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IEC 2025 | The potential mechanisms underlying innate immunity dysregulation in NORSE/FIRES

Aurelie Hanin, PharmD, PhD, University Hospitals Pitié Salpêtrière, Paris, France, comments on the potential mechanisms underlying innate immunity dysregulation in new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES). This dysregulation has been correlated with more severe magnetic resonance imaging (MRI) abnormalities and worse functional outcomes. Dr Hanin suggests that targeting inflammation may improve long-term patient outcomes. This interview took place at the 36th International Epilepsy Congress (IEC) in Lisbon, Portugal.

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Transcript

So there are multiple studies published in the past years. One of the studies investigated what could be the mechanisms underlying this innate immunity dysregulation in NORSE, and they notably observed, so it was a study conducted at the Mayo Clinic in the United States, and they observed that some of the patients with NORSE and FIRES presented with important dysregulation of the monocytes...

So there are multiple studies published in the past years. One of the studies investigated what could be the mechanisms underlying this innate immunity dysregulation in NORSE, and they notably observed, so it was a study conducted at the Mayo Clinic in the United States, and they observed that some of the patients with NORSE and FIRES presented with important dysregulation of the monocytes. And so when they were exposed to bacterial stimulation, so because the monocytes were too activated, the neutrophils can be too activated after and they can produce more cytokine or be more active to cytokine produced by other cells. So it was an explanation for this innate immunity dysregulation in these patients. And regarding also the prognosis, so what we observed initially at Yale University and the NORSE/FIRES studies, so the correlation between the cytokine levels in serum and in CSF with the prognosis, the functional prognosis of the patient. So it has been confirmed by other teams, notably a team in South Korea, that has highlighted that patients with the more important dysregulation in the CSF cytokine profile presented with more important MRI abnormalities and a worse functional outcome at three months. So I think that if we can target the inflammation, we may be able to improve long-term patient outcomes.

 

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