{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T14:18:48Z","timestamp":1779891528314,"version":"3.53.1"},"reference-count":33,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2018,8,22]],"date-time":"2018-08-22T00:00:00Z","timestamp":1534896000000},"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":[[2018,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Although secondary hemophagocytic lymphohistiocytosis (<jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>) has been reported in children with critical illness of various etiologies, it has not been reported in patients with febrile infection\u2013related epilepsy syndrome (<jats:styled-content style=\"fixed-case\">FIRES<\/jats:styled-content>). We describe a series of patients with concurrent <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">FIRES<\/jats:styled-content> in an effort to establish common pathophysiologic abnormalities.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Five patients with <jats:styled-content style=\"fixed-case\">FIRES<\/jats:styled-content> who were assessed for <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content> were identified from a neurocritical care database. All were previously healthy and had extensive diagnostic testing. All had clinical deterioration with multiorgan dysfunction prompting <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content> screening 20\u201029\u00a0days after hospitalization. Markers for inflammatory dysregulation were assessed in cerebrospinal fluid (<jats:styled-content style=\"fixed-case\">CSF)<\/jats:styled-content> and serum at various time points. Outcomes were assessed 6\u00a0months after presentation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Three patients met clinical criteria for secondary <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>. Elevation of specific cytokines\/chemokines was variable. <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content> neopterin, high mobility group box 1 (<jats:styled-content style=\"fixed-case\">HMGB<\/jats:styled-content>1), and C\u2010X\u2010C motif chemokine ligand 8 (<jats:styled-content style=\"fixed-case\">CXCL<\/jats:styled-content>8) were significantly elevated in all. Interleukin\u20101\u03b2 (IL\u20101\u03b2) and IL\u201018 were not elevated in any of the samples. Treatment and outcomes were variable.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>We describe 3 patients with <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">FIRES<\/jats:styled-content>. The co\u2010occurrence of these 2 rare disorders suggests the possibility of a common immune dysregulation phenotype prolonging epileptogenesis. <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content> screening in critically ill patients with <jats:styled-content style=\"fixed-case\">FIRES<\/jats:styled-content> may yield a broader understanding of shared inflammatory processes.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.14524","type":"journal-article","created":{"date-parts":[[2018,8,22]],"date-time":"2018-08-22T16:32:23Z","timestamp":1534955543000},"page":"1753-1763","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Fueling the <scp>FIRES<\/scp>: Hemophagocytic lymphohistiocytosis in febrile infection\u2010related epilepsy syndrome"],"prefix":"10.1111","volume":"59","author":[{"given":"Raquel","family":"Farias\u2010Moeller","sequence":"first","affiliation":[{"name":"Department of Neurology Division of Pediatric Neurology Medical College of Wisconsin Children's Hospital of Wisconsin Milwaukee Wisconsin"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reghann","family":"LaFrance\u2010Corey","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Immunology Center for MS and Autoimmune Neurology Translational Neuroimmunology Lab Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Bartolini","sequence":"additional","affiliation":[{"name":"Clinical Epilepsy Section\u00a0and Division of Neuroimmunology and Neurovirology National Institute of Neurological Disorders and Stroke \u2010 National Institute of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth M.","family":"Wells","sequence":"additional","affiliation":[{"name":"Department of Neurology Children's National Health System Washington District of 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