Zespół padaczki związany z gorączką infekcyjną: czas śpiączki leczniczej i intensywność leczenia wiążą się z gorszymi wynikami funkcjonalnymi

BACKGROUND: Febrile infection-related epilepsy syndrome (FIRES) is a rare, severe epileptic encephalopathy characterized by refractory status epilepticus, primarily affecting previously healthy children aged 3-15 years. The pathogenesis remains unknown, and outcomes are predominantly poor. This study investigates the acute phase treatment of FIRES, focusing on antiseizure medications (ASMs) and deep sedation with therapeutic coma (TC) for the treatment of status epilepticus, to examine associations with long-term functional outcome. METHODS: We retrospectively analyzed data from 93 children in a multicenter registry-based cohort from our FIRES registry, assessing acute phase ASMs and duration of TC. Neurological outcomes were assessed using the modified Rankin Scale (mRS), with correlation analyses, multivariate logistic regression, and Cox proportional hazard analyses to explore associations between treatment intensity, functional outcomes, and survival. RESULTS: Higher ASMs count was associated with worse outcome, with each additional medication for seizure control corresponding to 52% increased odds of mRS ≥4 (adjusted odds ratio 1.52, 95% confidence interval 1.17-2.10, P < 0.001). Longer duration of TC correlated with a more ASMs and higher mRS scores, indicating inferior outcome. CONCLUSIONS: In our analysis, increased acute treatment intensity and longer durations of TC were associated with worse functional outcomes. These observational associations are likely confounded by underlying disease severity. In the absence of proven causal therapies, the data might be compatible with spontaneous reduction of seizure activity over time in some patients and highlight the need for further research on pathogenesis and pathogenesis-targeted therapies for FIRES.