Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis
Abstract A 14‐year‐old boy presented with a prodromal respiratory infection followed by super refractory status epilepticus. A diagnosis of Febrile Infection‐Related Epilepsy Syndrome (FIRES) was made. Initial MRI study and CSF analysis were normal. He required multiple anticonvulsants owing to the...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2021-03-01
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| Series: | Epilepsia Open |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/epi4.12454 |
| _version_ | 1818675743892176896 |
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| author | Salvadeeswaran Meenakshi‐Sundaram Muthukani Sankaranarayanan Murugan Jeyaraman Chitra Ayyappan Somalinga Nagendran Karthik Suresh Pandi |
| author_facet | Salvadeeswaran Meenakshi‐Sundaram Muthukani Sankaranarayanan Murugan Jeyaraman Chitra Ayyappan Somalinga Nagendran Karthik Suresh Pandi |
| author_sort | Salvadeeswaran Meenakshi‐Sundaram |
| collection | DOAJ |
| description | Abstract A 14‐year‐old boy presented with a prodromal respiratory infection followed by super refractory status epilepticus. A diagnosis of Febrile Infection‐Related Epilepsy Syndrome (FIRES) was made. Initial MRI study and CSF analysis were normal. He required multiple anticonvulsants owing to the refractory nature of the seizures. The course of the illness was rather stormy, laced with various medical problems viz. hepatic dysfunction, sepsis, hemodynamic, and hematological abnormalities which posed several challenges in the management. Hemophagocytic lymphocytic histiocytosis (HLH) was identified as the etiology of the illness and was treated but without success. The case report highlights the several immunomodulatory strategies that were employed to treat the disease, despite which the outcome was unfavorable. |
| first_indexed | 2024-12-17T08:32:26Z |
| format | Article |
| id | doaj.art-c2e08889966649f8854911526e9c51ff |
| institution | Directory Open Access Journal |
| issn | 2470-9239 |
| language | English |
| last_indexed | 2024-12-17T08:32:26Z |
| publishDate | 2021-03-01 |
| publisher | Wiley |
| record_format | Article |
| series | Epilepsia Open |
| spelling | doaj.art-c2e08889966649f8854911526e9c51ff2022-12-21T21:56:34ZengWileyEpilepsia Open2470-92392021-03-0161222710.1002/epi4.12454Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosisSalvadeeswaran Meenakshi‐Sundaram0Muthukani Sankaranarayanan1Murugan Jeyaraman2Chitra Ayyappan3Somalinga Nagendran Karthik4Suresh Pandi5Department of Neurosciences Apollo Speciality Hospitals Madurai IndiaDepartment of Neurosciences Apollo Speciality Hospitals Madurai IndiaDepartment of Pediatrics Apollo Speciality Hospitals Madurai IndiaDepartment of Pediatrics Apollo Speciality Hospitals Madurai IndiaDepartment of Neurosciences Apollo Speciality Hospitals Madurai IndiaDepartment of Neurosciences Apollo Speciality Hospitals Madurai IndiaAbstract A 14‐year‐old boy presented with a prodromal respiratory infection followed by super refractory status epilepticus. A diagnosis of Febrile Infection‐Related Epilepsy Syndrome (FIRES) was made. Initial MRI study and CSF analysis were normal. He required multiple anticonvulsants owing to the refractory nature of the seizures. The course of the illness was rather stormy, laced with various medical problems viz. hepatic dysfunction, sepsis, hemodynamic, and hematological abnormalities which posed several challenges in the management. Hemophagocytic lymphocytic histiocytosis (HLH) was identified as the etiology of the illness and was treated but without success. The case report highlights the several immunomodulatory strategies that were employed to treat the disease, despite which the outcome was unfavorable.https://doi.org/10.1002/epi4.12454anticonvulsant polytherapyFebrile Infection‐Related Epilepsy Syndromehemophagocytic lymphocytic histiocytosisimmunomodulationsuper refractory status epilepticus |
| spellingShingle | Salvadeeswaran Meenakshi‐Sundaram Muthukani Sankaranarayanan Murugan Jeyaraman Chitra Ayyappan Somalinga Nagendran Karthik Suresh Pandi Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis Epilepsia Open anticonvulsant polytherapy Febrile Infection‐Related Epilepsy Syndrome hemophagocytic lymphocytic histiocytosis immunomodulation super refractory status epilepticus |
| title | Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis |
| title_full | Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis |
| title_fullStr | Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis |
| title_full_unstemmed | Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis |
| title_short | Super refractory status in a case of Febrile Infection‐Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis |
| title_sort | super refractory status in a case of febrile infection related epilepsy syndrome due to hemophagocytic lymphocytic histiocytosis |
| topic | anticonvulsant polytherapy Febrile Infection‐Related Epilepsy Syndrome hemophagocytic lymphocytic histiocytosis immunomodulation super refractory status epilepticus |
| url | https://doi.org/10.1002/epi4.12454 |
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