MELAS Syndrome is a mitochondrial disease associated with epileptic seizures. The case of two siblings with genetically confirmed MELAS is presented, with a history of recurrent status epilepticus accompanied by neurological deficits, failure to thrive, hyperlacticaemia, and brain lesions resembling strokes. The neurological presentation with epileptic status is highlighted to be considered in the corresponding differential diagnosis in emergency rooms and pediatric hospitals.

Status Epilepticus (SE) in children exhibits high levels of morbidity and mortality and has been poorly studied in Perú. Objective: To describe the frequency and clinical characteristics of children with Status Epilepticus treated at Cayetano Heredia Hospital in Lima. Material and Methods: Retrospective and observational study, case series type, in children under 14 years of age, with Status Epilepticus treated between January and December of 2021. Results: SE was recorded in 28.8% of children with epileptic seizures seen during 2021. Twenty-three children presenting 46 SEs were included. The median age was 2 years, the majority (73,9%) being males, with diagnosis of epilepsy or brain malformation (21.7%) and treated in the pediatric emergency (91%). The predominant semiological pattern was of generalized motor type (52.2%), with remote symptomatic etiology (73.9%), and EEG recorded in ...