Treatment of NORSE/FIRES with Tocilizumab in a child and a young adult.

New Onset Refractory Status Epilepticus (NORSE) is a rare and devastating condition occurring in a previously healthy patient. It is called Febrile Infection Related Epilepsy Syndrome (FIRES) when preceded by a febrile infection. It often leads to intensive care treatment, including antiseizure drugs in combination with anaesthetic agents, and sometimes ketogenic diet. The mortality rate is high, and severe epileptic and neuropsychiatric sequelae are usually observed. Based on the possible role of neuroinflammation, intravenous immunoglobulin, corticosteroids and immunomodulatory treatment (anti-IL1, IL6) can be added. We describe here a child and a young adult with FIRES, both treated by Tocilizumab. We observed a rapid positive response on the status epilepticus and a good tolerance, but different neurological outcomes for our two patients. Further prospective studies may be necessary both to confirm the efficacy and the safety of this promising treatment and to optimize the immunomodulatory strategy in FIRES/NORSE.