Efficacy of anakinera and plasmapheresis in a ۱۰-year-old boy suffering from febrile infectious related epilepsy syndrome ( FIRES )

INTRODUCTION: FIRES is a destructive convulsions status and resistant to treatment. mortality > ۳۰% and it leads to worsening of the prognosis in cognition and motor performance in the future. This aim study evaluating the effectiveness of anakinera and plasmapheresis in treatment a ۱۰-years boy with FIRESCASE PRESENTATION:The patient's primary complaint was a generalized tonic-clonic seizure with LOC.which accompanied by fever and headache since ۵ days ago,and no other clinical findings.All lab tests were normal and after receiving levetiracetam ,transferred to PICU. Due to the continuation of seizures ˒other anticonvulsant drugs were prescribed. primary brain MRI and CSF analysis were normal. Unfortunately, CSF analysis autoimmune panel was not availablel. Due to status epilepticus, was treated with pulse methylprednisolone and IVIG. Finally,after intubation, driped midazolam and thiopental . But had not reducing seizures, and serial EEG showed epileptic encephalopathy. the possible diagnosis of FIRES, Anakinera was started and ۲۴ hours later the seizures were significantly reduced. After one month, in MRI, hypersignal changes in T۲ and FLAIR were observed in the cortical and subcortical areas of the brain. After two weeks of treatment with anakinra and controled seizures, the drug was stopped and ۴۸ hours, the patient's seizures started again, after which ۵ courses of plasmapheresis were performed and the seizures decreased, and after ۳ months of hospitalization, was treated with anticonvulsant drug therapy and Rehabilitation with seizure control was discharged. ۶ months after discharge, patient has a normal MRI, the ability to speak, crawling, swallow and recognize.Discussion and conclusion: FIRES is a rare, destructive and treatment-resistant condition. In some studies, anticonvulsant and anesthetic drugs have not been effective. With a combination of anakinera and plasmapheresis, we can hope to treat patients, although we need more information on the treatment of these patients.

Keywords:

anakinera , plasmapheresis , FIRES , status epilepticus