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

• Published in: Italian journal of pediatrics Vol. 48; no. 1; pp. 199 - 9
• Main Authors: Pavone, Piero, Corsello, Giovanni, Raucci, Umberto, Lubrano, Riccardo, Parano, Enrico, Ruggieri, Martino, Greco, Filippo, Marino, Silvia, Falsaperla, Raffaele
• Format: Journal Article
• Language: English
• Published: England BioMed Central 16.12.2022; BMC
• Subjects: Amino acids; Analgesics; Antibodies; Autoimmune diseases; Child; Cognitive ability; Convulsions & seizures; Differential diagnosis; Drug Resistant Epilepsy - diagnosis; Drugs; Encephalitis; Encephalopathy; Epilepsy; Epileptic Syndromes - complications; Epileptic Syndromes - diagnosis; Epileptic Syndromes - therapy; Family medical history; Fever - diagnosis; Fever - etiology; FIRES; Humans; Immune response; Infections; Inflammation; Literature reviews; Male; Metabolism; NORSE; Parents & parenting; Pathogenesis; Pediatrics; Plasma; Polymerase chain reaction; Review; Seizures; Status epilepticus; Status Epilepticus - diagnosis; Status Epilepticus - drug therapy; Status Epilepticus - etiology; Thyroid gland; Traumatic brain injury; Ultrasonic imaging; Viral infections; Italy; FIRES; NORSE; Status epilepticus; Encephalopathy
• ISSN: 1824-7288; 1720-8424; 1824-7288
• DOI: 10.1186/s13052-022-01389-1

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.