Febrile infection-related epilepsy syndrome in childhood: A clinical review and practical approach

Febrile infection-related epilepsy syndrome (FIRES) of unknown aetiology is an extremely rare but severe epilepsy syndrome. It is characterized by a nonspecific febrile infection a few days before the onset of superrefractory status epilepticus, followed by refractory epilepsy and high morbidity in previously healthy children and young adults. To date, FIRES is incurable and irreversible. The clinical course may depend more on time than on therapy, while the outcome may depend more on the clinical spectrum than on therapy. Based on a literature search, retrospective data analysis, and personal observations, this review aimed to explore the clinical spectrum and therapeutic options for FIRES to improve outcomes by optimized and more standardized diagnosis and therapy, including adapted immunotherapy and a less aggressive approach to manage seizures, as seizure-freeness is difficult to achieve and, therefore, not the primary goal for cryptogenic FIRES.