نبذة مختصرة : Introduction: Super-refractory status epilepticus (SRSE) is a pathology that affects the neuronal environment depending on the types of seizure and their duration.Case presentation. This paper presents the case of a 7-year old child presenting with super-refractory status epilepticus and multifocal seizures. Metabolic, structural, infectious, toxicological and autoimmune causes were discarded, while different anticonvulsive agents were administered without any clinical improvement; seizures were controlled 6 weeks after admission to ICU. A 12-year follow-up was performed, during which time the patient presented recurrent status epilepticus with autonomic seizures and progressive cognitive decline.Discussion: This type of status epilepticus is part of the syndrome known as Febrile Infection-Related Epilepsy Syndrome (FIRES), a possibly autoimmune form of epileptic encephalopathy that is refractory to acute and chronic management. There is no report in the literature that includes long term follow-up, therefore, there is no actual consensus about the appropriate management of the chronic phase of the disease.Conclusion: FIRES must be considered as one of the possible etiologies of super-refractory status epilepticus, so early management strategies (like ketogenic diet) can be used in order to achieve control of the critically ill patient, control long term seizures and improve cognitive outcomes, having as the final result a positive impact on the quality of life of the patient. ; Introducción: El estado epiléptico superrefractario (EES) es una patología con importante morbimortalidad que afecta el ambiente neuronal según el tipo y duración de las crisis.Presentación del caso: Se presenta el caso de un escolar con estado epiléptico superrefractario y crisis multifocales. Se descartaron causas metabólicas, estructurales, infecciosas, toxicológicas y autoinmunes y se utilizaron diferentes manejos anticonvulsivantes sin respuesta, lográndose control de las crisis 6 semanas después del ingreso a UCI. Se realizó un ...
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