Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation
Febrile infection-related epilepsy syndrome (FIRES) is a challenging condition with unfavorable outcome in most cases. Preliminary evidence suggests that some interleukins, in particular IL-1 Receptor Antagonist (IL-1RA), could be elevated due to a functional deficiency of anti-inflammatory pathways...
| Main Authors: | , , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2022-01-01
|
| Series: | Epilepsy & Behavior Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2589986422000089 |
| _version_ | 1818011513025200128 |
|---|---|
| author | Marco Perulli Gianpaolo Cicala Ida Turrini Elisa Musto Michela Quintiliani Maria Luigia Gambardella Silvia Maria Pulitanò Sarah Bompard Susanna Staccioli Laura Carmillo Gabriele Di Sante Francesco Ria Chiara Veredice Ilaria Contaldo Domenica Battaglia |
| author_facet | Marco Perulli Gianpaolo Cicala Ida Turrini Elisa Musto Michela Quintiliani Maria Luigia Gambardella Silvia Maria Pulitanò Sarah Bompard Susanna Staccioli Laura Carmillo Gabriele Di Sante Francesco Ria Chiara Veredice Ilaria Contaldo Domenica Battaglia |
| author_sort | Marco Perulli |
| collection | DOAJ |
| description | Febrile infection-related epilepsy syndrome (FIRES) is a challenging condition with unfavorable outcome in most cases. Preliminary evidence suggests that some interleukins, in particular IL-1 Receptor Antagonist (IL-1RA), could be elevated due to a functional deficiency of anti-inflammatory pathways. Therefore, treatment strategies acting on innate immunity could represent a targeted treatment.We describe the case of an 11-year-old child with super-refractory status epilepticus (SE), lasting more than two months. After being treated aggressively with antiseizure medications, anesthetics and empiric treatment for autoimmune encephalitis without success, she responded to anakinra and ketogenic diet. Escalation of the therapy was supported by the finding of a very high serum level of IL-1RA. This immunomodulatory approach allowed to discharge the child from intensive care 48 days after the SE onset. After more than one year follow-up the patient has moderate intellectual disability but with good language skills; she is seizure free and without motor deficits.This case suggests that serum IL-1RA serum levels may help to support treatment escalation. Moreover, anakinra and ketogenic diet represent encouraging immunomodulatory strategies which deserve further studies and could potentially have a synergistic effect. Finally, structured neuropsychological testing is an important outcome measure that will help to define the effectiveness of different treatment strategies. |
| first_indexed | 2024-04-14T06:09:40Z |
| format | Article |
| id | doaj.art-aab4a949d4ee43f6bc20cacba6c3157c |
| institution | Directory Open Access Journal |
| issn | 2589-9864 |
| language | English |
| last_indexed | 2024-04-14T06:09:40Z |
| publishDate | 2022-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Epilepsy & Behavior Reports |
| spelling | doaj.art-aab4a949d4ee43f6bc20cacba6c3157c2022-12-22T02:08:25ZengElsevierEpilepsy & Behavior Reports2589-98642022-01-0118100531Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulationMarco Perulli0Gianpaolo Cicala1Ida Turrini2Elisa Musto3Michela Quintiliani4Maria Luigia Gambardella5Silvia Maria Pulitanò6Sarah Bompard7Susanna Staccioli8Laura Carmillo9Gabriele Di Sante10Francesco Ria11Chiara Veredice12Ilaria Contaldo13Domenica Battaglia14Dipartimento di Scienze della Vita e Sanità Pubblica, Università Cattolica del Sacro Cuore, Roma, Italy; Neuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Corresponding author at: Child Neurology Unit, Department of Pediatrics, Fondazione Policlinico Universitario A. Gemelli IRCCS, Catholic University of the Sacred Heart, Largo Francesco Vito, 1, 00168 Roma, Italy.Dipartimento di Scienze della Vita e Sanità Pubblica, Università Cattolica del Sacro Cuore, Roma, Italy; Neuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDipartimento di Scienze della Vita e Sanità Pubblica, Università Cattolica del Sacro Cuore, Roma, Italy; Neuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDipartimento di Scienze della Vita e Sanità Pubblica, Università Cattolica del Sacro Cuore, Roma, Italy; Neuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyNeuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyNeuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyUnità di terapia intensiva pediatrica e trauma center pediatrico, Dipartimento scienze dell’emergenza, anestesiologiche e della rianimazione, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, ItalyDipartimento di neuroriabilitazione, IRCCS Ospedale