Abstract
Background: New-onset refractory status epilepticus (NORSE) is a rare clinical presentation affecting previously healthy individuals. Febrile infection-related epilepsy syndrome (FIRES) is a subcategory of NORSE and applies when a preceding fever occurs. The NORSE/FIRES Family Registry has been developed to gain insight into risk factors and to assess the spectrum of clinical outcomes amongst survivors. Methods: Survivors, surrogates, and physicians can enter patient data into the REDCap-based registry: https://www.norseinstitute.org/norse-registry-2. Information collected includes medical history, clinical presentation, and quality of life, among others. Participants are invited to complete follow-up surveys for up to two years following presentation of seizures. Enrollment is ongoing in multiple languages. Results: 56 participants are enrolled from 12 countries (2-78 years, median: 12.5, IQR: 20.5, 31 survivors). At ≥6 months after onset, survivors experience a mean of ≥12 seizures per month and remain on a median of 4 (IQR: 3) anti-seizure medications. The median quality of life amongst all survivors was rated 4/10 (IQR: 3.5). Conclusions: Preliminary data suggests that survivors of NORSE/FIRES have a high seizure burden and poor quality of life. This international multi-lingual family registry will help develop hypotheses for future studies and provides an opportunity for families to contribute to the scientific understanding of this disease.
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