Charakterystyczne cechy zapisu elektrycznej aktywności mózgu w ostrym stadium zespołu padaczki związanego z infekcją gorączkową u dzieci

BACKGROUND: Febrile Infection Related Epilepsy Syndrome is a rare and refractory severe syndrome characterized by status epilepticus and encephalopathy. Early administration of immunotherapy agents has been shown to significantly improve outcomes. This study aims to analyze and summarize the characteristic electroencephalographic features during the acute phase of cryptogenic FIRES in children, in order to facilitate early diagnosis. METHODS: A retrospective collection of patients diagnosed with cryptogenic FIRES was conducted. The EEG characteristics during the acute phase of these patients were summarized. RESULTS: A total of 31 pediatric patients with FIRES were included (23 males, 8 females), with an age of onset ranging from 2 years old to 13 years and 8 months. All 31 cases exhibited background slowing. Multifocal epileptiform discharges were observed in 30 cases. Extreme δ brushes were noted in 23 cases. Frequent clinical seizures and/or EEG ictal events were recorded in all cases. Ictal EEG exhibited Zig-Zag sharp or spike waves in the θ-β frequency range, with or without the onset of low-amplitude focal fast activity. The ictal discharges exhibited focal, multifocal, and migratory characteristics in an alternating, overlapping, or independent pattern, with relatively localized spread and rarely generalized spread to all leads. CONCLUSIONS: "Migratory Zig-Zag Pattern", the relatively characteristic acute-phase ictal EEG finding may serve as an specific electrophysiological feature suggestive of FIRES in children. When combined with other ancillary examinations, they may assist in the early clinical diagnosis of FIRES and guide early immunomodulatory interventions to improve clinical outcome.