Pediatrico “Bambino Gesù”, Roma, ItalyDipartimento di neuroriabilitazione, IRCCS Ospedale Pediatrico “Bambino Gesù”, Roma, ItalyUnità di Neurologia, Ospedale V. Fazzi, Lecce, ItalySection of Human, Clinical and Forensic Anatomy, Department of Medicine, University of Perugia, Perugia, Italy; Sezione di Patologia Generale, Università Cattolica del Sacro Cuore, Rome, ItalyDipartimento di Medicina e Chirurgia Traslazionali, Universià Cattolica del S. Cuore IRCCS Policlinico Universitario A. Gemelli, Roma, ItalyDipartimento di Scienze della Vita e Sanità Pubblica, Università Cattolica del Sacro Cuore, Roma, Italy; Neuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyNeuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyDipartimento di Scienze della Vita e Sanità Pubblica, Università Cattolica del Sacro Cuore, Roma, Italy; Neuropsichiatria Infantile, Dipartimento di Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, ItalyFebrile infection-related epilepsy syndrome (FIRES) is a challenging condition with unfavorable outcome in most cases. Preliminary evidence suggests that some interleukins, in particular IL-1 Receptor Antagonist (IL-1RA), could be elevated due to a functional deficiency of anti-inflammatory pathways. Therefore, treatment strategies acting on innate immunity could represent a targeted treatment.We describe the case of an 11-year-old child with super-refractory status epilepticus (SE), lasting more than two months. After being treated aggressively with antiseizure medications, anesthetics and empiric treatment for autoimmune encephalitis without success, she responded to anakinra and ketogenic diet. Escalation of the therapy was supported by the finding of a very high serum level of IL-1RA. This immunomodulatory approach allowed to discharge the child from intensive care 48 days after the SE onset. After more than one year follow-up the patient has moderate intellectual disability but with good language skills; she is seizure free and without motor deficits.This case suggests that serum IL-1RA serum levels may help to support treatment escalation. Moreover, anakinra and ketogenic diet represent encouraging immunomodulatory strategies which deserve further studies and could potentially have a synergistic effect. Finally, structured neuropsychological testing is an important outcome measure that will help to define the effectiveness of different treatment strategies.http://www.sciencedirect.com/science/article/pii/S2589986422000089FIRESNORSEInterleukinAnakinraKetogenic diet |
| spellingShingle | Marco Perulli Gianpaolo Cicala Ida Turrini Elisa Musto Michela Quintiliani Maria Luigia Gambardella Silvia Maria Pulitanò Sarah Bompard Susanna Staccioli Laura Carmillo Gabriele Di Sante Francesco Ria Chiara Veredice Ilaria Contaldo Domenica Battaglia Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation Epilepsy & Behavior Reports FIRES NORSE Interleukin Anakinra Ketogenic diet |
| title | Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation |
| title_full | Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation |
| title_fullStr | Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation |
| title_full_unstemmed | Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation |
| title_short | Fighting autoinflammation in FIRES: The role of interleukins and early immunomodulation |
| title_sort | fighting autoinflammation in fires the role of interleukins and early immunomodulation |
| topic | FIRES NORSE Interleukin Anakinra Ketogenic diet |
| url | http://www.sciencedirect.com/science/article/pii/S2589986422000089 |
| work_keys_str_mv | AT marcoperulli fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT gianpaolocicala fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT idaturrini fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT elisamusto fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT michelaquintiliani fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT marialuigiagambardella fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT silviamariapulitano fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT sarahbompard fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT susannastaccioli fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT lauracarmillo fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT gabrieledisante fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT francescoria fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT chiaraveredice fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT ilariacontaldo fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation AT domenicabattaglia fightingautoinflammationinfirestheroleofinterleukinsandearlyimmunomodulation |