-asserted-by":"crossref","first-page":"1956","DOI":"10.1111\/j.1528-1167.2011.03250.x","article-title":"Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children","volume":"52","author":"Kramer","year":"2011","journal-title":"Epilepsia"},{"issue":"Suppl. 8","key":"10.1007\/s13311-014-0265-2_bib142","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1111\/j.1528-1167.2011.03230.x","article-title":"Febrile infection-related epilepsy syndrome (FIRES): does duration of anesthesia affect outcome?","volume":"52","author":"Kramer","year":"2011","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib143","doi-asserted-by":"crossref","first-page":"2033","DOI":"10.1111\/j.1528-1167.2010.02703.x","article-title":"Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES)","volume":"51","author":"Nabbout","year":"2010","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib144","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1111\/j.1469-8749.2008.03216.x","article-title":"The ketogenic diet improves recently worsened focal epilepsy","volume":"51","author":"Villeneuve","year":"2009","journal-title":"Dev Med Child Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib145","doi-asserted-by":"crossref","first-page":"40","DOI":"10.2967\/jnumed.110.077214","article-title":"18F-FDG PET reveals frontotemporal dysfunction in children with fever-induced refractory epileptic encephalopathy","volume":"52","author":"Mazzuca","year":"2011","journal-title":"J Nucl Med"},{"issue":"Suppl. 4","key":"10.1007\/s13311-014-0265-2_bib146","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1111\/j.1528-1167.2012.03618.x","article-title":"Mitochondrial diseases and epilepsy","volume":"53","author":"Bindoff","year":"2012","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib147","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1111\/j.1469-8749.2012.04435.x","article-title":"Febrile infection-related epilepsy syndrome (FIRES) is not caused by SCN1A, POLG, PCDH19 mutations or rare copy number variations","volume":"54","author":"Appenzeller","year":"2012","journal-title":"Dev Med Child Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib148","doi-asserted-by":"crossref","first-page":"e1000381","DOI":"10.1371\/journal.pgen.1000381","article-title":"Sporadic infantile epileptic encephalopathy caused by mutations in PCDH19 resembles Dravet syndrome but mainly affects females","volume":"5","author":"Depienne","year":"2009","journal-title":"PLoS Genet"},{"key":"10.1007\/s13311-014-0265-2_bib149","doi-asserted-by":"crossref","first-page":"1255","DOI":"10.1038\/ng.2441","article-title":"De novo gain-of-function KCNT1 channel mutations cause malignant migrating partial seizures of infancy","volume":"44","author":"Barcia","year":"2012","journal-title":"Nat Genet"},{"key":"10.1007\/s13311-014-0265-2_bib150","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1038\/ng.292","article-title":"15q13.3 microdeletions increase risk of idiopathic generalized epilepsy","volume":"41","author":"Helbig","year":"2009","journal-title":"Nat Genet"},{"key":"10.1007\/s13311-014-0265-2_bib151","first-page":"71","article-title":"Severe myoclonic epilepsy in infancy: Dravet syndrome","volume":"95","author":"Dravet","year":"2005","journal-title":"Adv Neurol"},{"key":"10.1007\/s13311-014-0265-2_bib152","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1016\/j.braindev.2009.09.017","article-title":"Epileptic encephalopathy in children possibly related to immune-mediated pathogenesis","volume":"32","author":"Specchio","year":"2010","journal-title":"Brain Develop"},{"key":"10.1007\/s13311-014-0265-2_bib153","doi-asserted-by":"crossref","first-page":"e172","DOI":"10.1111\/j.1528-1167.2011.03193.x","article-title":"Acute-onset epilepsy triggered by fever mimicking FIRES (febrile infection-related epilepsy syndrome): the role of protocadherin 19 (PCDH19) gene mutation","volume":"52","author":"Specchio","year":"2011","journal-title":"Epilepsia"},{"key":"10.1007\/s13311-014-0265-2_bib154","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1212\/WNL.0b013e318227046d","article-title":"De novo SCN1A mutations in migrating partial seizures of infancy","volume":"77","author":"Carranza Rojo","year":"2011","journal-title":"Neurology"},{"key":"10.1007\/s13311-014-0265-2_bib155","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1055\/s-0032-1323848","article-title":"Febrile infection-related epilepsy syndrome without detectable autoantibodies and response to immunotherapy: a case series and discussion of epileptogenesis in FIRES","volume":"43","author":"van Baalen","year":"2012","journal-title":"Neuropediatrics"},{"key":"10.1007\/s13311-014-0265-2_bib156","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1055\/s-0033-1358601","article-title":"Immunity activation in brain cells in epilepsy: mechanistic insights and pathological consequences","volume":"44","author":"Ravizza","year":"2013","journal-title":"Neuropediatrics"}],"container-title":["Neurotherapeutics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s13311-014-0265-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s13311-014-0265-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S187874792300898X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S187874792300898X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s13311-014-0265-2","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T06:47:38Z","timestamp":1761806858000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S187874792300898X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,4]]},"references-count":156,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2014,4]]}},"alternative-id":["S187874792300898X"],"URL":"https:\/\/doi.org\/10.1007\/s13311-014-0265-2","relation":{},"ISSN":["1878-7479"],"issn-type":[{"value":"1878-7479","type":"print"}],"subject":[],"published":{"date-parts":[[2014,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Mechanisms of Epileptogenesis in Pediatric Epileptic Syndromes: Rasmussen Encephalitis, Infantile Spasms, and Febrile Infection-related Epilepsy Syndrome (FIRES)","name":"articletitle","label":"Article Title"},{"value":"Neurotherapeutics","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1007\/s13311-014-0265-2","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2014 The American Society for Experimental NeuroTherapeutics, Inc. Published by Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}