FIRES – Pathophysiologie, therapeutischer Ansatz und Folgen

Abstract

Background The acronym FIRES stands for febrile infection-related epileptic syndrome, which is a rare epileptic syndrome in the pediatric population. The initial presentation of FIRES is similar to febrile seizures (FS). Both start after a febrile episode; however, in FIRES the epileptic seizure evolves into a super refractory status epilepticus within days despite appropriate treatment. FIRES needs to be diagnosed early and treated by a multidisciplinary team to control the status epilepticus (SE) as fast as possible. Limiting the duration of the SE is paramount for the prevention of catastrophic sequelae such as severe neurologic disabilities or even death. Objective/Conclusion We describe possible pathophysiological mechanisms and summarize important clinical features of FIRES. The aim of this review is to raise awareness, foster early recognition and improve neurologic long-term outcomes. Moreover, we propose a diagnostic approach and list therapeutic options providing an algorithm.

Multimodal Management of Febrile Infection-Related Epilepsy Syndrome in a 17-Year-Old Male

“…Pediatric cases of acute-onset intractable seizures accompanied by encephalopathy were first reported in the 1960s [ 2 ]. The syndrome was described under multiple names, including devastating epileptic encephalopathy in school-aged children (DESC) [ 3 ], “idiopathic catastrophic epileptic encephalopathy” [ 4 ], “severe refractory status epilepticus due to presumed encephalitis” [ 5 ], or acute encephalitis with refractory repetitive partial seizures (AERRPS) [ 6 ] before eventually receiving a new name: febrile infection-related epilepsy syndrome (FIRES) [ 7 ].…”

“…Therefore, the goal of treatment in the acute phase is to control status epilepticus. Patients typically require multiple AEDs to suppress seizures; successful combinations of first- and second-line medications vary among FIRES patients [ 2 ]. It is not uncommon for patients to require multiple intravenous anticonvulsant infusions in addition to four to six AEDs to attain seizure control in the acute phase.…”

“…An effective therapeutic option is pharmacologic burst suppression coma with intravenous barbiturate, benzodiazepine, and ketamine infusion, but prolonged burst suppression may be associated with longer intensive care unit (ICU) stay and worse cognitive outcomes. In addition, first-line management of FIRES requires immune modulation with interventions such as steroids, intravenous immune globulin (IVIG), plasma exchange, and ketogenic diet [ 2 , 13 ]. Second-line immune modulation with an interleukin-1 receptor antagonist such as anakinra can be pursued if there is no improvement with AEDs and first-line immunomodulatory therapy [ 2 ].…”

“…In addition, first-line management of FIRES requires immune modulation with interventions such as steroids, intravenous immune globulin (IVIG), plasma exchange, and ketogenic diet [ 2 , 13 ]. Second-line immune modulation with an interleukin-1 receptor antagonist such as anakinra can be pursued if there is no improvement with AEDs and first-line immunomodulatory therapy [ 2 ].…”

“…FIRES is associated with a mortality rate of 10-12%, most often secondary to complications, such as respiratory failure, sepsis, cardiac arrest, intracranial hemorrhage, hypotension, or multiorgan failure [ 2 ]. The median duration of ICU stay is 20-40 days regardless of outcome [ 1 ].…”