期刊
EPILEPSIA
卷 64, 期 6, 页码 E87-E92出版社
WILEY
DOI: 10.1111/epi.17591
关键词
FIRES; inflammatory epilepsy; NORSE; pediatric neurology; status epilepticus; superrefractory status; tocilizumab
New onset refractory status epilepticus (NORSE) is a rare and devastating condition that affects previously healthy individuals. When preceded by a febrile infection, it is known as febrile infection-related epilepsy syndrome (FIRES). Treatment involves intensive care and the use of antiseizure drugs, anesthetic agents, and sometimes a ketogenic diet. Neuroinflammation plays a role in the condition, and intravenous immunoglobulin, corticosteroids, and immunomodulatory treatment can be added. Tocilizumab may have a positive response in treating status epilepticus but has varying neurological outcomes. Further research is needed to confirm the efficacy and safety of this treatment for FIRES/NORSE.
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 anesthetic 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 with tocilizumab. We observed a rapid positive response on the status epilepticus and 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.
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