Introduction: Febrile event-associated epilepsy syndrome (FIRES) is a serious neurological condition that affects healthy children. It leads to a super-refractory status epilepticus, with a high mortality rate, neurocognitive sequelae and/or intractable epilepsy. The mechanisms that generate it are inflammatory and autoimmune, for this reason immunomodulatory therapy is indicated.

Objectives: To describe two patients diagnosed with FIRES, in whom a positive response to treatment with tocilizumab was observed.

Material and methods: Case report.

Results: Two previously healthy male patients, 9 and 5 years old, who presented with febrile symptoms and subsequently super-refractory status epilepticus, received sedation and barbiturate coma, remaining in a paroxysm-suppression pattern for 70 and 65 days, respectively. They received multiple antiepileptic drugs in addition to immunomodulatory treatment with corticosteroids, gamma globulin and Rituximab, the second also plasmapheresis, without a positive response. Ketogenic therapy was added, in the first case suspended due to adverse effects and in the second one, with partial response. In studies carried out, infectious, metabolic and structural causes were ruled out. A search for antibodies for immune-mediated encephalitis was performed, with Anti-GAD being positive for the youngest of the patients. After treatment with Tocilizumab, they progressed with electroclinical improvement and extubation at 78 and 56 days respectively.

Conclusion: Tocilizumab is an IL6 receptor blocker that should be considered early, especially in cryptogenic FIRES that have not responded to the first line of treatment. Although more follow-up is needed to evaluate the long-term evolution of these patients, we highlight the good clinical response.

Maria Fernanda Mejia Zuñiga
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Diana Angulo Manchola
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Paulina Carullo
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Eliana Julian
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Lucas Bongiorni Del Barco
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Manuel Rivera
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Martin Carnevale
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Paulina Yañez
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Rocio Alamo
Rául Carrea Institute for Neurological Research- FLENI-
Argentina

Angeles Scheteinschnaider
Rául Carrea Institute for Neurological Research- FLENI-
Argentina