Dados do Trabalho

Título

Febrile Infection-Related Epilepsy Syndrome (FIRES) as a cause of super-refractory status epilepticus: a case report

Apresentação do caso

A previously healthy 14-year-old female was brought to the emergency department with focal to bilateral tonic-clonic seizures that started abruptly at the day of admission. There was a report of odynophagia, headache and fever over the past five days. She persisted with recurrent seizures despite first-line treatment, as well as continuous infusion of sedatives. After therapeutic optimization, including the administration of six classes of antiepileptic drugs, the patient maintained recurrence of seizures. The electroencephalogram (EEG) showed a severe disturbance of the background activity, with a burst-suppression pattern. After a through clinical and laboratory evaluation, the hypothesis of an autoimmune encephalitis was raised, and the patient was started on immunosuppressive steroid therapy (methylprednisolone). The clinical response was satisfactory, however, after the third day of therapy, the infusion was interrupted due to a severe respiratory infection. Following the interruption of therapy, a deterioration of the EEG pattern was observed, as well as clinical recurrence of focal seizures, requiring an increase of the intravenous sedatives. Given the respiratory infection, intravenous immunoglobulin was administered (total dose of 2g/Kg). The patient remained without recurrence of clinical seizures, but EEG worsening was maintained. Therefore, barbiturate coma with thiopental was started, with no satisfactory response for 48 hours. After adequate treatment of the infection, a second immunosuppressive steroid therapy was started (methylprednisolone 1g/day for five days). The patient, then, presented clinical control of seizures and improvement of the electroencephalographic pattern, with gradual reduction of sedatives.

Discussão

Status epilepticus in previously healthy individuals is known as new-onset refractory status epilepticus (NORSE). When it affects young patients with a history of febrile infection preceding the seizure by two weeks to twenty-four hours, the condition is known as febrile infection-related epilepsy syndrome (FIRES). It is a rare condition, with an estimated incidence of one per million. The response to antiepileptic drugs is usually unsatisfactory, with refractoriness to the use of anesthetics and barbiturate coma.

Comentários finais

The case presented demonstrates the difficulty of clinical management of FIRES, due to the high refractoriness to standardized treatments for status epilepticus.