Febrile infection-related Epilepsy Syndrome (FIRES): a severe encephalopathy with status epilepticus. Literature review and presentation of two new cases

Italian Journal of Pediatrics

Review

Piero Pavone¹  Martino Ruggieri¹  Filippo Greco¹  Umberto Raucci²  Giovanni Corsello³  Riccardo Lubrano⁴  Enrico Parano⁵  Raffaele Falsaperla⁶  Silvia Marino⁶ 

[1] Department of Clinical and Experimental Medicine, University Hospital “Policlinico-San Marco”, Catania, Catania, Italy;Department of Emergency and Clinical Pediatrics, Bambin Gesù Children’s Hospital IRCCS, Rome, Italy;Department of Health Promotion, Mather and Child Care, Internal Medicine and Medical Specialities, University of Palermo, Palermo, Italy;Pediatrics and Neonatology Unit, Maternal-Child Department, Santa Maria Goretti Hospital, Sapienza University of Rome, Latina, Italy;Unit of Catania, Institute for Research and Biomedical Innovation (IRIB), National Council of Research, Catania, Italy;Unit of Pediatrics and Pediatric Emergency, AOU “Policlinico”- PO “San Marco”, University of Catania, Catania, Italy;

关键词: FIRES;    Encephalopathy;    NORSE;    Status epilepticus;   

DOI  :  10.1186/s13052-022-01389-1

 received in 2022-07-26, accepted in 2022-11-24,  发布年份 2022

来源: Springer

【 摘 要 】

FIRES is defined as a disorder that requires a prior febrile infection starting between 2 weeks and 24 h before the onset of the refractory status epilepticus with or without fever at the onset of status epilepticus. The patients, previously normal, present in the acute phase recurrent seizures and status epilepticus followed by a severe course with usually persistent seizures and residual cognitive impairment. Boundary with “new onset refractory status epilepticus (NORSE) has not clearly established. Pathogenetic hypothesis includes inflammatory or autoimmune mechanism with a possible genetic predisposition for an immune response dysfunction.Various types of treatment have been proposed for the treatment of the acute phase of the disorder to block the rapid seizures evolution to status epilepticus and to treat status epilepticus itself. Prognosis is usually severe both for control of the seizures and for cognitive involvement.FIRES is an uncommon but severe disorder which must be carefully considered in the differential diagnosis with other epileptic encephalopathy.

【 授权许可】

CC BY   
© The Author(s) 2022