Febrile infection-related epilepsy syndrome

Febrile infection-related epilepsy syndrome, abbreviated FIRES, is the onset of severe seizures, including status epilepticus, following a febrile illness in someone who was previously healthy. The seizures may begin as focal seizures but often become tonic-clonic, and complications often include intellectual disability, behavioral problems and ongoing seizures. The underlying cause is unclear. An upper respiratory tract infection or gastroenteritis often occurs one day to two weeks before onset, and diagnosis involves extensive testing to rule out other possible causes. It is described as a type of new-onset refractory status epilepticus. The seizures are often resistant to treatment, so high doses of benzodiazepines or barbiturates are often used with care in an intensive care unit, a ketogenic diet may help in some cases, and the medications anakinra and tocilizumab have been tried. The source gives the risk of death despite treatment as about 12 percent. It newly affects about one in a million children per year, with onset generally between ages 2 and 17, and males appear more commonly affected. Cases consistent with it were first described in 1961 and the current name came into use in 2010.