Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature
Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epilepsy syndrome which is characterized by acute onset of refractory status epilepticus following a febrile infection occurring in previously normal children. Despite the various treatment options that have been tried, exact tre...
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Hacettepe University Institute of Child Health
2017-08-01
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| Series: | The Turkish Journal of Pediatrics |
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| Online Access: | https://turkjpediatr.org/article/view/1015 |
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| author | Caner Alparslan Fulya Kamit-Can Ayşe Berna Anıl Nihal Olgaç-Dündar Dilek Çavuşoğlu Zeynep Göç |
| author_facet | Caner Alparslan Fulya Kamit-Can Ayşe Berna Anıl Nihal Olgaç-Dündar Dilek Çavuşoğlu Zeynep Göç |
| author_sort | Caner Alparslan |
| collection | DOAJ |
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Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epilepsy syndrome which is characterized by acute onset of refractory status epilepticus following a febrile infection occurring in previously normal children. Despite the various treatment options that have been tried, exact treatment strategy is still undetermined. This is the first pediatric case of FIRES from Turkey which was successfully treated with intravenous immunoglobulin (IVIG). A previously healthy 8-year-old boy was referred to our hospital with a pre-diagnosis of status epilepticus and encephalitis. He presented with acute onset of convulsions and unconsciousness following fever and malaise lasting 7 days. On physical examination Glasgow coma scale was 12, his pupils were miotic. He had cafe-au-lait spots on his body. His fundus examination, cerebrospinal fluid findings and cranial magnetic resonance imaging did not reveal any abnormality. Results of comprehensive search for metabolic, toxicological, infectious and autoimmune etiologies were all negative. Generalized slowing was seen on the electroencephalography (EEG) of the patient indicating possible encephalopathy. The patient developed convulsive status epilepticus and was intubated on day 5. His seizures were controlled by continuous infusion of midazolam, thiopental and used for 4 days. Phenytoin, levetiracetam, topiramate were used simultaneously. IVIG was administered as an immunomodulator for refractory seizures on day-9. The patient was extubated on day 11. The diagnosis was made after a comprehensive negative search for central nervous system infection, autoimmune and metabolic diseases. At follow up it was learnt that he had had only two seizures in two years. Status epilepticus did not recur. Clinicians should keep in mind FIRES which is a diagnosis of exclusion especially in refractory status epilepticus. IVIG treatment could have a benefit in these patients.
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| format | Article |
| id | doaj-art-6771d596ff63426f80056c71b059ff60 |
| institution | OA Journals |
| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 2017-08-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-6771d596ff63426f80056c71b059ff602025-08-20T02:01:53ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212017-08-0159410.24953/turkjped.2017.04.014Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literatureCaner Alparslan0Fulya Kamit-Can1Ayşe Berna Anıl2Nihal Olgaç-Dündar3Dilek Çavuşoğlu4Zeynep Göç5Department of Pediatrics, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey.Department of Pediatric Intensive Care Unit, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey.Department of Pediatric Intensive Care Unit, Katip Çelebi University, Izmir, Turkey.Department of Pediatric Neurology, Katip Çelebi University, Izmir, Turkey.Department of Pediatric Neurology, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey.Department of Pediatrics, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey. Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epilepsy syndrome which is characterized by acute onset of refractory status epilepticus following a febrile infection occurring in previously normal children. Despite the various treatment options that have been tried, exact treatment strategy is still undetermined. This is the first pediatric case of FIRES from Turkey which was successfully treated with intravenous immunoglobulin (IVIG). A previously healthy 8-year-old boy was referred to our hospital with a pre-diagnosis of status epilepticus and encephalitis. He presented with acute onset of convulsions and unconsciousness following fever and malaise lasting 7 days. On physical examination Glasgow coma scale was 12, his pupils were miotic. He had cafe-au-lait spots on his body. His fundus examination, cerebrospinal fluid findings and cranial magnetic resonance imaging did not reveal any abnormality. Results of comprehensive search for metabolic, toxicological, infectious and autoimmune etiologies were all negative. Generalized slowing was seen on the electroencephalography (EEG) of the patient indicating possible encephalopathy. The patient developed convulsive status epilepticus and was intubated on day 5. His seizures were controlled by continuous infusion of midazolam, thiopental and used for 4 days. Phenytoin, levetiracetam, topiramate were used simultaneously. IVIG was administered as an immunomodulator for refractory seizures on day-9. The patient was extubated on day 11. The diagnosis was made after a comprehensive negative search for central nervous system infection, autoimmune and metabolic diseases. At follow up it was learnt that he had had only two seizures in two years. Status epilepticus did not recur. Clinicians should keep in mind FIRES which is a diagnosis of exclusion especially in refractory status epilepticus. IVIG treatment could have a benefit in these patients. https://turkjpediatr.org/article/view/1015childepilepsyfebrileinfectionintravenous immunoglobulinsyndrome |
| spellingShingle | Caner Alparslan Fulya Kamit-Can Ayşe Berna Anıl Nihal Olgaç-Dündar Dilek Çavuşoğlu Zeynep Göç Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature The Turkish Journal of Pediatrics child epilepsy febrile infection intravenous immunoglobulin syndrome |
| title | Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature |
| title_full | Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature |
| title_fullStr | Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature |
| title_full_unstemmed | Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature |
| title_short | Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature |
| title_sort | febrile infection related epilepsy syndrome fires treated with immunomodulation in an 8 year old boy and review of the literature |
| topic | child epilepsy febrile infection intravenous immunoglobulin syndrome |
| url | https://turkjpediatr.org/article/view/1015 |
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